BLACKWELL'S

V I M B H U S W , MJl
of California, San Francisco, !:lxw of 7(1(11
Ll~liversi~y
Series Editor, Diagnostic hdiologiss

VISHAL PALL, MBBS

Gorrrnmcnt Mcdiral Cr,llrge, Cha~ldig-~rh. Intlia, Clasr o f l9i)fi
Series Editcrr, LJ, of Texas. Galveston, Rrsidrnt in Internal Medicine &
Prrvcntir,e Medicine

TAO LE,MD
Uuivrrsity of Califoi-tlia, San Francisco. Class or 10116
ALFXANDER GRTMM, MD
St. L o u ~ sUrliversity Schuol of Meclicirle. CIms nf 1999

Blackwell
Science
CONTRIBUTORS
Robert Nason
I ' n i v r r s i t ~of'Texas Medical Rmnct~,Class of "(109
Tishiha Wang
University of Texas M~diralBranch. Class of 21702
Kristen Lem Mygdal, iMD
: ~ ~ of ILZerlicinr. R r s i r l e ~ in
Ur~ivetsitvo ~ b n s School ~t Rarliolog
Fadi Abu Shahin. MTI
Uni~crsin,of Uaniascl~s.Svria. Class of IY!Cl
Jose M. Kerro, MD
La SSae University3Mexico Citv
Vipal Soni, MD
LIC.L4 Srhonl nf hledi~irlr.Class nF 191)tI
Hoang Nguyen, MD, MEA
For tl~weqternUniversity. Clay\ of 2001
 Laura Uell'ortn~
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nsnev Mead, Dxfnrrl OX? OEL.. Enalaiid
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P.O. Box 2li9

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Ounn O X I 4 41'K
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(Trlephnne nrc1lt.m: 4-W 123T4fi500:
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frnln thr pt~hlislirr.rxrrpl hy ;I rrvie~vrrwho may
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No tire
i s this volume have takrn care that rhr infnrmation ct~ntainrdhr.1-eir~
The a ~ ~ t h nof' is nccunte and cornpati-
M e with rhe swncl:~rdsgenrr.11~-;ic-r-epred; ~thy t tinic. of pul,licatiort. Nwcrrl~rles~, i t is ditficult to enrurc
that :ill rhr infnrniaeioll p+n is rnrirt.1~acrlwate F r ~ all r ~irci~nlsr;~nct-s. ~ I C .puh1ishc.r ;mrl a ~ i t l i n mdn n o t
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CONTENTS

Acknowledgments x
Preface to the 3rd Edition xiii
How t o Use This Book mFi
AbbreGations xvii

General Surgery Abdominal Aoxtic Aneurysm B Y

I= r
Acute Cholecystitis
, AA %

Appendicitis
Cecal Carcinoma
Esophageal Carcinoma
Gastric Carcinoma
Gastric Leiomyoma
Intestinal Obstruction-Acute '7
Tntussusception + ?
-""
Pancreatic Carcinoma :
,<
Genetics Down's Syndrome I

- -*
Marfan's Syndrome
Hematology/Oncology Acute Lymphocytic Leukemia (ALL) I
p,"'
Acute Myelogenous Leukemia (AML)
"I

Anemia-Aplastic ;-' "I

Anemia-Autoimmune Hemolytic 1
Antiphospholipid Antibody Syndrome !' '
Burkitt's Lymphoma 1 -
Chronic Lymphocytic Leukemia (CLL) I ^'
Chronic Myelogenous Leukemia (CML) ':
, - 7
Deep Venous Thrombosis
Disseminated Intmvasmlar Coagulation (DIC) ' ' '
Dysbetalipoproteinemia P -a

* ""$
Hairy Cell Leukemia
Henoch-Schonlein Purpura P s

Histiocytosis X-Eosinophilic Granuloma ,, !

Histiocytosis X-Hand-Schu12eer-Christian
Disease Imfi
Histiocytosis X-Letterer-Siwe Disease 1 " A

Aadglcin's Lymphoma " 1

Idiopathic Thrombocytopenic Purpura (PP)' 1
Multiple Myeloma K C 7

Myelofibrosis with Myeloid Metaplasia ' 'i

Non-Hodgkin's Lymphoma A - I
i
Polycythemia Veza (PCV)
Sarcoma Botryoides I: - I

Sickle Cell Anemia I

 Thalassemia-Beta rn
Thrombotic Thrombocytopenic Puxpura r
Transfusion Reaction-Acute Hemolytic
T m s h i o n Readon-Febrile Nonhernolytic "

Von Willebrand's Disease F

,- >
Waldenstriim's Macroglobulinemia I
" - ,
Wiskott-Aldrich Syndrome
Immunology Serum Sickness 1
*
Infectious Disease
8-
Syphilis-Tertiary (Aortitis)
Neonatology Erythroblastosis Fetabs
Sudden Infant Death Syndrome (STDS) ' '
Acute Tubular Necrosis (Am)
'- '
Adult Polycystic Kidney Disease (APKD)
Alport's Disease
8 " .

Amyloidosis-Primasy
Benign Prostatic Hypertrophy (BPH)
1
Bladder Cancer
Bladder Outlet Obstruction, Nephrapathy "

Diabetic Nephropathy
Goodpasture's Syndrome
Hypertensive Renal Disease
IgA Nephropathy {Bergefs Disease) I
--
Lupus Nephritis
Membranopro~iferativeGlomerulonephritis
(MPGN) mI.
r-
Membranous Glomerulonephritis
Minimal Change Disease
Prostate Carcinoma 7- 1
Renal Cell Carcinoma 1

Renal Infarction
Renovascular Hypertension
Seminoma
, "
Testicular Choriocarcinoma
>
Testicular Dysgenesis I

Testicular Teratoma (Mixed)

Testicular Torsion i "

Urate Nephropathy
I -.
Wilrns' Tumor
Pulmonary Adult Respiratay Distress Syndrome
(ARDS) Em
Asbestosis FT
Asthma I

Atelectasis-Postapexative 1
Bronchiectasis 1~
Churg-Strauss Syndxome
COPD-Chronic Bronchitis
COPD-Emphysema
Fat Embolism
Hypessensitivity Pneumonitis
Idiopathic Pulmonary Fibrosis (IPF)
Lung Carcinoma
Malignant Mesothelioma
Pleural Effusion
Pneumothorax-Spontaneous
Pneumothorax-Tension
Primary Pulmonary Hypertension
Pulmonay Embolism
Saxcoidosis
Silicosis
 l ~ tproduction ofthis book, we have had o he sup-
T h r o ~ ~ g h othe
t1n1-1or man): friends and colleag-ues. Special thanks to o u r sup-
port team including Anu Gupta. PLlldrua Fellows, Anastasia
hnclcrson, Srishti Gupta, Mona Pall.-Toriathan Kirsch and Chirag
, b i n . For prior contributions we d ~ d n kGianni Le Nguyrn,
Talm Mathur, Alex Cirimm, Sonia Santos and Elizabeth
Sanders.

\.ire have enjoved working with a wnrlrl-class ii~ternationalp u b

lishing group at Blackwell Srirnce, including Laura DeYoung,
Amv Nu tthrock, Lisa Flanagar~,Slla~vnGirsbergel; Lorna Hind
and Gordnn Tihhitts. For help with securing images for the
ell tire series we also thank Lee Martin, Kristopherjones, Tina
Pan iz7i a i d Petcr Anderson at t h e University o t Alailama, thc
Armed Forcrs Institiare of' Pathology. and many of our fellow
Blackwell Science a ~ l t l ~ o r s .

For submitting comments, corrections, editing, proofreading,

and assistance across all oS t h e vignette titles in all editions, we
c o l l ~ tii~ely
c t1i;mk:

Tam Adamovich. Carolyn Alexander, Kris Alden, H e n ~ y E.

Aryan, Lynlna~zRacolor, Natalic Barleneva, Ileam Bartholomew,
Dchas11isl-iRehem, Sumit Bhatia. Sanjay Rindra, Dave Brinton.
< J ~ ~ l i a nBrown,
ne Alexi~ndel-Brnwnie, Tamara Callahan, David
C~nel;,Rryatl Casev, Aaron Gaughey, Hehert Chen. Jonathan
C:heng-, Arnold Cheung, Arnold C h i n , Simion Cl~iosea,Yoon
Cho. Samuel Chumg, Gretchen C:onant, IrIadirnir Col-ic,
CIlristoplirr Cosgr-ove,Ronald Cowan, Kar.ekjn 12. Cunningham,
'4. Sean Dalley. h m a D i ~ ~ i c l a r n ~Sl~nir
~ d i , Das, Ryan Arrrlando
Davr,John Dabid, Emmanliel rle la G ~ I zRobert , DeMrllo,
Navncut Dhillon, Sharrnila Disqanaiku, David Donson, hdolf
Etchegaray, hlea Elwehio, Priscilla A. Frase, David Frenz, Kristirl
Caumt-1: Yohanncs C~hreegsiallher, h i 1 Gehi, Tony George,
L.14. Govancn, Pat111 GoyaP, AIcx Grimm, Rajeev Cuptit, Ahmad
Halim. Sue Hall, David Hasselbacher. Tamra Heimert, Michelle
Hig-ley. Dan I-Ioi t, Eric Jack~on.Tirn Jackson, Sundar Jayarman,
Pci-Ni Jone, Aarchan Jnshi, Rajni K. Juitla, Faiya7 bpadi, Seth
Karp, h r n n S. ICessclheim, Sana Khan, Andrew Pin-wei KO,
Francis Kong, Pat11Konity.kv, Warrcn S. Krackov, Benjamin I-I.S.
Lau, An11 LaCasc~,Connie Lee, Scott Lee. G u i l l e ~ ~ nLehmann, o
Kevin Leung, PaliI Levett, IYarren Levinsoti, Eric Ley. Ken Lin,
Pavel Lobanov, J. Mark Maddox. Aram Mardian. Sarnir Mektit,
Gil MeImed,-JoeMcssirla, Ruberr Mows. Michael Murphy, Vivek
Nzlndkami, Siva Narapan, Cawell h'gnyen, Linh N p v e n ,
Dcanna Nohlt-za. Craig Nodnrft, George Noumi. D a r i t ~T.
Okrida, ,%darnI,. Palance, P a ~ dPamphn~s,JinfiaPark, Sonny
Palel, Kcarclo Pietrohon, Rim L. RahI, Aashira b n d e r i a ,
Rachan Reddy, Reatriu Reig. Maiilou Reyes, Jererny Richmon,
Tai Roe, Rick RuIler, Rajiv Roy, Diego Ruiz, Anthong Russell,
Sanjap Sahgal, Ulmimala Sal-kar.John SchiIling, Isahell Schmitt,
Daren SchuhmacI~er,Soi~aIShah, Fadi Abu Shallin. Mae Sheikh-
Ali, Edic Shen, Justin Smith,Jol111SnlIak, Lillian Su,Julie
Sundararn, Rim Suti, Seth Sweetscr, A n t o ~ ~Talavern,
io Merita
'San. Mark T a n a h , Eric Taylor. Jess Thompson, Indi Trehan,
Kay~noncITurner, Okafo Uchenna. Eric U y p a n c o , Richa
\.'amla, John Wages. Alan Wang, Eunice U1ang, h d y Mreiss, ,%my
Williams, Brian Yang, Hatry Zaky, Ashraf Zaman a n d David Zipf.

h r genernuslv conrribu ting images to thc entire Uulrlw~ly~~ind

Clinical I f i g x p l t p Step 1 series, rve collectively thank the staff at
Blackwell Science in Qxford, Bostnn. and Bcrlin as well as:

~ ~ . Mead: Rlackwell Science Ltd, 1996.

I\xfnrrl. J. ~ M e d i r i ?Osney
Figures 2.14, 2.15, 2.16, 2.27, 2.18, 2.31, 2.35, 2.36, 2.38, 2.43,
2.tj5a. 2.65b, 2.65c, 2.103h, 2.105b. 3.20b, 3.21, 8.27,8.2%,
S.733, 8.7fc,10.#lh, lO.gtia, 1P.28a, 14.6, I.Lf.16,13.50.
* Bannisrer R, Reg): N,Gillespie S. Inf~ctiuusI l i s ~ c t . c ~2""
, Edition,
Osuev Mead: BIackwell Science Ltrl, 2000. Figures 2.8, 5.4,
5.28. 18.10, MT5.3:!,MT5.6.
Berg D. A d-r~nnrpdClin &.a/Skillr mnd Phg~icafL)inposi<c.
Rlnrkw~llScience Ltd., 1999. Figl~res7.10, 7.12. 7.13, 7.2. '7.3,
7.7, 7.5, 7.9, 8.1, 5.2, 8.4, 8.5. 9.2, 10.2, 11.3, 11.5, 12.6.
C~~srhiel-i A, fIe11nessy TPJ, Greenhalgh RRM, Rowley DA,
Grace FA. C l i n i r n l . 5 ' 1 1 ? ~Iq)sney
. Mead: BIackwell Science
Ltd, ilc391j. F i g ~ i r e13.19,
~ 18.22. 18.33.
* Gitlespie SH , Barnford K M~ifimf
Mirml~ioktgand I! fPction af a
Glrirrrr. Osney Mearl: BIachvell Science Lid, 2000. Eipi-cs 20,23.
Ginsberg 1.. I.rciure lVotes on Nmruluml, 7." I':clirimn. Osnry Mead:
Black~vellScience 1,tcl. 1999. Figurc~12.3, 18.3, 18-31).
* Elliott T, Hastingq M, J>esselberger U. Lecture hlutrs mr M ~ d i r a l
1 2 f i m l ) i u l u ~3
'' Osiley Mead: Blackwell Sciet~ceIAd,
, I.~~li/ion.
1 9 9 7 . F i ~ i r e s 2 , 57,8,9,
. 11, 12, 14, 15, 16, 17, 19,20,25,26,
27. 29,3U, 34, 35,52,
 Mehta MA, Hornrand Ak! Hn~rnnloln~v nl rt Glrmr~,0 0 ~ n e y
Mcad: DIackwc11 Science Ltd. 2000. F i p r e s 22.1, 22.2, 22.3.

l'leaw let tis know if' your name has been rnissecl or misspelled
a n d we will br happy to make the update in the next edition.
 PREFACE '60 THE 3RQ EDITION

Wc were v e r y pleased with t h e ove~~.vhelrningly

positive stliden t
reeclhack Tor the 2nd edition of ollr I h d w u n d C?inirrc! ~ ~ i b r l l ~ / ~ f c
series. \Tell nver 1 00,000 copies of the LTCLTbook4 are in print
and have been used bv shldcnts all over the world.

Over the last hvo years mrc have accu~nulatedand incorporated

over a thousand "updates" and improvements suggested by you,
our r.eaclers. includulg:

I * many additions of specific boards a i d wards testable contetlt

I d ~ l e r i n n sof reclundant and overlapping cases

I reordering and reorganization of all cases in both series

a new maqter index by case name in each Atlas

correczion of a Cew Cactrial errors
diagnosis and kcarmen t updates

I * addition of5-20 new cases in every hook

* and thr addiriorl of clinical e x a m photographs within LTCb-

A nalnm?

And most important of all, the third rdition sets now include
two brand new COLOR ATLAS; supplcments, one for each
Clinical Vignette serics.

* The CTCIJ--13ccsirS r i w r p Cdur Atlar I ) includes over 250

(St*
color plale5. divided into gross pathology, mici~oscopicpathol-
ogy (I~istology), hematolop, and mictol->iology((smears).

Thc I 'CV-CJdni(*rrlS r i m r ~C h r Atlas (St+ 2) has over 125 color

plates, including patirnt images, dermaloIogy and hmdus-
copy.

Each atlas image is descriptirelv cagtionrd arid linked to its cor-

responding Step 1 case, Step 2 case, and /or Step 2 MiniCasc.
How Atlas Links Work:

Step 1 Book Codes are: h = Anatomv Step 2 Book Codes are: ER = En~ergenryMedicine
RS = Beha\+c>r:tl Sricncu I M l = l n r w nal Merliril~c.Vnl. I
BC: = Rinrhemist.7 IM!' = Internal hlpriiriue, \bI. 11
54 1 = J~!irrc>hinlt>~. Vd. 1 hEL1 = Nt.ui~,logv
Mr?= hiirra,l~iology Vnt. IT O R = OB/C;\N
P1 = P a t l ~ n ~ ~ t ~ y s iI'nl.
n l o I~ , PED = Pe~liatr~cr
~ 11
P2 = P a t l i o p h v s i o l o ~Vnl. SITR = S I I I X ~ ~ !
P.7 = Pa~huphy>ioIuby.
Vfh. 111 PSY = P~yrli~atry

\ n
-
PH P l ~ ~ ~ r ~ ~ i ~ ~ c o l o ~ ~ ~ M(: = ViniCase

Case Number

, ER-035A, ER-0350

/M-p3-032A
Indicates Type uf Image: I [ = f lumatnlnp Indicates UCVl or UCV2 Series

PC; .
I (:rrlss Pathnli~v
PM Microcctrpir lbarIiol[>p
;
.

if'rhe Case numhcr (032, 03.5, etr.) is not f o l l o ~ ~ ebyd a letter,

then thcre is only cine image. Otherwise A, B, C, D indicale
up to 4 ima~es,

Bold Faced Zinks: Irl order to give you access to the largest
numbcr of-images possiblc, we have cliosen tn cross link he
Slep 1 and 2 series.

* IT the link i s l3old-faced this indicates thal the Iink is clirect

(i.e., Step I Case with the Basic Science Step 1 Atlas link).

If~helitlkisnotbold-facedr h i ~indicates that thelink is incli-

rect (Step 1 c x e wit11 Clinical Sciencc Step 2 Atlas Iink u r vice
versa).

i& havc also irnpl~tn~nred

a fm structutal changes z~ponyour
rcqucst:

Each currcnt and fi~riireedition of mu- popular First Aid fw

thr i:SMI,IS Strj) I (Appletoil Pc T,ange/McGraru-Hill) atlrl Irir.~f
ilidjnr thr! ITTiVILl St+ 2 (12ppleton kk Lange/McCraw-IIill)
honk will be linked to the corresponding UCV rase.
\& eli~ninateclUCZ' + Fimt Aicl links iu they frequently
hccu~r~erjltl of date, as thc firsf A it! t>ooksare re\riscd yearly,
* The Color Atlas is also specially designed for ql~izring-
captions are descvi ptivp and do not ~ i away
~ ethe case name
directly.

We hope the updated GCV series mill remain a ilrliql~eancl well-

integrated st~idvtool that provides compact clinical col-relations
to basic science information, They are designecl to he easy and
f t ~ n(cornparat;vely) to read, and hrlpful For both licensing
exams and the warrls.

We invite your corrections and suggestions Tor thc fourth edi-

tion of these books. Fnr the f i n t subillission of tach factual cor-
rection or new vignette that is selec~ecffur i~~clusion in the
fourth cdition. you will receive a personal acknowledgernenr in
thr rerised book. If y o u snbmir over 20 high-qllality corrections,
additions o r ncw l-ignet~eswe will also consider inviting you to
become a "Contihutnr" on the book o f your choice. If you are
interrsterl in I~ecominga potential "Contributor" or "Aulhor"
on a future LTCV book, or-working with our tear11 in cIe~,eloping
additional books. please also e-mail us your CW/resurne.

U'e prefer that you submit correclions o r sugg-cstionsvia

eIectronic maiI to UCVteam@yahno.com.FIcase includr
"Underground Vignetres" as the subject of yol~rmessage. Kyou
do not have accesq to e-mail, usc the follo~vingrllailing address:
Rlack~vellPublishing. Attn: UCT Editors, 350 Main Str eel.
Midden, MA 021 48, USA.

Viltos Rhws/,chon
Vishnl Pall
Ton I,P
O c t o b ~ r2001
Tlris qeries was originally dcveloperl IO adcll-eqs the inc~exqinji
nt~mherc~fclirlical vigmecte ql~estionqotl n~eclicalexaminations,
including the LTSMLE Step 1 and Step 2. It is alsn designcrl to
st~pplementant1 rr~rnplemenI the popular ATTI Aid /;7r I ~ P
CL%iZJ.: ,$/PSI I (hppleron 8: Langc/McGmw Hill) and fin! Aid
,fm. tJw tCrSiIfESi+ 2 {Appleton II: Lange/McGraw Hill).

'ach LTCV 1 book uses a serqiesof approxirnatelv 100 %upra-

prototypical" cases aq a way to condense testable f a c and ~
associations. Tllc clini.cal vignetres in this serics arc designed to
iricorparatt as manv tcsmhlc facts as possible into a cohesive
ant1 metnorable clinical picutre. The ~ i g n e t t e srepresenr
com~msite\drawn from general and qpwialry testhook?,
reference honks. tFlousands of USMLE swle q ~ t e ~ t i o n and
s the
personal experience of the authors and r~viewers.

Althc~ugheach case tends lo present a11 t h signs,

~ symptoms,
ancl cliagnostic findings for a particulzr illncss, patients gener-
a1Ty will not present with such a "complete"picture either clini-
cally or on a medical examination. Cases are not meant to
simulate a potential real patient or an exam iignctte. All the
boldfaced hbbuzzwords" are for learning purposes and are not
expected to he fol~ndin any one patient with the
r~ece.;.iar-ily
rliseasc.

Definitions o f scIrc tud important trrms arc. placed within the

vignrties in (SL~AI.I, ~ A P S )in parent tieqeq. Other pal-en thetical
rcrnark~oftcn ~-efcr to thr pathophysiulop or mechanism of
diwaqe. The forinat shot~ldalso help stl~rlerlt? learn ta present
cares ~ i ~ c c i n c tduring
ly oral "huller" presentations on clinical
rotations. Thc cases are mcant to serve ;15 a crmdcrlsrd review,
not as a prirnarv reference. T h e information pro\-icled in this
hook has 13een prcpared wit11 a great cleat of thought and care-
ful rr:search. This br~okshoulcl not, ho~vever,bc considered as
I a u r solr wurce of information. Comctions. sr~ggrs~ions and
sithn~issionsof new cases are encoi~ragecland will I>eacknowl-
cdgcrl and incorporatrd when apprnpriate in fit ruse edE [ions.
5-ASA 5-aminosalicylir acid
-4BGs arterial hlood gases
hRVn ad tiarn;rcin/b~eomycin/vi~icri~tine/dacarb-nxine
,ICE angiotunsin-convertin g en q r n e
ArnH adrenocol-ticotropic hormone
XDF-I aritidt 111-eticlinrmone
AFr' alpha fetal protein
AT aortic insuff~cierlcy
AIDS acquired iinm~~nndeliciencv s~ldrr)me
ALL acute lymphocytic leukemia
ALT nl,~ninet ratlsaminasc
AMI, acute rnyrlogenous leitkrmia
1l.K h antiiluclear antibody
m s adult respiratory distress syndrome
ASD atrial rrpml defect
'IS0 ail ti-ctreprolysin 0
AST aspartate transaminase
iZV arteriovennus
BE h-niiiam enema
BP hlnod preswre
13UN blood rtrea nnlrogett
CAD coronal? artery discase
CALLrZ comnlon acute lyrnphobla5tic leukemia antigen
cnc completc blood count
CI-IF congeu~iveheart failurc
CK CI-eatinekill as^
CI.1, cElronic 1)qnphocytic leukemia
CML chronic myelogennlis Ieukemia
CrnT C ) ' ~ O J Iegalo\7irus
~
CNS central nen70ussyiterrl
COPD chrnnic nb~tr-ucrivepulmonary disease
CPK ci~atinephosphokinase
CSF cerebrospinal fluid
CT computed romogl-aphy
CV,4 cerebrovascular accidenr
rnR chcst x-ray
nIC clisscminaied itlrl-a\mcularcoapibtion
DIP dislal inte~=phalan~eal
T)KA diabetic kctoacidosis
DM diahetec mellitus
DTRs deep tendon reflexes
DVT clcep venous thl-onlhoqis
ERlJ Epstcin-Barr vir-us
ECG rlcctrocardiograpFi~
Echo rchuc;~~c~iu~aphy
EF cjrction fwctior~
EGD esol>haqogaqrmds~nrlenoscopv
EMG electromyograpl~~
ERCP encloscopic rctro<qaraclecliola1l~opalzrreat~)grap1~~
ESR erythrocyte sedimentation rdtf
FE\ ' fi~rcrdcxpiriitnry vnl~tine
FNiZ fine needle aspiration
FTIZ-A RS fluoscszcnt truponrmi~larllokody absorption
FVC f{~rceclti tal capaoi tv
GFR glomrnllal- Siltralion 1-ate
GH !growth hnl-monc
GI gastrointestinal
GM-CSF granulocyte macrnphagr colon? ~tin~nlatinfi
f ar
- .tor

GU ge~iiiot~~ii~a?y
Hrn' hepatitis A \ims
hcC; hr~maizchorionic go~~adotrc~ph in
I-IEENT head, eyes, ears, now, and thl-nat
HnT human i r r i mruiorleficiencr, vin-us
HI.:\ 11u1nanleukocyte antigc11
1-1r l history of pl-esen ill ~zess
HR hear1 t a t e
HRrG Imrman l-ahies imrnunc globulin
I IS herrdi tam .;pher<)c~locis
ID/(:C: idenrification and chief complairi~
IDDM insulin-deperidm~dia11e1e.i n~ellitm
Ig imrnunoglohiilin
IGF insulin-like g r o ~ q hIjctor
IM itn tmtnuscular
.PT jugular venous preswu.e
KLrR kidnevs/ttretrr/hradclcr
LDH lac^ ate dehydrogenase
LES lower esophageal sphincter
k.F-s% lilrer.fi~nctiontests
LY lumbar punutu7-e
LV lcft vuntric~~lar
LvH left ven i r i c ~ar.
~ l1 1 ~ ~ ~ 1 - t r o p h y
T.yt PS electrolytes
MCHC mt-an corp~~rcular Il~moglohinconcen t r ~ ~ t i o n
\4c\- mean carp~~scnlar volurnc
MEh- nlrrltiple e~~rlocrinc neoplasia
MGUS

mvocardial infarctio~i
mcchlorc.chairiii~eJ\.inc~istine (Onco\wrin)/
procarhazir~e/pi~prl~~ison~
MR rnagn'ctic rrsoilance (imaging)
KHL IIO~I-Hodgkin's lymphoma
KIDDM non-i1isr1lin-rle1~~11clent
dialrrctcs n~ellii11s
NPO nil per os (nt~thingFly rrlou th)
NSXl D
PA
PIP proximal in terghalangcal
PRS
PE pt~ysicalcsam
P ~ s pulmorl;uv Funrtinn lerts
PM I
I'M N
PT
PTTA
PTH ~ ~ ~ w d i v v mlzorilloncirl
PI-I' partial ~hromhoplastiri ~ir~re
PL'D prptic utccr disrase
RBC red blood crH
RPR rapid plasma l-eagin
RK respiratoq mtr
RS Rcrd-Stn-r~herg(cell)
RSI r . i ~ l > \t , e ~ i t r i r ~ ~ l a r
RVH r i g l ~ tv~ntricularhvpertrt~plir
SUFT mall 1 x 1 ~el. F I ~~tv-th I rc111g11
STAIIH wndrorne of inappropriate \ecrriic~noTAD1-l
S LE
STD scxuall!r tranlirni~terl disease
T I T 5 ~hyroirlr ~ ~ n c t i otests
n
1P'l
TSI-I lli~roid+timt11aiing Ilorrnonc
TIBC total iron-Frinding rnpaciry
TIPS liansiug~~lar intrahepatic portosY4stenlir s111~1a
t
TPO
TSH
Trl'
LT.A ul-innlysis
LiGI upper GI
rs
VIIRI, Vcncrcal 13iqease Research Lal>oratory
1's vital s i g n s
IT ventricular tac11vc;~rdia
M'BC wllite blond culI
M-PMr Il.blff-Parkit~son-'It'tiite(s~di*ome)
SR x-ray
 IDJCC 60-year-old white male undergoing a routine
An a~y~iptomaLic
phyical exam i s dismvered to have a prilsating abdominal mass.

HPE T h e patient has a history of occasional abdominal pain and

hypercholesteroEemia that has been poorly con tl-ollerl hy diet
ancl mr-dication.

PE Pulsating, painless upper abdominal mass approximately 3 cm 2

m
m
in diameter.
s
V,
C
Imaging KUl3, laletxi: calcification of aneurysm wall. CTJUS, abdomen: ;D
G)
dilated aorta w i l t 1 il-regular calcifierl wall; large, eccentric m ~ ~ r a l m
;D
thmmhus wen. <

Gross Pathology Mosl aneur>'srnsaye Incated Iwtweet~renal arteries and iliac

hifi~r.catiorb:thron~busmay alsn he 111-esent: in trnrnural
clissectinn nlay also he wen.

Micro Pathology hrlenrysm wall contains all three la!.et-q (inritna, media,
advent i tia) (''IHI~F'' b \ ~ ~ ~ ~ ~ % h ~ ) .

Treatment Surgical replacement with graft {if > 5 cm or symptorrla~ic)

;
consider endovascular sten t/gr.af't.

Discussion The iisk nf rupturt*with potcntiallv fatal bleeding increases w i t h

sizc. Al~dominalaortic aneurysm i q ur~rallvcauwrl by
atherosclerotic rlispase and is often associated with coronar?.
artery disease. I t is also rn11scd by trarima, i~~feclioxi (r.g.,
syphilis), cystic medial degeneration, and art eri tiq. Sequelae
incluclc rupture, embolisalion, infrrtioi~,V ~ S C I I I J I - O C C I L ~ S ~ O ~ ~
secondxr-y ro rhr-mnhus fortnation, a n d compression of acljacen t
structures ( e . g . ,urctcrs, vertebrae).

Atlas Link P ~ T ~PG-P2-001

T-I

A B D O M I N A L AORTIC ANEURYSM
 I DJCC h 42-year-old wI~itcfemale. llic mother of five, dt.veIol>sacute
intermittent pain in the right IIpper quadrant ant1 r i ~ h scap111a
t
after eating a fatty meal.

HPI She is nCNative American ancestry anrl i.; 30 pn~uncisoverweight.

Shc also complaii~sof' nausea anrl has vomited thrce times. Skrr
has I~ikcIsr\.eral pi.ini. epi~orlesol \ i ~ n i l a rpain rolluwing mrals.

PE VS: I'cvcr; tachycnrdia. PE: obese; tcnclcrnrss in l i ~t upper l ~

abtlomiiial rl~iarlc~!i L w i i 11 inspiratory arrest on palpation
(Xlu K I ~ II ~ SHS A ) ; hypoilctiv~lmwrl sounds.

Labs Hvpel-rliolc~terolenlia.CRC: Ie~~kocytosis with mild

neutrophilia. Elc\r~tedclii-ec.1bilir~rl~in
; elevated a l k a l i
phospl~atase.

Imaging IlS: rIistcnrled gallbladder with d thickening r o r ~ t i ~ i r l i i ~ g

mliltiple echogenic shadows (stnncs). NIK,HIDA: hilure to
\<stla!i7t, # j ~ l l l ~ l ; ~itidic-;~~e< nhstl-uction by stone.
~ l ~ l ~ r -C ~ JirS rit~rr
~

Cross Pathology Gailhlaclrlcr inflammatio~iranging tioin wall ecleini~to acute

gang?-rncI\+ th ~iccrr~sis, pur fo~-m;~t
irlr~,ancl per.fnr;ltion with
p ~ r i i o ~ i i l iMOSI
s. S I ~ I I P IXI-P cotnpnwrl of cholesterol: less
commc~nare pigmented stones made principally ol- 1rncnuj11-
~ a i e r I>ilir\~hin
l and r a l c i ~ ~salts.
~n

Micro Pathology

Treatment Conscrvarivc trc:ltmcnr includcs n o oral i~ltakc,rli~sogastric

aspiralicyn, TCT fluids. analgesic$. ancl anlibiotics: cholecystectomy
is rlclinitivc 1i.ratmeitt.
( I r.il~;{l ly li~paroucol~ic)

Discussion A l t h o u ~ hcal~iiliarr involsccl in moqt c a w s oi'actile rhc~lecsq~itis,

acaIculous cases aricr a f1e1ni tnhiliat y inainr surgeries, srvcrc
traunia ~ 3 1b u r i ~ smid , ~epsisa ~ l di l l I ~ pmlpal-turn
P qtate.
DiiTei.rntial rliagnosis i n c l ~ ~ r lappcndicilis.
c.~ panci-catitis,
- i l peptic
~ > ~ i - f i ) ~t-d ~ ~ ~ l c r~iv~!c)n~pFiri
t; tiq. m\~orardialinfarction,

and riqht lower lohc pneumonia. Clinical risk factor-s inclttde

t l ~ cfour Fs:fat, female, forty, and fertile.

Atlas Link

* ACUTE CHOLECYSTITIS
 ID/CC A 17-vea~oldtnale snident presents with anorexia and poorly
Ioci~liretlge~umhilicalpain followed by nausea and two
cpisodcs of vomiting.

HPI the pain shifted ta the right lower

Four hours after 131-eser~htion,
quadrant and lnc devcIopcd a low-grade fever.
CI
-
rn
Z
PE 1's: mild tarhvrarrlia; lotv-grade Cever. PE: right lower quadrant m
m
tenderness with guarding and rebound; pain in right lower
q~mclrarttwhen pressure applied to left lower qr~adrant
?
Ln
(Rol-srnn'q SIGN) ; pain localized to junction of outer and middle
third of the line from anierinr snperior iliac spine to l~mbilicus
5
m
G)
30
(M(.R~~IZN~,'I"S
POIV 1.1 ; rig11t IOIVP~ qltadranl pain elicited 1 3 ~ <
passive hip flexion (PSC~AS S I ;N)~ atid bv pas~iveintcr~lal arid
external rotatinil of hip ( ~ ~ ~ T L * R A T Csicw).
>K

Labs CRC: elevated WBC counrf predominance of neutrophils

Normal serllrn amvlase. I!A: t~ol-mal.

Imaging KLJB: right psoas siradow hl~11-red: ~ent-ralizedileus with air-fluid

Ie\.els; increased sofl tis~lledensity in sight lower quadrant; smalI
rarIinp,~ql~r Fecalith i n rig111 lowrr qr~adrant.LTS: noncompressible
tubular strl~cturein right lower quadrant.

Gross %thology &rlv Iesinn : hyperr~nicappendix with Iihl-innus exudate; late

lecion: purnlent ex11date wit11 ilccrosis and pcsforatioit; fecalizh
occasionally pi-esunt.

Treatment Appendectomy with preoperative antihiotic coverage.

Discussion The p ~ a kincidence or appendicitis i s in tlnc second ancl bird

decndes. Caures include obstruction by fecaliths (33%) ancl
lymphoid hyperplasha (GO<% "u) it is occasioni~H): cazisecl kv ttlrnmrq
(carrirlnitl turnor i~ l l ~ einns1 conimc)n Lllrnor of the appenclix).
para~ites, forcign bodies, and Crohn 's discasc. Complications
includc perforation, periappendiceal abscess. per-i~oni tis, and
gunm~lizeclor wo~rnclsrpsis. Differential diagnosis should
inclt~dernrel-oculitiq. rnesent~riclym[rharlrtliris,acute salpingi~s,
ectopic pregnancy, pain on o-vc~lation( ~ ~ r r r , ~ s r : ~ arrd-~~~uzj,
Meckel'r, tli\le~,lict~litis.

AtZas Links ITElTII PI-a2-003, an-a2-003

APPENDICITIS
 HPI She 11x5 atso notired sipificant weight loss and intermittent
diarrhea.

PE Mar-kerl pallor; palprrhle I r f t ~111~1-aclatic11lar

lymph node
IOU' 5 NC)I>F) : palpal>le niaqs in right iliac fosqa:
(VIRC'I
hepatomcgaly.

Labs C:RC/PRS: microcvtir, h ~ p u c h r ~ ~anemia.

~ t i c Positive stool
guaiac test: rlrvatcrd serum rarcinoen1l9r~~orlic; ~ t - t tigen (CEA)
1ez.t-1%.

Imaging RE: Iai-~e,i r r q ~ ~ l ah~ngaritlg

i. mass in cerum. US: metastatic
hepatic nodules. Colonoscopv: largr fungating growth in cecum.

Gross Pathology Cauliflower-like, Fungating, nonobstnacting growth in cecum:

Inav hc polvpc3ir1, ~ e s ~ i lne ~. c. ~ n s t r i c t i n ~ .

Micro Pathology WellLrliiTerentiated artenoc:~rcinoma.

Treatment Righr hpmicolec~nmyw<th remporary colostomv; adjuvant

c ~ t ToSlow up for recurruncc by mori itori ng C:FA
c h ~ ~ nh~t-apy:
levcls.

Discussion Earlv detecuon of cccal c;~rrinomai.i I ~ screening

v l'nr occult
blood in the stool. It is the suco~ldmost conlmon rallse or
umnccr d r a t h ; its ir~cidellceirlcwaseq markedly alter age 50.

Atlas Link mP G - P ~ - O O ~

9 CECAL C A R C I N O M A
 ID/CC A 6Ryear-olcl hlack male pruse~lt~jivilh anorexia, progressive
dysphagia. oclynophagia, arlrl weight loss.

HPI Tl-lc patirnt has been drinking w r y hot tea since hc was E I years
olcl and smokes or~epack ofrigarcttcs per clay, His history also
rcvuals heaby alcohol intake; ncc;lsional cot ~ g hvomiting,
, and
Cl
regurgitation: and scvcrc dysphagia with solids, progressing to rn
liquids.
z
m
7u

PE Emxciadon: fixecl, nonpainful supradavicdar node; pale

?
!e

Labs CBC JPBS:hypoct~romic,microcytic anemia. Hemoccult-pusitire

;e
<
srtml; hyoalhuminrl nia.

Imaging UGl:irre@arfungaTatingesophagealmassinmiddletl~ll-rlof
eqophagus ui th CT, chest: ii-regular
nhstt-~~ction.
esophageal m a s with invasion of mcdiastitilrm and enlarged
para-aortic lymph nocle.

Gross Pathology Large fungatirig mass prorruding towdrrl esophageal lumen.

Micro Pathology Sqnamot~scell carcinoma on biopsy.

Treatment Laser ablatiori of t unmr with palliative slenL placement;

palliative radiotherapy: surgical rrsecliol~rollowed by
cl~emotherapyplus radiotherapy for curable tumors; evenl~ral
gastl-ostomy tuhr pl~cernen t.

Discussion T h e most cornmu11 variant of esophageal carcinoma is

squarnous ceU carcinoma, which is assuciated with aEcollol rind
tobacco use and is more common in blacks. A less cnmmoiz mri-
ant is adenocminoma. which r~suallyir~trcllvesthe distal third of
thc esophagu.; and is more coinnlon in whiles with Barrett's
(glandlilar-metaplasia of thu squanluus epi theli~irl~
of thc disl;tl
esophagL~sis causcd hy chronic. untreated gastrocsophageal
reflt~xrliscasr).

At\as Links PG-P2-OOSA, PG-P2-005B

* ESOPHAGEAL CARCINOMA
 ID/CC tlrl %?year-old whitc male complains of anorexia, frequent
vomiting, a n d a gnawing midepigastric pain of several mnnths'
d~u-ation.

HPI Thc pain is not relieved by antacids or milk. The patient has lost
significant weight over t l ~ epast fcw m o n ~ h sdue to diarrhea after
e v r r y meal.

PE Pale. emaciated male in modcrate dist~.ess:left supraclavicular

lynph node (\r~'r~t:~nw's
NCIDF.) palpablc.

Labs CRC: hypochromic, rnicroeic anemia. Stool positive for occdt

hlood; L R s normal.

Imaging UGI: 1;ir.ge fungacing lesioil on grratci- cllrvature of stomach

with kislulous tract running to uarlsverse colon. EGD: same.

Gross Pathology Pol;vpaid, raised, f m g a tirlg tnass p t-qjecting into lrunen : sitnated
at distill erld of'qrnmach.

Micro Pathology Biopsy rcveals a well-differentiated adenocarcinorna with

signet-ring cells.

Treatment S ~ ~ r g e rradiotherapy;
v; c l~ernotherapy.

Discussion Most cnmmonly found on the lesser curvature in the antrum

and pyloric areas. aderlocarcinomas nlav hc one of two types:
intestinal ancl difftwe, C11ronic atrophic gaytri [is, pernicious
anpmia, infection with H. #'Ion', post~usgicalgastric rcmnai~tr;,
and type A blood are all predisposing risk factors for the devel-
opmcn t 05 adenocarciraoma. I t most cammonlv spreads hematogc-
nousI!~ tn the liver- and may spread trar~speritoneallr;to thc
rrvaries (KRUKENRERC
TL-MOR).

*
5

> <>
v GASTRIC CARCINOMA
 ID/CC A -14-year-old nrale is aclrniltetl to the hospital follo~~ing episodes
uf vomiting blood (HI+.M.IAII ~ U I : S I >ancl
) p a s s i ~ ~black,
g tarry,
foul-smelling stools ( LTEI.V,NA) .

HPE THe has experienced recurrent painless hematemesis and melena

Ii)r sew t-a1 vears, I x ~repcatcd
t c.va1~1xtionshave heen negative.
t)

Labs CRC: rnicrocytic, hypochromic anemia. N;lsc)gastric aspirate 11x5

?
E
L
coffee-ground appearance. ;D
rn
rn
n
Imaging UGI/EGLI: fixin m a w in fundus c ~ stomach
f with 2-cm ulcer o n <

Cross Pathology nodrllai-

I'ostoprrativc specinlerl I-evealsa firm, circ~rrr~srribecl
mass within the gastric wall cowrccl ky Initcosa.

Micro Pathology Ill~orliugintrrlacetl bzmdles of spincllc-shaped ccrlls: nn

ctvidei~cenr anaplasia.

Treatment S ~ ~ t g i cresection.
al

Discussion G a s ~ i clcio1nyom;-1i s 1 1 1 most

~ common benign tumor of the
stomach.

? GASTRIC LEIOMYOMA
 IDJCC male p~esellts wi ~h crampkg abdominal pain anrl
A ~lO-vc;~~--old
vomiting of 3 hourq* rlul-ation.

HPI H r also romplains of a n inability to pass stool or flahls

~ ) the- pitst IS days. Twn years ago, Ilc i~r~clrrwerli
( O I K T I I J A T I For
an emergency appenrlectr~myLi ,r a r-tlpt~~rerl appcndis.

PE Ileh!dl-ation ; abdominal distention: gerirr,tli/ed mild tenderness

ovrr al)clr>ine~iwi thouc rebouncl o r guarding: tlowel S ~ U I ~ ~ S
hearrln$ high-pitched tinkles during pain paroxysms.

Labs CDC:/PRS: Ie~iknqt tra tion. Ser.lxrn amylnse

nri s with I~emoconccn
levelu ilormal.

Imaging XR, ;~htloint=n:" ~ t r p l n c l r l ~pattern

r" or ~nultiplcdilated loops of
s m d bowel and multiple air-fluid levels: colon and rectlim
gaqless (air in rolnn or rectam wor~ldi~idic;~te an in~csrit~al
ilenq): iln rrcp ail- unclez dii-lph1~1g111.

Treatment J3'fluid aild cluctrc~l!re repl;lrrmenr; na50pstric suctir~n/

c l r c r ~ i n ~ ) ~ ~ e br-oad-spectm~m
saio~~; antibiotics: sr1rgcr.y.

Di~cussion T h e most comrnori cause5 OF small howel ohstl-l~rtinnarc

intestinal adhesions secondary to prior abdominal srrrgery,
intussuscepiion, volvtplus, :tnrl incnrucratccl hernia: 1l1crrlosi
cornmoil cnusus of large I~owelobslrur-tinn arc carcinoma,
volvulus, and s i p o i d diverhditis. Complications iriclc~dr
strangulation ~ I C necrosis
I of the hc>~vrl ~v.111 luading lo
perT[~ratiotl,peritonitis. sepsis. arlrl h o c k .
 A11 18-monthsld male is bro~ig111 to t11e emergency room by his
parent? becallsc of acute, intermittent abdominal pain,abrlonli-
1x11 distcntiorl. and parsaqe oT'*redcurrant jelly" stools.

The chilrl had previously hcrn wrll, m d h i s iniinl~rtizarion

scherlule is roi~ipletc-.I-le vomited twice fi~llowingarlmissian.

Child ca-ying ancl scrcarnir~g,with knees dr;lr\n In al~domcn;

; ~ b c l o r n ~tenclcr
n and distended; oblong (saurage-shaped) mass
in atwIornen ( rnr~stoften in right uppcr quad[-all[) that hardens
wirh palpation: exartlir~ingfinger stainctl will1 mucus and blood
o n digit;al rectal exan~ination.

Labs on stool exam; no pathogrn on ston7 cultrlre.

Ko pm-asiitr.~

Imaging XR, ;ilidolnen: ga? i r l small inte.itirlr ancl a h ~ e n c et>Ccccalgas

s h x r l o ~TSE:
. telescoping of ileum i n t o cecum.

Gross PathaZogy Zh~r.ingoperation, thme lavers ar-e seen: entering or inner tube,
rc.ti11ning or middle tube, and sl~eatlror cmter- tube: orltur ~ u h e
innri- and mirldle togetlzur crtllcd int~zs-
rallerl it~tussuscipicr~s;
suscrptum.

Micro Pathology Ischemic necrosis M ~ I F I alcn~ghingof rnucora, producing "red

currant jcll!~" sronls.

Treatment Hvrlt-nstatic (barium) or pncrl~nalic(air) reduction using an

enema; surgical ruduction c)i. refertion ir that fails or is
cont~.aindiciltcdowing t o perfbratinn o r ganFcnc.

Discussion Ninety-five pcrcrn t nf case5 o f i 11tus~usccptionare irliopa~liir

and usuall!. otiginale near the ileocccal ,jurlctio~i.The condition
i s as.ioci;ued with adennvirus infcc~ions,~ . h i c h produce
liyperplasia of Peyer's patches in t l ~ cterminal ireurn, which
s e ~ ~ ase sr7 11irll~sfor i ~ ~ t ~ ~ s s ~ ~ c11~i~p also
t i u seen
r i . with lead
points ( e . ~ .Meckcl
. 's diver~ic~llum, prjlyp5, parasites.
cluplicatinns, I~cmar~gioinas, a n d suture lincs) .

Atlas Link

7 INTUSSUSCEPTION
 ID/CC 1%3 1 -y=al.-oldmalt=complains of pruritus and abdominal pain
that radiates to his back along with significant weight loss
(15 kg) over tTic past 4 months.

HPI H c also status that his urine is dark a n d tIiat his stools are
claycolored (A(;] ~o~.rr;).He admit? to a history of smoking
(60 pxk-ycars) and Ilea\? alcohol u\r ti111 multiple prior houb
of pancreatitis.

PE Cachectic malc; scleral icteru.; (indicate? jaundice);

hepatomeply palpable gallbladder ( C r ~ r r u v c ~ r sSII:Y)
i ~ ' ~ ':~hard
8-cm mass palpahl~in midepigastric reginn.

Labs Markedly elevated direct bilirubin (20 mg/dL); ahscnce of

markcclly elevated alkaline phospharase;
~ ~ r i r l ; ~urr~bili~lvgen;
r.)
rnildlv c1t.vatt.d tri111sarni1iasr.s;normal P T elevated caccinoem-
bryonic antigen (CEA) and CA 19-9.

Imaging CT/US: mass in head of pancreas; dilated intrahepatic bile

ducts. ERCP: abrupt cutoff of main pancreatic duct. LTGI:
ilarrowerl lumen ol'duorlenllm.

Gross Pathology Hitr,rl nodular mass lui th ill-rlefiiied Ircjr-rlers invading parlcreatic
pwenc hmla a n d obsfructing c o m m o n bile duct around Iirad of'
pancreas wit11 Inca! extension and livcr metastasus.

Mino Pathology Pancreatic mass biopsy rrcvcals a pclorlv cliffcrcntiated ductal

adenncarcinoma it1 cl ilslers, secrw ing r n ~ ~ c ar~rl
i n rlen~ecollage-
nmlq r3esmopl;lsiir strnma.

Treatment Surgical pancreat icnr[r~ode~zertomv('M'lit wtr's I'ROCEDL'RE) ;

supportive and palliative care (hiliary
cIlr=lno~hcrapy;
decnmprewinn to relieve-jaundice; celiac plexus block for
pain).

Discussion Chronic galIhlaclder disease, diahetc? mcllitus, 'tiureditar? pan-

rr-e;iriI is, chronic pancreatitis, cigarette smoking, diets high in
meat and rat. xnrl o c c ~ ~ p a t i n nexposure
al s prr-
tn c a r c i n o ~ n arc
disposing factors. Pancreatic carril-loma carries a poor prognosis
(8.5% ;lare already locally inv-nsivt. or-nle~astaticiilthe time of rli-
ngnosis) nild is associated with a mutation in the I<-ras oncogrnu
ancI thc p53 tumor suppressor genc. I:ornplica~ions i t~clode
I~ypt-1-c~qiitahilitv (resulting in migratory thromhophlebitis,
also known as thc Troussear~5ign).

Atlas Link PG-P2-010

? PANCREATIC CARCINOMA
 I DJCC A newhorn fir1 is brought into the genetic.; tlrpal-tment for a
karyotypu study.

HPI She was Imrn or a 45-year-oldmother w h o f c d s t1lat her child i s

developmentally retarded ~vithcharacteristic "mongoloid" facial
features; her pregnancy was rinrvcntful.

PE Generali7erl hypotonia; flattcncd facc and low-set ears:

macroglossia;flattcnrd r~asalbridge a n d e p i c a d d folds; silver-
whitt- spols (In rhe periphrry of irises (BUL~SI I ~ E ~ .;POTS);
. D dngle
transverse palmas crease ( s r ~ r . \ uc : ~ ~ ~) :swidely
i . split fixed S2
{dliu to an alria1 septic deCert).

Labs hr-yotype: 47,XX; bisomy 21.

Imaging KUR: double huhble (rlilatcd stomach and proximal rlonde-

n u m ) clue to duodenal atresia. XR, plain: hypoplastic middlc
arid terminal pl3alang~sor firth digits (.AGKOMICRI,\).

Gross Pathology Rrachycephalir tlrad; small brain with shallow sulci: hvpoplasia
of frontal sinuscs; endstarclial cashic~rrderect.

Treatment Snrgery for c u ~ ~ g ctanlihean tlefects and ctuodet~alatresia:

tr-ai n ing in specialized groups.

Discussion The most common chromosomal disorder, Dorm's syndrome is

m o ~ I'requentlv
t car~seclIJT trixomy 21 ( r l ~ ~toc .no~idisjancrinn);it
is lcss co~nnlonlycaused by rnosdcirm or a Rohertsonian
1 randocatinn. 1 I i 5 associatrd wirh a higher incidence with
advanced maternal age (i~ldic;~tiun for prenatal screening); a
highcr incirlt.ilce of cardiac defects. eqpeciallv cndocardial
cushion dcfccts: ancl a higher incidencc of acute Iymphocytic
lenkemia ancl presenile dementia of Nfieirner's type.

pkq DOWN'S SYNDROME

 ID/CZ A 7-yc.i1rulclbou i s brough to the optnnietrist fnr diminished
visual acuity and requests a prescription Tor cycg4;rsscs.

HPI T h e boy has an ~ ~ n u s r l I~ody

al ~ i l long
haI~it~is h arms and leg?:a
Family hir;toryreveals similar body proportio~lsin or her familv
rncrnhcrs. I-Ic i s r+cFerrrdto his family doctor, who on carcfill
o discloses thi11 at1 uncle died nf a ruptured aortic
c l ~ t e e f r i ing
aneurysm,

PE 'Sa11: long extremities; a r m span greater than hciglrt

(oor.rr 1 IUSTENCIS~ELL~X)
: long, slender Fingers ( m c . 1 r v o n ~ c m ,;~ )
dislocation of lenses ( ~ r r m ~LENr . ~TI^) ; sr\fert- rnyc~pia;inguinal
1ie1.ni;t;high-archrd palate; Aar feel (1,~:s IJL%NUS);aortic diastolic
murmur (anriic insuficiency): funtlel chest due to pectus
rxc;w;ltrim: scoliosis of ~horacicspine.

Labs E klcreased urinarv h ~ m x y p m l i n e .

Imaging C?IR/CT/MR: marked dilatation or a5cenrlin.gaorta. XR. plain:

ihoraciu and lurn bar kyphoscoliosis. Echo: rnim valve prolapse.

Micro Pathology C y d c medial necrosis of anrta may lead to dissection, rupttrre,

aneurysm. or aortic insttfficiency; elastic lung fibers lortllous
and thickened; crnph~semaformation.

Treatment Spinr bracing; uphthalmologic correction; endocartlitis prophy-

li~xis:P-adrenergic blockers;aortic va11-e replaretn~nt.

Discussion A svsternic conneclive tisnie disease characteri7~dhv an

autosomal-dominant pattern aC inheritance, Mnrfan's !iyndi-nrnu
i? dne to a defective chromosome 15 fibrillin gene,a glvcopro-
trin secrcted by fibroblasts that acts as a scaffolding for the
deposition of rlastiri.

Atlas Link F 1' 1 I I IM2-011

fi MARFANfS SYNDROME
 to !lor pediatrician hecause
A 5-year-olcl white frnlale is h~-ought
of h e r , marked weakness, pallor, bone pain. and hleetling from
her nn5e ( T : P I S T . ~ I S ) .

HPI She ha5 a history of progrcssir.ely irlc1,easing farigahiliq :and

rearrent infections over the past few ~ ~ l o n t h s .

PE VS: fwer. PE: marker1 pallor; epistnxis; ecch ymrrtic patches over
skin ; sternal tenderness; slight hrpa~osplet~umegaly with
nontender lymphadenopathy no bigns of meningi lis; IIOI-ma1
Funduscopic exam.

Labs CRC/PHS: nnrmtrcytic, llormochrornic anemia; absolute

lymphocytosis with excess blaqts {> 30%) and neutropenia;
thrombocytopenia. Common acute Iymphoblastic lcukemia
antigen (CALLA) (CD 10) positive;tcmlinal dec)?;y~ransferase
(TDT) positive (marker of irnrnatlire T' anrl B lymphocvtes) on
c n m c marker st~trlics;negai ~ V PInnnmpnt test fix Epstein-R~rr
virus.

Imaging CYR: no lymphaden opatliy.

Gross Pathology Neoplastic infilrr-atinnof lp~nphnodus. spleen, liver, and bone

marrow with loss or normal mchitect~lre.

Micro Pathology Myelaphthisic bone rnwrow (distorted archirect~tresecondary

to spare-occupying Irsions) with l~mphobla~tirinfilmation;
Iynmphoblast~with inconspicuouq nurlenli. condensed tllro-
matin, and scan 1 c v t o p l a ~ ~ .

Treatment Treat infection with a11tihinlics, anemia ~ 5 t hhlood irat~sfi~sionr;,

thrombocytopenia wit I1 pla~eletroncentrations. Remission
induc~ionand ronsnlidation chemotherapy. Coiidder hone
marrow transplant.

Discussion Acute Iyrnphocytic loukcrr~ia(ALL) i s the most common

pediatric neoplasm;i t accounw for 80% of all childhood
Icnkcmias. It c;irries a good prognosis.

Atlas Links

Lq ACUTE LYMPHOC,,, L E U K E M I A (ALL,

 IO/CC A 25year+ld woman prcsunts with high-grade fever,
menorrhagia, and marked weakr~esq.

HPI Over he paqt seve~xlwccks, she has also hacl recurrent

infections.

PE Marked pallor; ~ ~ u l t i p Ipurpuric

e patches over skin;
hepatosplenomegalv; gingival hyperpIasia; sturnal renrlerness;
nt3rmal fundr~scopicand t~clurologiccsarn.

Labs CBC/ I'M: normocytic, nomochl-omic anemia; thrombocytopenia;

composcd riiairlly of myeloblasts and prornyelocytes
leukoc~~osis
(rrnnmnn~ring,early blast cells): neutropenia. Prolongecl FT
' and
m.
Gross %thology Bone ei.nsion clue to marrow expansion;chloroma formation,
~nainlvin skull; spIenurnegaly.

Micro Pathology bTy~lohlastrwith myc~urnonocyl.icrlirrerentiation replace normal

mar row ( m r . ~ o ~ t i r HONF
~ l ~ rMcAKKOW) ; basophilic cytoplasmic
bodies (ALTER RODS) in mvelorytes; peroxidase-positivestains or1
bone marrow a n d gingival biopv,

Treatment Chcmothe~apy:dl-trans retinoic acid in acute promylocytic

Irukemia; bone marrow transplant during firfr remission if
HM-maiched donor availahlr.

Discussion Acu tc mvelogeno~~s leukemia (AWL) ic no1 as common in

children as i s ALL. A1 increased risk is aswciatcd wit11 ioni7ing
radiatinn, henzcnc exposure, Tlow~l's~yllclromu.3 r d cytotnxic
cliemotl~urap~lilic agents.

Atlas Links !TZEZ H-F2-014A, H-P2-014B, R-P2-014C, H-P2-014D

ACUTE MYELOGENOVS LEUKEMIA (AML)

 ID/CC A 12-year-old male presents with high fever; markcd pallor, and
epistaxis; he has a history of recurrent UlUs ancl high-grade
fever that have heen rreatrd with parentcral arlribiotics.

HPI He has also sho~ynmarked weakness over the past 3 months. He

lives in thc vicinitv o f an industrial itnil that handles petroleum
rIisti!lates such as benzene.

PE VS: fever. PE: marked pallor of skin and con,juncriva:nral and

nasal mucoaal petechiae; purpuric patches visible on skin: no
significant Ivmphadenopathy; no hepatosplenamegaly.

Labs CRC/PBS: anemia, neutropenia, and thrombocytopenia

( P A K C ' ~ T ~ ~ P F . N anemia
~,~); with low rrticulocyte cotlnt; nnr~nal
RRC morphoIogy, Normal seri trn bilirubin; negative Conmbs'
test: normal chromosonial sturlies.

Gross Pathotogy Increased ycIlow rnarrow and decreased rr-ct marrow.

Mirro Pathology Hypncellular hone marrow with empty spaces populated by fat
cells, fibrous stroma, and scattered lymphocv~es;markcd
decrease in all cell lines.

Treatment Retnoval of myclotoxin (in this case, benzene); hnne rnarroiv

transplan ~rtion:immunosupprt~ssiveacatmen t with anti-
thyrnoqte globillin : niyeloid growth Fdc tors (e.g.,GM-CSF) fhr-
neulropenia.

Discussion Sixtv-five pel-cent of cascs are idiopathic. -4plastic ancmia

following $rug or toxin exposure may be close dependent
(e-g., hcnzcnt., cytotoxic drugs, radiation) or idiosyncratic
(e.g..chloramphenicol) . Other causrs include viral infection
anrl Fanconi's anemia, an autosomal-rrceqsivedisorder in DNA
repair.

Atlas Link r"lH-P2-015

*
- + ANEMIA-APLASTIC
 -4 ti6-year-olcl white m a n recently diagnosed with chronic
lymphocytic leukemia cornes inlo he errlergency room
complaining or fatigue and ~achyca~dia.

HP I He also states that his urine I-tas been progrt.ssivelv turning dark
and red ovcr the course of the day.

PE VS: mchycarclia, PE: dyspnea; pallor ef skin and mucous mem-

slight jaundice; splenomegdy.
bi.ar1~4;

Labs CBC/PBS: severe anemia; positive Coomhs' test; seticulocytosis;

sphemcv~osis;"'hite cells." LA: lternnglobinuria. Tnci-eased
serum indi I-ecrbilirubin.

Gross Pathology Congestive splmomegidly (due to extravascular hemolysis in Ihc

spleen).

Treatment Prednisone; transfi~sions:sple~~ectoms;immuno~upl>r-es~ive

Efrt~gs.Discontinue any offending drug.

Discussion A4utoin~mune hemolvcic anemia is idiopathic in a b o ~ 1 ~ 5 0of%

cases; it is characteri7erl Ily autoanti bodjcs against RBC
mcn~branes(Rh} , compIemen~activation, and phagoqtosis of
RBCs bv splenic macraphages. Three main types ex is^: warm
antibody (80% tto I)O%: assnciarerl with lelikemia, lytnphorna.
SLE, and \-iyjr;ll infrctions); cold reacting antibody (1 0%;
associated with EBV/rnycoplasma infections and lymphoma) ;
and dn~g-ind~aced (methyldopa, qllinidirle, penicillin).

Atlas Link

9 ANEMIA-AUTOIMMUNE HEMOLYTIC
 ID/CC A 35-year-nld w m a n is admitted to the hospital with left4ded
weakness upon awakening.

HPI Shc has no history of prior hcadachus. scizurrs, hvpcrterision. or

cliabrtus and nuiehcr smrkcs nor lakes drugs. Her first three
pregnancies wcrc spontaneously aborted; the foun-th reculted in
unexpected fetal death.

PE VS: normal. PE-: patienr r o n ~ c i o r l mikt

~ : pallor; left herniplegia
with exaggera~erld e ~ tetl~lor~
p rellexes i u ~ dextensor plantar
rcspunsc ( P O S I T ~ TBmm.sw;l'ssrm) : no nrck rigidity fundtls
normal; n o carotid h r ~ ~nn i ~cardiac
: tn~innnrs;reddish-blue
rnottling o T skin in fishnet pattern ( z.mno RI:~CL~L-\RIS)on
cxtrc~nities; positive I Tomans' sign in left leg.

Labs CRC: rnilcl thrombocrytopenia. Prolonged nor-ma1 bleeding

and clotting times; false-positive VDRL (titel- < I:18): FTA-ABS
for syphilis negative: ELTSA ~horuspresence of anticardiolipin
antibody (ACA).

Imaging CT, t~exrl( 2 4 hours later): hypodcrlrii~( d i ~ to

e infarrt) i11 right
internal capsuIc.

Treatment Anticoagulant therapy 74th heparin: uqe of lowdose aqpirin and

heparin, either alone o r in cornhination wit11 prednisone, is
atlvocated during pregnancy in case5 ~ith a complicated
obstetric history (e.g., SpnntanPnlIC ahortioris 01- intrauterine
clurnisc).

Discussion The prewnce nf lupus anticoagulant and ACA defines

antiphospholipid syndrome; it is li~rthercharacterized by
recurrent deep venous thrombosis in thu lower cxlreniities,
in t l I-endl
~hr.c~rnbosiq ~ and hcpatic veins. pulmonary
hypertension. cerebral artery ocdusion associatccl wit11 strokc
and tranqient ischemic attacks (TI&), and neurologic tinding5
that resrmblr inulti-infarct dementia or epilepy-

* ANTIPHOSPHOLIPID ANTIBODY SYNDROME

 A 9-year-old girl, thc daughtcr of African immigrants, presen w
with a largc swelling of the left side of her face and jaw of
3 weeks' duration.

Trvn weeks i l p , she c o m p h i n e d of loosening of the upper

~ c c r ~ nleftd molar. Duspitc the sizc uf the tumor. there is no pain
a ~ s o r i a t e dwith it.

Rillor; large, firm, ill-clrfinetl maw el~cnmpawinge n tire upper

mandible, prodttcing mild ipsilateral exophthalmos wit11
deformation (311 left side of lace.

Labs CBC/PBS: nurmocytic. normochromic anemia; mild leukope-

nia; posi~iwdirect Cnombs' t a t . Karyotype: chromasomal
~ l ? u ~ s l o c a ~ t(8;14)
ion involving c-myc gene.

Imaging ( X U :nn evidence of mediastinal widrminl: (vs. Hr>dgkin's

Ivmphorna).

Gross Pathology Firm, ill-cletined rumor involving upper mandible ancl

defotmi ng neighhori~ig5trl1chires,b u t no ulceration or
uccl-osis; no satellite adenopathy.

Micro Pathology Giemsa-~tainedFNA show cells of uniform size with iloilgtanulal-

hasnpl~ilicnucIei and some vacuoles, 2 to 5 nucleoli, and evenly
distributed chromatin surrounded by small, ~ h i n rccentric
,
cstoplasm that is pyroninnphilic; high mitotic index and typical
"starry sky" imagc pattern ( d u e to cliff~~re
disrrib~~lion
of
macrophages arnrmg tumor cells).

Treatment 14igt1ilose, short-term chemetherapy: rtlkalini7e ~~rirle,

Cnl-ce
rliuresi%;Ilnrle inarrow rransplantation; inrrathecaI metholrexare
Tor meningeal prophy1;~~is.

Discussion Burkitt's lymphoma is a small n o n c l ~ a ~ elymphonla

d
(non-Hodgkin's lymphoma). It is a pclorly dirferentiatcd &ell
IynphohIastic l y n ~ p h o m aThe . endemic (hfiican) fonn is
c h a ~ ~ c t c r j z eb~rjaxv
d tumors and is assoriared wilh EBV
infection; t h c noncndernic (M1estern) Term i5 characterized I>v
atldominal anrl pelvic involvement. Thc condition was first
drscribed by Denis & u ~ - k ii ~n t1958 in Uganda.

Atlas Link

*
A - BURKITT'S LYMPHOMA
 A 65year-old male visits his ranlily doctor Cnr a rol~tinea11nual
check~tp.

On rlirecterl hisiory, he admits to a weight loss ofahout

12 pounds over the past 4 mnn I hs. togrther with upisucles of
epistaxis and extreme Fatiguc.

PE Generalized ~ ~ o n t c n dIymphadenopathy:
cr pallnr; enlargement
of spleen and liver.

Labs CBCJPBS markedy elevated WBC cormt ( 1 24,000); 90%

lymphocytes; no lymphohlasts; mild rhrornh(~cytopenia;
Coomb+positive hemolytic anemia; smudge cells (fra~;ite
lymphocyt~s).

Imaging

Gross Pathology Lymph node enlat-~ementi~lrno~t

always pr-csmt;
with tumor r-lndule fol-mariotl.
I~cpa~osplcnornepl,?~v

Micro Pathology Bone marrow biopqy reveals es~ensivc.infiltration, mainly by

normnl-lnoking lymphr)cytes and a frw Fymphohlastq M-i th small,
dark, round nuclei and scant rytoplaqnl; liver, s p l r e ~lymph
~,
nnde involvetnenr common: I3 Iymphocvtcs fail to mature
~'"Pc'IIc

Treatment for complic;-ltions

Cf~errlotherapy;prcclnisone or spler~ecrotr~v
such as autniininur~el~crnoly~icancmia or immune thrombocy-
topenia.

Discussion Chronic lymphocytic le~~keinia (CLL) is a malignant neoplastir

disease of R lymphocytes that exprers r h e s~lrfkcemarker CD5
(usually in T lymphocytes); it iq cl~aracterizcdby slow
progression of anc-mia. I~e~noIytic anrmia. recurrent infections,
Iyrrlph node enlargeinen t. and bleeding episodrs.

Atlas Link

C H R O N I C LYMPHOCYTIC L E U K E M I A (CLL)
 A 40-vcar-old white male visits a doctor fur a life ixhsti!~ance
pllvsical examina iioti.

The patient has no majnr complaint5 except for occasional

fatigue (due 10 hyperm~taholicstale) and increasing abdominal
girth (clue to enlarged spleen).

Pallor of skin and inucolis mernl~r;it~es:

markedly enlarged
spleen; pain on palpation over sternum (due to tna rt-ow nverex-
pansion); nr) Ivmpha~Eenopathy:1 1 0 othcr abnormalities found.

Labs CRC/FBS: markedly elevated WBC count ( I 30,000): itnmatore

gr;inuioq~csmixer1 u i ~ hnormal-appearing ones: basophilia;
eosinnphilia; early ~ I i r o m h o ~ ~ olate
~ i st :h r n m l ~ o c ~ ~ o p c n i a .
Low lenkoqte alkaline phosphatase; elcva ted serum vi t anii TI
B , , Icvcl. Lxvoqyt: chromosornnl tratlslocatinn t(9:22)/bcr-abl
gene (PH~L\I~F,I,PFI~
4 I:HKOVOSI)MI:).

Imaging US. zlhdorne~i:splet~oinegaly.

Gross Pathology Skull chlorotnas ( ~ n a l i g n at,~greencolored

l tnnlor arising from
~nvelnidt iqsue) ; enlarged and congested spleen with arcas of
throinhosi~and microinfarcts; hrpatornegaly ( d u e t r> pro1iFera-
lion and infiltrai ion bv grannlove precursors and maturc
granulocytes).

Micro Pathology Hep;~~kc 4tlusoirIaZ Iruk~micinlilrrateq: congestive splenomegalv

1vitl1 rnyeloicl metapbasia; PFliladelphia chromosome in all
myelnid pl-ngeny.

Treatment Hydroxvurea; rw-interferon; leukapherr5is; bone marrow

tran5plantatiun {the crnly potentiafIy curative trealtnent )
T t e a n w n ~ineffective afrpr development of hlast crisis.

Discussion In chronic rnveloge~locisleukemia (CM?,), clearh us~~allvres~~lts

frnrn ac.ceIet;l~edtiansformation into acute leukemia (RUW cnrsrs)
within 2 co 5 yean.

Atlas Links

7 C H R O N I C MYELOCENOUS LEUKEMIA (CML)

 ID/CC A 55-year-old male presents with swelling, pain, and redness of
the right leg.

HPI: He is retired and leads a sedentary lifestyTe. Hc admits to a

70-pack-yeas s m o k i ~ ~history
g and occasioi~alalcoIiol in take.

PE 1's: fever (38.4"C:) ; tachycardia (HR 106); mild hyperterhsic~n

(BP 142/92); notrnal Rn. PE: right lower extremity swollen;
pain eIicited on calf palpation and on dorsiflerbon of right foot
( H O ~ LS' YSICY) .

Labs Blood D-dimer elevated.

Imaging US. Doppler: thrombi occluding right cornmon Femoral and

popliteal veins. Venography: <goldstandard for diaqnosis, but
rarely indicated.

Treatment Anticoagulation u.i LII TV heparin, Cnlln~vedl ~ long-term

y
an~icoagulationwit11 oral warfarin or subcutancuiis low-
mol~cutar-weightheparin.

Discussion S~ircliow>triad (venouq staslis, vessel wall injury, and

hypercoagulabIe state) contributes to the furmation of vrnous
thrombi. C:omplications of DVT inclttdc ptllrnonarv embolism
and venous ulceration. and insufficiency. Appl-nximately 200,000
deaths per year in [he Unitecl Statcs arc attrill~itahleto pul-

*< DEEP VENOUS THROMBOSIS

 ,I 25vea1--oIdwhite fernale continues to bleed sreadilv after a
nartnal, spontaneous vaginal delivery.

Mx~iualexploration ofthe uterus iox~ealssetairred placental

iis.;lie that requires rlilatation anrl curettage; 50 minz~tcsafter
thr procedure, the patient hegin5 tn bleed prafwely from her
gums and conritlues to b l e d vaginally.

Diffuse hlceding in gums and oral rnucosa; bleeding diathesis

of skin (huth petcchiae and purpura) with oozing from
venipuncture sites.

Labs Low fibrinogen. CIIC: low platelet count. Prolonged PT and

activated PTT; elevated fibrin split prodtlcts, csprcially
Ddimcrs.

Cross Pathology May see ctxnplicaltions such as renal cortical necroqis, limb
~hrornhosiswith pang-cne, and ischemic adrrnal necrosis.

Micro Pathology Microthrombi in astl-tioles and capillaries, l e a d i t l ~to

microinfarcts in practically anv organ : also hemorrhages and
pctcchiae in ini~olverlorgans.

Treatment Treat underl$ng disorder; fresh fyo~enplasma: fihritlogen

cl-~opretipitare:platel~rs;arninocaprnir acid with heparin.

Discussion r)issernina~edii~travasc~ila~- coagulation (DIC) is a Illeeding

disorrler that is clue to cons~~mptinn of plateletc, fibrin, and
cr>aplatio~l, factors secondary to excessive clotting in
n i i t : r o c i r ~ c ~ ~ i aI t~isi (precipitated
~~~. b y cancer, parn-negative
septicemia, burns, mu ltiplr trauma, and abstetric complications.

DISSEMINATED INTRAVASCULAR COAGULATION (DIC)

 ID/CC : 35-year-old man corn plains of pain in his calf rn~lscles
I while
walking that is rclieved by rest (INI+.I<UII'I.I?N.I C I A I~UICATILIN)
with exertional rIlest pain.
tc~gett~rr

HPI Ilcl~asafan~iEyhistoryofpremah~reatheroscleroticcoronary
artery disease (CAD).

PE 15: mild hypertension. PE: obese; palrnar xanthomas and

tendon xar~tl~omas:orange-yellow discoloration of palmar
creases (pathognomonic for dysbetatipoproteinemia);
tuhoeruptive xanthomas on pressure sites (elbows. buttocks,
;~nclknees) ; wuak peripheral pulses.

Labs LFTs normal ; lipid profile reveal5 elevated total cholesterol,

triglycerides, and VLDL and reduced LDL and I-FX)L,chylomi-
plasma; clec~t-ophor-esis
crori remrlaills present i11 f'ds~i11g reveals
beta migrating VLDL:isoelecrric t'ocuqing shows ETI/ETI
genotype (nearly pa~hognoitini~ic) .
Imaging ?Ingio, coronary: arherr~sclcroticcoronary artery diwa.;e
confi~-med.
0
GI
<
Gross Pathology Yellowish in tralutnin a1 atherosclerotic plaques sccn in tlze aorm -
0
,
and rx her h r g e V P S S ~ ~ F . z
r)
0
r
Micro Pathollogy CIiaractcristic atherosclerotic plaques. o
G)
<
Treatment Weight reduction t o ideal body weight, regular exercise, avoid-
ance of nlcohol and other trig1yccricle-raising drugs; low-fat,
Irw-cl~oleskrolldiet; in resis~nn1 cases, gernfibrozil, high-dose
nicotinic acid (niacin), and HMG-CoA redudaqe it1 hi hi tom
(statin drugs) may he nsed.

Discussion @sbetalipoprotein~'mia (TYPE rIr I ~ E R L I P O P R O T E ~ E ~ Mis)

clef ned as the prcsunce of VLDL particles that migrate to the
hela position on electrophoresis (normal \%DL particles typi-
ral ty migrate to rhe pre-hpta location}. Rela-WADI,particle5 are
chylomicrons ancl VLDL remilants caused in part by a mutant
apo E rhal impairs the Iiepatic llptake of apoproteitl-Ecnntaining
lipoprnleins (Vl,f)l, a n d c hy/lotnicroilf).

7 DXSBETALIPOPROTEINEMIA
 ID/CC A 61-vrar-old white male presents with marker1 weakness,
gingival bleeding, and an abdominal mass.

HPI He has a history of'recurrent bacterial infections and has no1

u-aveleil nuuide thp United States.

PE Pallor; marked splenomegdy: mild hcplziurnegaly: no I p -

phadenopathy, icterus, or iiscites.

Labs CRI: JPBS:anemia; decreased WBCs and platelets

(~.~NCYTOPENI,\);lymphocytes with characteristic long, thin
cytoplasmic prqjections ("I 3.41RY CELLS").

Imaging CXR:normal. CT/US. ahdompn: massive splenomegaly; mild

hepatomegalv; n o Iymphadunopathv; no evidence of portal
hvpcrtension.

Gross Pathology Liver; spleen. and hone tnarrnw infiltt-aterl Ily leukemic cells;
~pler~onlegaly nrav he significarlt.

Micro Pathology &one marrow largely repIaced by leukemic cells ( M +TIOPMTI-IISIC

ROVE MARROW); large proportinn are hairv cells and contain
tartrate-resistant acid phosphatasc (TRAP): splenic biopsy
rrvtxls Ieukerrlic in fill ration of red pulp by hairy cells.

Treatment Dcoxvcoforrnycin and =-inturforon are highlv effective;

splenectomy.

Discussion Hairy cell lc-ukcmia is a chronic B-cell malignancv; atitoitnmune

svnclrr)mes are frequently seen, inclirrling vasc~lliiisand arthritis.
I t i f alqo characrerized hv atypical rnycobacterial infections.

Atlas Link DTTZ H-P2-024

7. ? H A I R Y CELL LEUKEMIA
 ID/CC An 8-yearald white male presents with an rr-ytheinatous skin
rash over the buttocks and legs coupled with joint pains,
abdominal pain. ancl hematuria.

HPI Threc days before h e had complained of cough, coryta,

low-grde fever, and sore ihroat. He has a history of allergy to
dilsl and pollen.

PE VS: hyprrtension. PE: palpable pwpuric skin lesions o.~.cr

buttocks and legs; painful restriction of knee and ankle join1
movement with swelling.

Labs CBC: normal platelet count; normal coaplatioll tests. Increased

ESR increased BUN and serum creatininc. UA. RECs and RBC
casts;on urinarv serliment. Positive stool guaiac test (due to
orcaI t blnnrl).

Cross Pathology Nec~mtizingvascuIitis of k i d n e y and lungs. x OkAA

rn
2
Micro Pathology Renal biopsy s l ~ o ~ Ibcal
vs and seg~netlta!glomerulonephritis 3
~ vth
i crescents (11lesangioproliCerative); mesangfd IgA deposits o
r
0
on immunofluoresclmcc. G)

Treatment St~ppotlive;s~eroicls;high-dose irn1n\rnnglcrhu9i11 I herap7

U
GI
Discussion HennchSchrinleitl pul-prira ir: a generally selr-lin~ired.irliopathic
disotcler that is also known as anaphylactoid or vascuIar
~ j ~ t r p u rita ;i s a common vasculitis (s~nallvessel) in children.

Atlas Links FEPP PED-019A.PED-019B

7 HENOCH-SCHONLEIN PURPURA
 ID/CC ..1Gyear-old male is brought to a specialist bv his parents clue to
persis~rnrpain and tenderness on the right side of his chest of a
few months' duration.

HPI Therr i q no history o f tmurna to the affected area. The child is

otherrvise well and is growing normallv.

PE Exquisitely teilrlur si~ef o l ~ n doverlying ff~utthrib on right irk

anterir~rly:remainder of exam ~mrumarkable.

Labs Ro1rrin.c la11 przrameters normal.

Imaging CkT: punched+ut lesion in foul-111rib otl Tight sicle.

Gmss Pathology Intrarncdullary expanding, eroding lesion.

Micro Pathology Brownish granulation tissue containing abundant foamy

histiocytes ant1 eosinophils with leukoryte5 and giant cells.

Treatment Lcsions rcsolvc span taneouslv; surgical curetvage mav accelerate

healing.

Discussion F.ocinnphilic granuloma i q x rvpr of l a n g ~ r h a n ' cell

; histioqtosis;
it is an inclolcnt disorcler that affccts cliilclren and young adults,
espe~iiillvmales. Solitary Imne leqicltl.: may be asymptomatic or
rnnv c a m p pain and tenderness and, in w m e instances, pathn-
logic fixture. h ~t twithout ailv systemic rnanfisla~ions.
Diagnosis i s IxtserI on radiopaphic dernonsfrariot-i ol'a localized
destr~~ctive lesinil arising Crom inside the marrow cavity. The
skull, mandible, and spine are cornrnon locariotls. I n some cases
tt~el-emay he spot-lmnenushealing nl- lihrosir, within a period of
1 to 2 veal-s. The disease mav xlqo he multifocal. involving the

* HISTIOCYTOSIS X - E O S I N O P H I L I C GRANULOMA
 ID/CC A 2-yea~rildboy is hrought in for a pediatric cons~~ltatirin
because h i 5 parents are concerned ahout the child's protruding
eyes (EXCJPAI'HA I .VOS) and excewive urine volume I~OL~YURIA).

HPI The parents also state that the child has hcrn febrile and has
harl multiple rat- infections.

PE Low weight for age; bilateral exnphthnlmns: painful swellings

over head (due to cystic bony Iesions) ; no icterus; no
lymphadenopathy; mild hepatosplenomcgaly.

Labs CBC: normal blood counts. lncreaqed serum osmolality;

decreased urine osmolality.

Imaging XR, skl~ll:multiple rounded lytic lesions.

Micro Pathology Bone Iliopsy Ft-orn skull lesions sllow gr~nulnrnatourlesions and
characteristic Langerhans cells with coffc-c-bcan-shapednucIcj
and pale, ahundant cytoplasrti: tennis-racket-shaped tubular
structures ( ~ R R I < C aK w r ILKS) nn elec trnn micl-o~copy;positive
$1 OO protrin and CD 1 antigen.

Treatment Cornhiua~iotlchemotherapy, ci ire1 lase of bony le\ic>nq.

Discussion A type of Langerhan's cell histiocytosis, I-Tancl-5chiiller-C:hristian

syndrome is nlultifocal, producing diabetes insipid~~s d u e to thc
involvement of [ h e hyporhala~nusand exophtl~;+lmc~s trorn
orbital infiltration by histiocyies.
 h 2-yearald wl~ilemale child is seen with complaints of fever
fc~llrrwedkv a &ffuse skin m h .

The chiEd wxs apparently/ we tl a month ago, Imrn aftrr an un-

cumplicated pregnancy and delivery

17% t;lchl;c;~rctia;fever. PE: mild pallor; otc>scopyof left ear

reveals cl~ill,poarlv n ~ o l > itympanic l~ membrane with pus behind
i t ([I rrrrs ~ I I : I ) ~ A;) generalized Ivmphaclenopathy:
hepa tosplenomegalv; diffuse maculopapular c8czematoussash.

Labs CRC: ane~nia:thrombocytopenia ~ vth i Ielrkopen ia

(PIN(:YI.OPKNI.Z):t-rlative eosinophilia.

lmaging CT. abdomen: he pato splen omeraly. X R cystic, rarefied lesions

on skliZI and pelvis.

Gross Pathology Skin shows prese~lcc~Textensiveeczematoid rash; large destrtlc-

tivr hone Icsiuns found on sku11 and pelvis.

Micro Pathology Eosinophilic grantdomatous lesions in aIl involved organs;EM

5h o w cal Langerhans cells with characteristic Birbeck
granules; t hcse cr llr: were further fbund to I>eH LA-DK-pa9itir.e
and expreqsing CDI antigen.

Treatment Corticosteroids; chemotherapy; surgery or radiotI~erapyfor

lnralirerl h ( n n disease.
~

Discussion Lrttcrer-Siwc disrasc is an acutc o r subacute clinical syndrome

of unknown ctiolop affucting children less that1 3 years old. It is
marked by revet- drw to Incalized infection followed by a rliff~lse
m;~r+ulopap~~lar ec/ernatous purplric skin rash and s~~hsequent
l~epatosplenomegalanrl generalized lymphadenopa~hy,It
shows sinlilari tics to acutc leukemia and r~thrrinfectious
processes, n i a h e t ~ sinsipidlts, exophth;lIrnos, and hone lesions
are ii~uallvwen in conrhinatiot~.

1"1 HISTIOCYTOSIS X-LETTERER-SIW E DISEASE

 ID/CC ,A 24year-old white male complaiiis of rapid enlargement of his
abdomen, producing a dragging crnmuon, along wit11 a painless
lrmp in his neck fnr lhe p ~ q 2t months.

HPI The palien1 also coinplains of iiltcrmittei~t fever, drrnching

night sweats. pruritus. ancl significant weight loss.

PE Paltor: unilateral nonterrder, rubbery, enlarged cervical lymph

nodes; splenomegaly; no enlargement of tonsils.

Labs CRC/PRS: neritrop hilic leukocytosix ~ i t 1'1t1npliopenia;

h normu-
cyric anemia. Elevatccl ESR: elevn~edserllm coppcr and ferri tin ;
ncgativu Man toux e\t.

Imaging CXR: bilateral hilar lymphadenopathy

GTOSS Pathology Itlvolvud Ivlnph nodes arc ruhherv and have "crlt-potato9
appearance of cut surf'acr. 1
rn
3
Micro Pathology I.!mph norlc biopsy shorrs large h i s ~ i o c y tceIls
nr~cIciancl eosinophilic 1111rleotus
~ with multiInhed
resembling owl's eyes 5
r
0
(REF.~-S~T.RNRFH~;I .t. i 1: 5 ) ; nn honc marrow in~olvemen t on bone m
marron biopsv.
<
\
a
z
r)
Treatment Radiotherapy and cl~etnotherapy. -
o
b
I 3
Discussion Four patterns of Ilodgkin's discasc are seen on lymph nodc <
biopsy: lyrnphocytic predotnir~ar~ce 5% to 10%: nodular sclerosis
65% to 75% (seen 11-eqnentlv in voung {wmexl): mixed cellularity
20% to 305%;and Iympl~ocvtedcpleterl 10%.Prognosis worsens
in this ordcr. Ann Arbor staging 1-11' with ~uhclassificatiunA
{no cclrlsti tl~tionalsynlp tom%)a ~ i r13
l (weight loss, fcver, nigh1
sweatu) !nos1 acrul-ately predicts prognosis. The disease spreads
to contigtrouq lymph nodes before heinatogcnous diaserrlir~a~iot~.

Atlas Links O 1!TT R-P2-029 DI7E JMI-049

.a'< HHOGKKIN'S LYMPHOMA

 ID/CC X 3-year-old white f ~ m a l ci~ hrougllt lo the ~rnel-grncy
room
with :I skin rash and severe epistaxis.

HPI T h c patien1 Zlad a LRI c o i l r i ~ t i n gof a srvverc c o u ~ hand a riiilny

norc 1 0 davs before the onset of her symptoms. S h e has no
prir~t11 irtory ot'prolonged bleeding follnrving mininlal trzltlma.

PE Mucosal petechiae; episaaxiq; hemorrhagic budlae in buccal

: p l c ~ 'n1o1
~ I I U C I I S ~~ nl~alpahlc.

Labs CRC: mild anemia: low platelet count ( I I1.000); RBCs and W C s
normal. P~.olnngt=clI>leedingrime; n o r n ~ a PTT;
l nt)rmitl PT.

Gross Pathology Purpura (tluc- In r ~ t r a v a ~ ~ l iocbt rlllor>d

~ froin inu-ava~cuIarspace
in 1r1qkin) : piii-~i7erlhemol-1-h:lges (I~I?:;:~J.:CII 1 , ~ ) : eccl~vmosis
( l a l - g ~rrhxn pui-p~lrx).

Micro Pathology Nc r r r n ~ ~hoilr

l marrow aspir;t!e with increased number of
m~gakaryocytes.

Treatment Prcclnisnnc; splrucctc-rmy;WIG

Discu5sion I t l i r )1>i11hi< I 11 r.ot~~l)orvtr,pPniT

~ ~ ~ I - P I(II'P)
I ~ X is a n a u t o b ~ ~ n e
r l i w ; ~ s r . 111 h>r.?n;ltiunof TgG antiplatelet antibodies anrl sul>5e-
qucn1 pIatrIt.1 deqtr uclion in 11iespleen. 1r often folfows a viral
inrection all<!i s srlf~limiterlin r.hilclren h ~ r chronic t i r l adults.
 IO/CC A 64-year-old blztck m;dr 1;11ffttr-rfrr)iii hone pain,weig11 t luss,
nnri casy Fatigability.

HPI I I c aIsc>co~nplains11f reamrent WRrs and frequent nosebleeds.

PE Pallur: bone tenderness in Ir~werback and rihr: petechiae on

huccal mucora: n o hepatospl~nomegalv.

Labs CRC JPBS: narrnocytic, normochrnic anemia: ~ ~ e u t r o p ia;

en
rouleau formation (KBCs adhering together like >tackof poker
cliipq). Elevated serum calci~un;normal a1kaline pl~osphatase:
mal.kedly increased ESR; gamma spike on serum protein
electrophoresis (monoclonal gammopathy) . LTk RenceJones
pmteinuria (clue to IgG light chains).

Imaging XR. plain: punched-out, Epic bone lesions in vertchrae, long

bones, and skull (xuial skrlcton).

GTOSS Pathology Multifocal replaccrnu~itof norrr~albone tiwue rvii h tumor cells

(~dasmacyiorna):prlvi~,skull, and spine most affuctrcl.

Micro Pathology Infiltration of bonc n~arrcnt~ l ~ v11osrnal-lookingplasma cells

(al~undan t cytopIasm. eccr-ntric nuclei) in ~tggregates;amyloid
deposits in kidney with re1131 ~ ~ ~ l - ncast
~ l ahrtnation
r and intcrsti-
tial fibrosis (can causc renal insufficiency);b o i ~ rerosion and
dc.?itruction of curtical hime.

Treatment Cliemoth~r-apeutir I-egimen; hvrlu~rion;treat hypcrcalccmia and

Ilvperuriccrnia. Considcr palliative rdrli;-ltioirtherapy.

Discussion MultipIr mvrluma is a primary mdignancy of plasma cells ~i~ljth

I-rplacemenz of nurmal bvne tnarrow; i t is [lie most cninmon
primal-v brine carlrei: The l~rognosisworsens with anemia, renal
failurc. and mr~ltiplelptic lrsior~s.

Atlas Links H-P2-0314H-P2-03 1B

"*1
I ' T MULTIPLE MYELOMA
 ,4 54-year-old white male complairw of easy fatigability, shortness
of t>reatl~.headache, and ligh~l~eaderlnessover l h e coursc of
aftnost onc ycar, with increasing scvcriy.

Eie has also noticed a feeling of heavineqs in his ahdornen and

increasing girth a? well as recurrenl deep p i n in the legs and
occavic~r~allv
in the uppcr abdomen.

Massive splenomeply: en larger1 liver; mudera~eamount of

aqcitic fluid; multiple petechjae on thorzcu and extremities; no
lymphadenopathy (one cliffrrential f r a tl~r-e~ l ~ a r ewith
d cllronic
mvelog~r~rnis leukemia).

Labs CBCJPBS: ancmia (Hb '7.2) ; low hematocrit; anemia;

immaturr. 1.lrBC1;and r~nrmohlitstssrc11siinril tat~eously
(~.EL~I~~F,~Z~THR~ I ~ I . ~ Steardrop-shaped
SMEAR); ~I~IC: FSiCs; giant
abnot.ma1 platclcts.

Imaging XK, plain: dcrise hones (generalized os~eosclerosis)

Gross Pathology Extramedllllsuy hematopoiesis, whiclr is prorninen t in liver and

~pEern,with sig~lifica~i
t increase in size and weight together will1
firm consistel~cy.

Mime Pathology Wry tap" on bone marrow biopsy: l-i>pcellularhone Inarrow

early in diseasc) ; significant increase in number
(h~-perc~llular
of ~negakar-yocytes;replaccmcn t of mar row cissur will1 fibrosis
(positive re~iculinon silver stair^): preservation of normal archi-
I I spleen.
tt'c(111.e ~

Treatment

Discussion Also callud ;ignogeiiic ~nveloidrnetitplasia. tnvelofibrosis wilh

rnyeloirl metaplasia is an idioparhic canclitiori in which in-
' ~ o f platelet-clerivecl growh factor (PDGF) and
creasrcl S C L ~ Lion
TGF-13 catrw%replacement of hone marrow tissue with fibrosis.

Atlas Links

7 MYELOFIBROSIS W I T H M Y E L O I D METAPLASIA
 IOJCC A 5Jyear-old whitc male notice5 painless I~tmpsPlilaternllv in his
neck h a t haw slowlv enlarger1 nvcr the past 3 nionrli%.

HRI AItho11g.h he clunics any pain, he ad~niwto havit~g;episodes of

mild fever, night sweats. and some weight loss over this period.

PE Bilatuml cervicill firm lymphadenopathy; pallor; ~pIenornttgaly,

Labs CBC: C;oombsrposiuve hemolytic anemia; t l i i . o ! n b n c ~ ~ n p ~ ~ ~ i a .

Elevated senm LDH (a uscfril ptopu.;! ic marker) : tlyp.pogamma-
glohulinrmia.

Imaging CT/lJS: I\mphacienopathy: splcr~umc.galv.

Gross Pathology Lymph nocles havc grayish hue o n outside and "cut-potato"
appe;lr<lxiceof cut sur-f'ac~.

Micro Pathology LvmpIl ilode biopw dcmunstrates notl~xlar.(~t~ell-dirferentiatecl) I

or diffuse-9pu (poorly clifikr-entjared) ly mphocctic Iymphoma; rn
3
histiocytiu and strrn cell lvm~>homn. 3
2
Treatment AlkyTaril~gagents in varior~scombination^; r-xrliotl~~rapy
if b
G)
-i
localizrd: bone marrow ~ransplantarion. \
0
2
r)
Discussion Primary maIignan t nroplasms of l~rnphocy~er: arise in IyrnpEloirl o
r
tiss11eanywhere in the hodv; thev occur mainly in lymph r~cldrs w
I 3
h m may involve inu-a-ahclominrrl organs and bone marrow. The <
prognosis is more dcpundcnt un g a d c tIlar~o n stage. Follicular
, s the 111r1stcoltlrnon form and are associ-
(B-ccll) I ~ m p l ~ o m aare
ated wi 1111 (14; 18) of 13cF-2 (an anti-apoptosis proruin). HTCT
patients h a w a highcr incidence of 11011-Hodqkin'5lylnphoina.

r"C1 NON-HODGKIN'S LYMPHOMA

 A 62-year-old~lrwislimale visits his fxrnilv doclor because o f
epistaxis, Eieadaclie, and dizziness.

The p a t i ~ n chnd black. tarry stools {VT:LI,.V.Z) "months ago and

M a s pi-rviuu.;lv admitted tu thr Ilr)spi la1 L'ni deep venous
thrombosis. Hc alcn drscri hes episndes or severe generalized
U crs) , primarily after showering.
itching ( I V ~ urr

PE VS:hypertenqion ( I%P1 70/ 100). PE: ahese and plethoric; mild

cngoraud, tortuous retinal w i n s with dark rerl hue on
cy~nc~sis:
( i i i ~ d ~ ~ ~palpable
c ~ p y : spleen.

Labs CBC: markedly increased RBC count, hemoglobin ievel, and

hematocrit: C1.TRC.;;md plat elel.; ;114<1 increawd. Normal Pn?,
Pc.o,. i n ~ dP-T: i~lc,r~aved
viramin F5, IeveIs: increased leukocyte
alkali~~e p hnsp hatafe; i ncrpawcl Feruln n n d urine uric aoicl
Ir.r.t=lq:demaqed erythmpoietin level ( c l i s t i r ~ ~ i i ~ l l c s
pulvcvthcmia vcrit from sccunciary p o l ~ ( ~ y h e ~ n i i ~ ) .

Gross Pathology Increased blood volume and viscosity (RBC s l u d e g and

t h l - o m l > i rol-mation
~~ n ~ a i n l yin licart alld h r a i ~ i )sttbr~orrr~al
;
plrltplpt !'unction (bleeding tcnrlcncy) : increast,d frequrn? af
peptic 111c~f.;1tir~ii.

Micro Pathology Bone Iniwrow I)inpsv SIIOM'S increase in erythroid series

precursors i11lr1, to a Irssei. exreti t, in ~rlegakaryocytesand WBC
precursors: t h r o ~ ~ ~ h us
forrnacion with microinfi~rctsill brain and
hrart; myelr~lihr~osi.; lnav t-nsue with cllaracteristic Iindings.

Treatment Phlebotomy: hylrnxvllrea; treat I~!-per~~riccrnia

Discussion Polyc! 111~1ni;l

i q rharilcirrijled hv increase i 11 FSC: mass \zrith
ittl

incrrasecl bloocl vul~~rnc and r . i s c o s i ~i:t nlay he primill-y

(pnlvcyiliemia vera) or q~cnnrS;u-y(due ra COPD, smoking.
011esi 0; eic.) . PC\' may pl-ng1-erqr o chi-onic n l v e l o g c i ~ o ~ ~ s
Icu kcmia. myclofihrosis. or acutc myulogrnou~luu kenria,

Atlas Link

* POLYCYTHEMIA V E R A (PCV)
 ID/CC A 4vear-nld fernale i s t>i-o(~ghl t>y her mother tn the pediamic
cl i nit afrer qlip (iti~lsblood and a "lump" in the child's vagina.

HPI The child's fathcr died of brain cancur. arid her rnother i s
rccriw ng treamwnl for b r ~ a scancer.
i Hrt- granrlfarhrr d i d of
rneiastaiic col(lr.ectal cancer,

PE Pelvic w a r n reveals ulcerated, polypoid, grape-like mass arising

from wall of vagina.

Labs Routinc Iah u-ark on urinc. I>luocl,ancl stool yields tlo pathologic
findings.

Gross Pathology Bulkv himor mass with mu1tilohecl papillary prqjections resem-
bling mass of q a p e s .

Micro Pathology Rinpw of trltnor tnasl; s l ~ c >desrnin-

~ ~ s and myoglobin-positive
(muscle ttlmor) , elnngated rhabdomyohlasts with largr. T
eosinophilic cytoplasm ancl cross-striations. 2
3
Treatment Sur.gical rrscc ti011 ~ i t :jc!jlivar~t
h c hernother-ap): radin~hel-apy. o
r
0
-
G1
<
Discussion Sarcoma hotrynidcs is a polvpoidal: subtype uf embryonal ---.
0
rhabdomyosarcoma that characteristically protrudes like a mass z
(7
of' graperi fi-om the cagina or kladrier.:i t i s [he mosl coin m o n 2
sarcoma in children. 12habrlan~vosarcorl~as m e aften found in o
m
"cancer families'' (e.g.. Li-Fra~~rriei~i5yndr.orne). <

2 ", SARCOMA BOTRYOIIIES

 A 10-year-old black child prewntq with a c l ~ r n n i cnonhe-
l c e r otl hi5 lower leg.

He hac had recurrent episodes of abdominal and chest pain

(due LC) micl-ovascttlar ocrl~ision)along with diminution of
vision. His tnar ernal cousin s~lffct-sTi-om a hloort disorder.

VS: fcvcr. PS: pallor: mild ictena: f~mtluscopyrevealq hypoxic

spots with neovascularization ( "SF;\ F:ws''); non hcaling c hrorlic
r~lccro n left luwcr*leg.

Labs CBC/PES: rlecreasecl hematocrit; megalohlastic anemia: sickle-

shaped N C s ; HoweM-Jolly bodies and Cabot ring; sickling o f
R&Cs on sodimn metakis~ilfitt.peripheral film (Sickledex prep).
Serwrn bilirubin rnoderalply eletraied:q1iantirath.e hemoglobin
eleccrnpl~oresi~shows 85% HbS. LIA: micsos~opichtmaturia.

Imaging CT/ US, abdon~un:srndl, calcified spleen.

Treatment I.ocal ~ h e r a p vfor leg ulcer: laser tlierapv for proliferative

rctinopathy; anti biotic prophylaxis against capsulated bacresia;
hydroqwrea may heIp irlcrcase lktal hemoglobin levelq.

Discussion Sickle celI anemia is rai~serlby a point mutation on the gene

totIing for the P chain uf l ~ c m r ~ ~ l u li~~show?;
i n : autosomal-
recessive inheritance. Gl~~mtnic acid is ar h s ~tuted
i bv valine nt
positinn ti. leading to chronic henlolytic ancmia. In the reduced
form. FIhS Corills poljmers that damagc the RBC membrane.
Fatturs that hastun sic kling i n ~ l ~ t dacirlosi.i
r and hyoxemia.
Prcni~taIdiagnosis is ax.;rilal~le
for at-risk fctl~scs.

Atlas Links

fl SICKLE CELL A N E M I A
 IDJCC An I I-month+ld malc presents with ntarked pallor, failure to
thrive, and delayed developmental motor milestones.

HPI TIIC child'^ parents are Indian immigrant<.

PE Marked pallor; mild ictcrus; frontill bossing and maxillary

hypertrophy ( ' ' ( : ~ I I P ~ I L ~FN.K~ C I E S:~splenomegdy.
)

Labs CBC: severc microcvtic, hypnchrcrmic anemia with anisopoikilo-

cytosis; dccrpaqed 1-erirulocvtosis.HbA absent; HbF 95%; mildly
increaserl unmnjupated bilirubin.

Imaging XR, skull (lalural): maxillary cwrrgrowth and wider~ingof

diploic spaces with "hair on end" appearance o f ff-nntal hone,
causecl hv vertical trabeculac.

Gross Pathology Expansion of hcmatvpoietic hone nlarrow, causing thillrling of

cortic-dl bborle or new bone formation.

Micro Pathology R ~ r marrow

l incrcascd; yeIlow 1nar1-owd e c r e a ~ ~ marked
d:
erytl~roidhyerplasia in marrow (ineffective erythropoiosis)

Treatment Rlood kanqfusion, folic acid supplement, iron chelation therapy

with desferriosamine to reverse hemosirlernsis, and bone
marrow transplantation using HLi-matched sibling dnnc~rr.
0
0
Discus5ion Beta-thalasscmia resulh from cl~cr-easeclsvntl~esisof fl-globin <
chaitiu rltw to errors in the ixanscription,splicii~gor translation
or mRNA. hlphx-thalasscmia results frorn decreased synthesis of
a-glohin cIlaii~sclue to delrtior~oi'onp or rnore or the four
rw genes that are notmally present.

^ I THALASSEMIA-BETA
 A "Lyearold white female cliagnosed 2 years ago as HIV positive
to the Plnrrgency mom hy her husband becausc of
i s 1>1.1>11ght
tachycardia, shortness of breath. hcadachr, intermittent
disorientation,at-sd aphasia.

Shc had started propht;lacdc TMP-SMX 3 weeks ago. On thc

previous day. she had finished her rnenqtrual period, which was
~ had lasted [or 7 rtavs. Her hnrband also points
a b ~ t n d a nanrl
our a generalized red rash all over her bodv.

PE I T S :ti~chvcardia:fever. PF,: pale skin and mlrrous membranes;

confusion ant1 apathy with lucid periods; petechiae on chest and
exrr~lni t ips; pnri tive Bahinski's sign.

Labs CBC/PBS: rnicroangiopathic hemolytic anemia {Hh 7.2) wit11

s ~ rking
i retinhocytosk ai~clfragmented RBCs (sr.~~sromms) ;
low platelet munt (50,000): negative Coninhs' test. Elevated
indirrct I~iliruhin(3.5).LJA: hrmatutia. Absent haptoglobin
(due lo in~ravascc~lar.
hemolysis); normal coagulation tests;
elevated LDH.

Gross Pathology Tllrnmh~tsformation i11 scveral o r p n s with platelet rlepletitln

and rr~ici-oangiopathichetnnlytic anemia: kiclney, brain, and
heart most aflectrd by thrombosis.

Micro Pathology Multiplc h y l i n r thrombi iri brain, rnyr>cardil~m,

renal cortex,
arll.enals, and pancreas.

Treatment Plasrnapheresis and fresh fro7en plasma e ~ r h a n g eprednisone;

;
splenectomy.

Discussion AZsu known as Musc hcowil/'s yi~drorne.r hromhotic tllroml~ocy-

topellit purpum ( n P ) is an idiopathic disease Found in
pregnant and W-positive pal ien ts anrl Ihllowing c~posurrto
drug5 s i ~ as
h antibiotics and estrogens.

THROMBOTIC THROMBOCYTOPENIC PURPURA

 ID/tC 18-year-old hospitalized inale cornplains of fever, nausea,
:\I]

vomiting, and chest pain fuIlowir~gi\ I~lotldtransfi~sion.

WPI He was invoIved in a rnc.>torrycleaccident and 1 . ~ rushccl

~ 5 to the
emcrgcncy room, where he received five units of blood before
heir18 taken tn the O R for repair of a ruphircrl spleen and liver.

PE VS: fever. PS: no hepatosplenorne~alvor lymphadenopathy;

sut.gicxl lapai-otomy worirld unr-emarkable.

Labs Positive Coombs' tcst (indicating a ~toan

r til~odiesto RBCs) ;
decreased serum haptoglobin: elevated inrlircct bilircil~in;
cola-cnIored urine (due to hen~oglr)hi t~uria).

Treatment hydrc-
Hyclratiorr: tbr-ce diuresis with martrlito! or r~~roscmide;
corlisnne: alkalinizt. urine wit11 HCO,.

Discussion ~ZCLIIPl l e r n ~ l ~tirci ~ n $ h i s i ~t.eaction

)~~ may he the requlc of cem-
plete complemt=ntactivation: most c o ~ ~ ~ m o it n lisya result of
mismatched blood, producing intravasclar hemolysis. I t severe,
rorlal shuidnwn or disseminated intrawscular coagiilarion (DICE

TRANSFU5ION REACTION-ACUTE HEMOLYTIC

 IO/CC During the administration of a blood transfusion. a '$5-ymr-uld
male I.)rcsorits zvi t l l fever, headache, and facial flushing.

HPI An hnun- larer h p cI~vt=lnpsfrank rigors. HP lias resrived several

transfusions in the p m , all of wh ich were unevcntf~~l.Thc last
oiit W,IS a few weeks ago.

PE G'S: !'ever; RP nol-tnal: t a c l ~ y a r d i i i .PF,: ~nai-krrl~>alloi.:

facial
flii.;hiii~;1 1 0 c~anosis.icier-(1s. or. rrspiraturv dis~r-rc.;r~irlerzt.

Labs CRC/PBS: negative direct and indirect Coonlbs' test. Normal

sel-11m hiliruhir~:n o d reprat czuss-
incon~patibititr'f i ~ i ~ noli
~nnlcliingol'donor ~ r u m
and patienl's hlood.

Treatment Suppc~rtirc;;In tipvrrtics: Ie~~kocyte-depIete

ruture transfusions
hy Liltration.

Discussion Frhrile nor1hemolviic t~-;lndi~qion rmction ic ratrqerl hy preformed

l e n k o a g u (qwttosirantil~orlicq)dt-i)elop~rlaf~el-P ~ P V ~ ~ P I S
l r i ~ t l u f l ~ \ i r ) i l sit: i s prinririlv :i type J
J hypersensitivity reaction. Skin
rash anrl pl-11r-itnsor anapliylaui5 occur it1 rrllel.gic r~acrirrns
media'ted by IgE (due to n type I hypersensitivity reaction).

* TRANSFUSION REACTION-FEBRILE NONHEMOLYTIC

 ID/CC A 12-vear-uTd while female is 11rc)l~gI11
trl thp emergency room

k~ecausrof uncontrollable bleeding following a tooth extraction.

HPI She has a history of prolonged bIeeding fc~llowirlgrr~iriii~~al

Li+auma.He]- father also ha5 a bleeding disorder.

PE Mucosal pctcchinc; cpisraxis.

Labs Pmlongd bleeding time; nlorlerately prolonged PTT; quantitative

xsay for factor VlII reduced; platclctc do not axkTcratc. with
trst; low von Willcl~mi~d'r
ri~toceti~l L;~ci(n-
(vMT) arltiget~levels;
lo~v\MIF acl hlty

Treatment v11T conccntrale (Tfl~maie-P);

Dcsmoprcssin. virallv ;~ttrn~~atccI
avoid aspirin.

Discussion R c-ommon congellital disorder of hcmostasis, von IVillrhrai~d'~

discasc is also callcd ~ a s c u l a rhcmopl~ilia.Tympes
I and J I are I
autosomd dominant; vWF factor is necessary fol- platclet
52
3
0
r
0
m
<
0
z
r-l

P
a
C1
<

* VON WILLEBRANDFS DISEASE

 ID/CC A 68-year-old ~vl~ite male visits his doctor complaining of weight
loss, increa~ingf a t i * ~ eweakness.
, headache, and visual
disturbances over the past sevcl-al:monrhs.

HPI I,lr also complain^ of eay bruiskg and bleeding gums while
I~rushinghis teeth.

PE Genrralized lpphadenopath~engorgement of retinal veins

with hemorrhages: r~locleratetiepatosplenomegalp

Labs CBC/PBS: anemia (Hb 7.3); RBC rouleau Formation. IgM

paraprotein (n~or~oclonal
spike on serum protcin
pier t mpliol-esis); increased sel-tim visco<i~y.U A : normal.

Imaging XR, plain: absence of lytic lesions (vq. multiple myeloma).

Micro Pathology L!mph node 1,iopw may be labeled pleornoz-phicIvmphorna;

Imne marrow and spleen typically infiltrated with plasma cell
precnrsar5 (plasmacytic lvmphoq.te\); mav show cytnplxxmic
cosinophilic. PM-posi tive inclusion bodies D L:T<:IrER RODIES) .

Treatment Fla.;mapheresis: chlol-a~nbucil;cyclophrlrphamid~.

Discussion Waldenstrbm's macroglohulinrmia is a m x l i p a n t Blympl-tt~cytc

diqorder chal-acterizrd by excessive XgM (rrracroglohulin)
production ancl hyperviscosity syndrome.
 IDJCC A 2-yea]-+Id male i% brought to liir pediatrician hecause of rccur-
rent epistaxis artrl chronic eczematous dermatitis.

HPI H e has a history of recurring pneumonia and hilateral chronic

s~ppi~rnrive otitis media. A male cousin sul'ress Crnm a similar
illness.

PE Epistaxis; ccxmatous dermatitis ovcr 1~0thlegs; several

purpuric patches over skin; mild splenomegaly and cervical
Ivrnphadenoparhy,

Labs CBC/PBS: thrombocytopenia:lvmphopenia. Decrexqed

isohemagglutinins; decreased IgM: increased IgE, ilorrnal IgG.
and increased IgA; inability ta form XgM antibody to carbohy-
drate antigens (i.e., capnilar- polysaccharides of bacteria).

Treatment Largely supportive; bone marrow transplant; splenectom):

L
Discussion Wiskoi t-Aldrich synrlrorn~is a rare X-linked recessive diseaqe f
with B- and T-cell deficiency chal-actcrized Ily a triad of 3
tharnboqtopenia, eczema, and recurrent pyogenic infections; it
0
i q due to a delelion of ihe WASP gene in the p l l region of the '3
X chrnrnosotne. The conditiot~is associated wirh an increased <
0
incidence of lymphornaq. Z
m

WISKOTT-ALDRICH SYNDROME
 IDJCC 11 &y~ar-oldwhite female is brought to the etnergency mom hy
her mother becalrse n i severe itching, joint pain, and a
generalized skin eruption.

HPI She had received an injection of penicillin 6 dayr3 before for

stre~~ococcal tonsiEIiCi5, Hrr- mother denies any reIemnt past
meclical history, including allergies. ORCC in the hospital, the
child rlevelnpcd fever, edema or t h c ankles and knees,
hematuria, and Ict1iat.p.

RE VS: li-vex-. PE: gcn erali zerl urticaria1 skin rash;axillar y arid
inp~inall~mpl~adcnnpatlly; splcnomegaly; rednrss a11cI swelling
or knees ancl ankles.

Labs Jncrea~edESR: decreased GS, C4 levelq. UR: proteinuria;

hcmaturia.

Gross Pathology Gcnrralized wheals throughoul hnrly.

Micro Pathology Vascular lesions show fibrinoid necrosiq and a ner~tropl~ilic

infiltrate: immune complex deposition in kidney andjoints.

Treatment aspirin; cpinephrinc if srvel-e.

Ai~tihistamines:cor~ico~tt~-r~ids:

Discussion Scrurn s i c k n e ~ iss a type III hypersensitivity reaction (irnmurle

c n r n p l ~ xrt isease) with a Iat~ncyperiod I~etweenexposure lo the
d e n d i n g agcnt { d r ~ l gserum)
~. and lhe appearance o f s i p s
and s y p l o m s : it is usrlally sellllimiting.

SER'UM SICKNESS
 A 50-year-old male presrnts with complaints of palpitations and
chest pain.

Ute pair) increases with physical activity and is relicved by rest.

He has multiple s e x d partners.

VS: high-volume, collapsing puke ( W ~ T E R - H A M M F RPULSE) ;

wide pulse pressure. PE: p15toI shots Ileal-rl over braclliaJ artery:
to-and-frv murmur hearcl over fernoral artery (Drr~nzrb'/'s
hi^-RML~R) ; cardio~negalv:loud aortic component of S2; gradc IT1
early diastolic murmur heard rddiatir~gdo\vn right sternal edge
{rn~~rrnur of aortic inco~nprrence);mid-dias~olicmtlrrnul heard
at apes ( , 4 u s n ~Fr ,INI- M I~ R M U R ) .

Labs ECG: left veniric~darhypertrophy with strain pattern. VDRL and

FTA-ABS positive.

Imaging CXR: "tree bark" calcification of asce~idingaorta ail$ arch of

aorta;mediasltinal widening and cardiomega?iy.Echo: aortic
incompetence; left vcntsiculal- hvpertrophy and dilatation.

Gross Pathology Gt-nss cardiac hypcrtrrlphy (cm- bovinum) ; aoriic aneurysm

in\,olving tlxe arch and thc ascending aorta and rxtenciing into
the sol-I ic valve, rendering it incompetunt.

Micro Pathology Obliterative endarteritis of viira vasortrrn; d~generationand

til~rosisof' outer two-thirds of aortic media; comperlsatory
irregular fihroits thickening or aortic i~ltima.

Treatment PenicilIin; surgical excisinn and rcpai~,

Discussion hortitis occurs in the tertiary stage o f syphilis, orten arising

many rlrrarles d t e r thc primary inrection. U'eakening of the
aortic wall cansrs clilalat i o n or the aortic root ac weIl as aortic
incompctrr~acarid aneurysms. Inti~rlalfiln-04s causcs nan-owins
oi' the openings of the coronary arteries {ostial stenosi5),
resulting in niyocardial ischemia.

Atlas Link

" SYPHILIS-TERTIARY (AORTITIS)

 IDjCC A terni funale n e ~ h o r nis notccl to have edema, d w n e a ,
cyanosis, and marked jaundice.

HPI Her mother is blood type AB rCh-negative. I-lcr previous

childbirth w a s an uneventf~llfull-term vagirlal delivery
cnnrll~ct~rlnutsidp the United States 4 years ago. T h e mother
did not receive any subsequent immunizations,

PE Pallor: marked jaundice; hypotonia; SS and S4;

h epatosplenom ~g~ly;generalized edema.

Labs BEaocI 6-pc of mother Rh negative: bfootl tvpe of-faliler A Rh

posirivu; blood type of first child A TUI positive. Mot her'q serum:
positive indirect Cwombs' test, anti-D antihndv titer > P:64.
Nenna te's serum: poqirive direct Cooinha' test. increaser1
indirecc bilirubin.

Gross Pathology Brain spccir~lunfrom au tops): reveals yellow ~tainingof hasal

ganglia by ~inconj~rgatedbilirubin (IU:IINI(XEH~~S).

Treatment Phototherapy (promotrs elimination of hilil-ubin); exchange

transfusion.

Discussion The mother prodt~cedanti-ll (I$) antibodies ow in^ to her

exposure rn D antigen during hcr dcLirery of an Rh-positive
i n b n t . In her sl~hseqzlent pregnancy, t Iiese an tihodies crossrcF
the plac~ntaand reacted with the fehlq's RBCs (Rh positive),
prodl~cinghemolysis and fetal heart failwe with g e n d i z e d
edema ( H ~ K O P I+:TA~.Ts).
S Tc1 pwxre;erlrR1k isoitnmun isatiotl, all
MI-negative i~lotherswith an MI-positive fetus shur~ldreceive
RhO (D) immune globulin following deliveries, abortions,
ectopic pregnancies. o r even amr~iocentesis.

ERYTH ROB LASTOSIS FETALIS

 IDJCC Paramedics arc c;~lleda1 7:00 a.m. h e c a ~ ~ as c2-month-old male,
thc child of Cuban in~mfgrants,canilot he awakened by his
rnoil~er:upon arrival, it is clear that tlie child has heen dear1 ror
at least 4 hours.

HPI The child was siight3y prernatllre, but aside from this, his history
was unremarkable. These was nothing that c o ~ ~directly
ld
explain t h e episode. On directed history, the mother admits
to being a smoker and remembers that the child had a URI
4 days ago.

PE No pathologic causc rr-vealed that could explain death.

Gross Pathology Autopy reveals pctechiae on pleural and poricardial surfacrs,

pulmonary congen ion, and scattcrcd foci of Iymphoqtic tissue
in interstitiurn nClungs.

Discussion Sudden infant death syndrome (SlnS) refers to death of an

infant under I year of age, usually during sleep. in which death
remains unexplained wen after co1np1eteautnpsv; most have a
history of mirlor UFUs.

SUDDEN INFANT DEATH SYNDROME (SIDS)

 IDJCC A 1 7-vrar-olrl whire male undergoing c hernotherapy fol-
dissprninated 1-lodgkin'sIvmphoma complains of serrrc
hcarlacher, nausea, and weight loss.

HPI TIIPpatien 1 had bren o n aminoglycosides, I l l i e n questioned, he

is ~ i n c ~ r t a iofn place and time, h11t despite his c o n f ~ ~ s i ohc
n
clescrihe5 llis l~ritieas appearing rerlclish+range over the past
CPW weeks.

PE Corlfi~wdbut alert: underweight; nc) aci~tedistress.

Labs I,yt PS: it~creasedp o t a s ~ i l l tUh:

~ ~ . oliguria: I~ematllria;mild
pmteinul-ia: g1-annlar casts in tlrine; renal k~hularepithelial ccIls
in scdirncnt; isotonic urine osmolality; elevated urinary sodium
(> 4 0 tnErl/L). Increased serum inorga~licphosphor-us;
azoternia with BL'N/creatinir~eratio of 3 (within t~nrrnallimitr).

Gross Pathology Kidi~cvsenlarged, flahky. ancl pale cclc~nii.

Micru Pathology Necrmis of t111~1lar rpizhelial cells (hat 5lough into lutnen,
forming casls aizrl causing hlockarle: hydropic ~ I e g ~ n ~ t a t iof
on
epirl~elium.

f reatment Discontinue offending agent; fluid and electrolytr management.

Discussion Acll te t 11bular necr-osis i s defined as acutc tu bulacr darnage

resulting in acute renal failure: it is causer1 bv prnlongerl
iqcllemia or toxi~ls(ncphrotoxic i l r ~ ~ g iind
s ) is usiially se~bersible.

Atlas Links a-'El PM-P2-048,PG-P2-048

Ik ACUTE TUBULAR NECROSIS (ATN)

- - - - - - -
 ID/CC A 4 7-yar-old whitc male enters the emcrgcrlcy r oorn cnt~zplaining
of :I sudden-nn~e~,severe headache that is the "worst headache of
his life."

SIP1 He also descl-ihw ~low-onsetdull p i n in his 1eTr flank and bloorl

in hic urinc. F-IU was recer~tlvtr-eatect for recurrent UTIs. which
were attril~utccftu an enlarqerl prosrate glar~rl.His father died of
chronic renal failure, a n d his p;lternal grandfather rlicd of
cerebral hemorrhap.

PE VS: h~pertension(BP 170,' 110 ) . PF,: palpable. non tender

abclomind mass n r I t ~ cth flanks; nuc ha1 rigidity

Labs CA: alhuminuria; rr1icrc)rcnpir hemahu-ta ( n o MTRCs or casts).

increaser1 BUN, crcatinir~c..
Sfigf~~lv

Imaging hngio. nruru: ruptu1,e.d herry aneurysm.CT/LlS, al~dntnen:

multiple kidney and liver cyst%.

Gross Pathology Kidney tnarkecllv c ~ ~ l a r g e,c~l n dhein.>:wi tli hunclrcds of cysts

hat almovt replace nor.mal parenrhvma: cvsts thick-~t.allrd,
ranging froin it f e tnillilnefers
~ to srveral cer~tirneterqin diameter.

Micro Pathology C:v?tic dilatation of tubt~les;epithelial cell hyperplasia: c ~ ~ b o i d a l

epi~helium lining cyst<.

Treatment Dialy~isancl rcurtl n-a~lsplaiitation.

Discussion Ad it1 t polycvrr ir kidnes disease (,4PF;D) is ;In autosomal-dominant

cliscasc causcd by :I dcfecl in chromosome 16 in which the renal
In hllnrlt-erlsof fl~~icl-filled
p a ~ - c ~ ~ c f ~isy cor~~er.tetl
m;t cvsts, resulting
ill p n s ~ e s i v urenal f~ill~re
in adulthoocl. Cmtq ITl,lV ;duo involve
s . sp1ee1.1.It is associated with 1w-t-y
the panclras. livcr. l ~ n ~ ganrl
anrurwms of the circle of M'iIlis, hypertension. and niit~aIyaIw

DpC"
; ADULT POLYCYSTIC KIDNEY D I S E A S E (APKD)

- - - - -- - --
 ID/CC A 5 - v r a ~ n l dfemale is brought
to the pediatrician because her
!nother tlnticecl blood in her urine and diminished vision acuity.

HPI Her family is Mormon. Her mother surfers frnm chronic renal
fbilur-e.

PE 1:s: BP normal. PE: appears well nourished: bilntcral

sensorineural hearing loss; bilateral cataracts.

Labs CIIC;/PBS: nnrmoc hromic, normoqtic anemia. High-ton?

ren~orineuralloss detected on audiomctry: eletpated serum
cre;uiiiinc and RUN. UA: proteinuria; hematuria; RI3C casts.

Gross Pathology Srnal't,s1tloot31kidney.

Micro Pathology Lorigjtudinal thinning ancl splitting r ~glomer~~tar

f hascrncnt
mern brane, procli~cingcha~acteristiclaminated appearance with
glornvl-r~larwlero~is;interstitial infiltrate containing fat-filled
rnarrnphages ( 1 ~ ~ rF. ~e A VC:EI.LS).

Treatment ACE i n l ~ i b i l o rrenal

~; transplantation

Discussion Alpart's ?mrlrnme can he autosomal-domi narlt car x-lit~kerl:and

is caused hy a defcct in tho or chain of tvpe n?' col2ayen. It is also
 A 4,Feyear-olrlwhite female complains of palpilacinns and
shortilcss of breath, morning s\velling of the eyeq. a1 Ins, arlcl
legs, and nt~mbilessof thc lower legs together with weight loss
and Catigue.

Her past medical history is unremarkable.

Mi lcl cardiomegal:?: rnacroglnssia; pitting edema in lowcr

extremities; ascites; cardiac arrhythmia on ausrulration.

Labs LJ-k proteinl~ria.ECG: ~ ~ e n t r i c ~ thvpertrophy

lar a r ~ dIcw vnltagc
(resttictive cadiomyopathy). Hymproteinemia; hyperlipidemia.

Imaging CXR: biventricular cardiac enlargerr~ent,

Gross Pathology Pathologic deposition of amyloicl glycnprotrin irl several organs,

primarily heart, kidney, and rertal ancl gingit;ll tiqsue; kidneys
palc. waxy, pa): and firm: spleen and liver ]nay be enlarged;
clecp-brown discolnration characteristic of amyloid-infiltrated
organs exposer1 to iodine.

Micro Pathology Applegreen birefringence in polarized light when stained with

Congo red; amyloirl depositinn in mesaugizim a5 well as in
er~dotheliun~ surrounding hepat ir si t~i~soids
and in spleen;
Elvaline thickening of arteriolx watlq, leading LO narrowing o f
lulnetl a n d ischemia.

Treatment Supportive.

Discussion Primary amyloidosis cnmlnnnlv prescn ts ~vivjlhnep hroric

synclrornc. hmyloidosiq mnv hc primary (in which the proteins
arc monoclonal immunoglobulin light chain) or secondary LO
ch t-rmic inflammatory stales (esperiallv rheumatoid ariht-itis a11d
tubel-culc~sir).The primary typc is ufien associated with &cell
clyqc~aqias,especially multiple myeloma, and in lhcse ca5es
Bence Jones proteins are altnost alwavs present in the serum
ancl mine.

Atlas Link
 .A 5fiyear.-old male corn plains nl' urinary frequency anrl
i n ~ ~ r r u p t i oofn thc urinary sb-rarrl owr tllch pa51 fi tt~nrllh~:
lie
~rlsocompl;~ir~s of haling wake up mt~ltipFe rime5 cluring the
night 10 urinate ( ~ n ~ m : ~ i , \ ) .

Tlic patirnt's histnrv iilcluclcr onc episodc of acutc UI-iriary

I - r t r n tion rmc n a o r l ~ hago that was i*rlie\,rtlrvit11 cathe1~1-iyation.
Hr denim any historr nFhc.lr~at~~ri;t (w. ur~rcinorrsaof the hli~dder)
or I~nckpain ( r s . rnctast;~si~rd pr.r~staiicr;rrcir>oma).He also
atlmits to liavitlg a reduced caliber of urine stream ancl terminal
dribhhg a? well as urinary hesitancy.

PE Dipj;ltal rrctal csam rcr~ralssmooth enlargement of the prostate

pl-otr-m-lingi r ~ t othe wcri~til:ovetlving rectal muccjsa mol~ilc;
bladder percussibfe trp to ~unbiIicus.

Labs UA: 2+ bacteria; positivtz nitrite and leukocyte cqtcrasc.

P~,nst;iie-hprcificanligen (PSA) lrvrlg nnrinal: ~ ~ r r ~ r l y n a n i i r
bladder neck obsimction with incrcasrd
sntrlie5 rlert-tonqtnt~
crcatiilinc :tr~clRP:h'.
reqirlual urine volume; milrlly cIc~;ltcdSCI-urn

Imaging P-S: hrnign-appraring enlargernrii t of' nirdian I o h ~ .

Gross Pathology En lar,ge-rtlprnszvle 1t.i1 1 wcll-de~n;~r.calerl

~ i~ctd\iles1111 1 0 1 crii in
diaiii~tel-in median lobe pl-mtate.

Micro Pathology Both strrlma ancl glandu shc31c' hyperplasia on 11ir)psv:

lih1-c)t1tvc~atlei10ttia1n~1~ perpla'iia 5ee11i n which ptnIiCet-ating
gla11ds arc s ~ ~ r r n n n d ebyd prolifrrating ~ m o o t l irn~lsclccells and
fihrt>l~laut~.

Treatment

Discussion A~t:c.-rlcpr.nrlentchanges ol'c s t s t j g r ~ l uand ;*ndr.ng~nr

arc.
Iwlie~lerltr, catlsc hen igtl pl-nutatic tlvpcrtl-ophy ( BPI-I):
:In increasing incidrnct. is notcd starting at 40 YtzillASof age.
up t o 75% o f ~ n r nIlv lllc a g r o f 8 0 vrars.
Tt ~~ffrcctq

Atlas Link

BENIGN PROSTATIC HYPERTROPHY (BPH)

 A 65-year-old white male cnmplnins of painless hemahria OF
several &T-s' rl~~t-alicl~~.

He i s a heavy smoker.

I.11ng~clcar; abdurnen nnntencler; nt, palpable masses; g c n i ~ ~ l i a

w i ~ l l i nrlortnal limits: n o Iy~~~pl~arlenopatl~y.

Labs CBC: slight nor,rr~ocytic,nnrrnucl~rolnicanemia. LTA: hemat~lria

and a l x u ~ ~ l a ppit11eIi;d
nt cclls.

Imaging IX'l-'/Cysrojiram: irregular filling defects above trignne.

Gross Pathology Pc'odular. cauliflower-like lcsior~~ vlhi cell tral necrosis ar~tl
~r~iiiirnalinva~inno f hladdcr wall.

Micro Pathology C;ytnIn~,rvof ltri1-1~sl~c~wsrnal ignailt cclls. Biopg' nl' hladrler shc~ws
p p d e I. siagt. R transitional cell c ~ o m ('1'C;C)
a arising fi-orn
arr)c.piihelil~rnand projecting inlo blatlder.

Treatment

Discussion Thrrr i\a t hl-eeCr)lrlincl-casc iri r-i\li i l l men, nnd thc average age
ai cliagnosis i< (ij.Risk fz~ctorsf o papillary
~ rxrcirloma of the
I>l;~clrler
inclrtde incll~stsi-a1 expnsllw Ln arylamines (especially
2-nap11t hr lalninr) , cigarette smoke, Sclr istosoma haerna tobitinl
infPcti011 (although innst Srlrbturomn infections are associated
wirh Fquamtnls ~iroptasis), analgesic abrse (especially
phenaceti~t) . ar~cllonglerrn cyclophosphamide I l~erapy.
Complirat ions i ~ c l u c l einvasiori ot per.ivesicrtlar tisst~c.ureteral
invasion w-i t l i 111-inaryol,s~ruction(luxling Ijycll-nnej~ h~osis.
pvelnnephl-iti~,ancl rcn;~lfailt~i-e) . anrl m r castases tr, tlle lung.
hone. and li~~er. TGC apprars 10 IIP asrnciaterl with rnuta~ionsill
rile p53 h ~ m o suppr-~fsor
r gene anrl clclrtion\ in chromosomes
9 p . i ~ i ( F 9q.

Atlas Link

-T
BLADDER CANCER
 IO/CC A 65-vual--ol(t rrlale prrsen ts with acute urinary retention.

HPL For lhe pxqt few yal-s, lae has notril m increased frequency of
mictut-itinn along w t h inct-ea~ecl hesitancy, urgency, dccseased
forcc a n d stream uf ilrinc, >l.ulcla furling of hcompTete evacuation
of thc I>ladcIrr.FOF-the past fcw months h e has h ~ g t nto
rxprricncr increasing fatigability and laqstude.

PE Pallnl-: hladder fill1 T ~ ahrlo~ninal

I exan~ination;recta! exam
reveals grade KD prostate enlargement.

Labs CRC: rlr>tmocv~ic ;~r~cmia.

L p s : hyncalcemia; h y l l e r p h n ~
~ > h i ~ ~ u Elevated
~ i i a . BUN and creatinine. t!A: pmteirl~~ria;
no KBCs or seen.

Imaging US, kidncys-s:biIateral hydroureter and hydmnephrosis.

Micro Pathology interstitial

and livrll-o~~rctcr.
In aclrli tion tn h~dl-otwphrosis
kidney di~eaqeis w e n on m icrnqcopic exxmination.

Treatment Transrlrethral resection of' I he pr'ostiilr (TURP) r o 1.~1ieve

the
r>hslrl~ctiotiis the hxsic atld most 11sefi11SIPP.

'Discus~ion Ol~stt-wtivrnephrnpattlv rcsz~ltsfrom t h r impaired orttfiow o f

urine hut rrlay 31x0 produce chronic interstitial damage.
Cll-~strurtivencphropatltv is common in chilcI31ood (from
congrni tal ahnorrn;~litir.q)and in irtdividuals older hat1
ti0 vearx, w h e n benign prosta~ichypertrophy and prmtatic and
gynrcoEogic cancers become Inore common.

7 BLADDER OUTLET OBSTRUCTION, NEPHROPATHY

 ID/CC -4 4%vean,ld white fefurnalc il; acl~nitrrclto the hospitill l ~ e c a t ~ s e
of worsening generalized edema a11cF rveakxlesl; along wir h
hypertension.

HPZ h isr01.y or tvpe 1 diabetes mcllitus hut no history

She has a l o n ~
01' Ilet~~atul-ia. .
rere11I sol-r tllmaf or skin i t-tiertinns.

PE \IS: hypertenqion (BP 160/ 110). PE: gep;ene&ed pitting edema;

n o cvirlcncc of pleul-al c f f ~ ~ s i oor
n awites; I t u ~ gI,ase% clr;~r-a n
auscultation; ,PTnormal: rici ther kidnt.): palp;ihle: F I I I I C ~ ~ I F C C ) ~ ~ C
exair) reveals prcsence ol' proliferative diahctic rctinopathy.

Labs Elevated fasting blood st~gar(23.1 mg/dL); ele\b;i~cdglvcosvtared

I~cmuglobin( I 0 % ): elevated BUN and serum creatinine;
decreased serum albumin; elevated blood chfialesterol. UA:
P I - ~ W ' T I of
L - ~stigar and S+ protein: bvoa~1 C ~ S I ant1
S fatty casts;
~ l e v n t t dql~xntiiative pl-otei11 (3.5 g m / P 4 hr).

Micro Pathology lncrezed rnesangial matrix cln srn;tl biopw: thickening of

capillary basement m e m h m e cnr~~hinerl with acellula~-
e( )ritiophilir n o r l l l l ~in~ i n e s a ~ i g i ~ (KMMI.
~m ISI 11. I . - W I L DIS~CLTE):
~~N
hv,llinc arrcriosclcrosis of both affcrrnt and effrrunt arterioles:
110 i i i i ~ n u CCIIII~IPX
~~e depopi~qqew.

Treatment Rloorl sugar control; control of svstcmic hypci-tension. pl-efcriiblg

tvi th ACE irlh il>itor; dielary protein ;~tirlphnsphare yes! tictioll;
avoiclancc or ncphrotoxic drrigs; dialysis ur rcnal o-anspIantation.

Wscussion Dinhrtic glon~crulosclernuisis a t-cnitl n1a11ilesiatinn o f diahe~ic

~nicrnanginl~atl~vatlrl presenf? at l e a ~ t10 vears aftet-diahere~
appears (mol-ccommonlv in IDDM); it is ustiallv thc prcludt. to
end-slage dial~rt
ic renal rliseaue.

Atlas Link mP M - ~ 2 - 0 5 5

D I A B E T I C NEPHROPATHY
 ID/CC 11S&year+ld ~ v h i t male
r cornplains of a chrwnic c o t ~ g hof'slrl~eral
mot1thc' dt~ration.ncc~~mpanicd by liyl~rl~carlotl~it.rs,
f311 i g IP. and

ninlnise: yrstpl-day lir co-righedup blood.

HPI He ;11.;n <lcsc~-ilws I p w r AIICI tit'acIaches in arlcIitivn

in~errnittei~t
ro small vc3l11ni~s 01' dark orange mine. Ile rlcnic~nlcohol t l s r
h u ~arliiii~s10I>eiilg a lieaw smoker.

PE L)il'fusr p111monn1-vcrncktrs hi1:ltcrallv.

Labs .hot em ia. I I)$: r l l i q i z r i : ~ :hmaturia: pro teinuriz. Iron deficiency

anemia: hloorl d ~ t c c t e c in i cputurn. tI3Gs: hvpoxcrriiit.

Imaging CXR: l ~ i l i l ~ talvt-oi;~r

- ~ ~ ~ ! i~ifilt~:~~es,

Gross Pathotogy Incl.~asci l l i w i g h ~ol' I~tng-5wit11 areas of r~ecrosis:kidneys

enlarged and pxlc with clecrcased crsnrirtcncy

Micro Pathology Kiclt i ~ hioprv

v st~owspl-r>liI'el-;r nWI'f1t i ~ i n gcrescentic
tiire, ,
gTgfomcruloncphritiswith a r r ~ u n ~ ~ l a tor
i n ~ieutrophils
u and
rn:~crnphaqe~ irl Blxvrnan''; C ~ I ~ S Ichal-3ctcrisric
I~C: linear T g G
deposits in glomerdar hacement membrane and alveolar septa
on i1nm1111oll~1nl-e~cc~1re; anti-glomerular 'basement membrane
hcrnorrhagic alveolitis on lt111g
antibodies in ~ n - u r nnccrc~rizi~ig
:
i>i<I]>5yn

Treatment ~Eier.spy.
I ' l a ~ n i aexchange: corticoctet-oids; immunos~~pprrssivt.

Discussion i~ heinorr3i:rgic al~erlli ti5 with ncphri ti\

C:ontllx~stI Ire's ~vnrlrotr~e
and iron rleficienn, ar~emixc : ~ r ~ ~17v c c lanti-;~lon~crr~lar bi1st.111enl
I S ~ I? rt'ac1io11).
~ d ( R ~ CI1 ~ V P L ' I - I C ' I tivi
mcmhr;mc a11t i l ~ r its

* GOODPASTURE'S SYNDROME
 EDJCC A 45-vual-uldblack male prescnts with ~ ~ r ~ c o n t r n lhypertension
led
and complains ol'severu occipi~alhearlache ancl ringing ill h i 4
ears.

HPI He a l ~ oreports markedly diminished urine output over the past

24 hours. On direc trcl que~tioi~ing,hc ;ilso reports some vis~ra'l
blurring.

PE VS: severe hypertension. FE: flrndurcopv re~~eals

presence of
papiIIederna with hypertensive retinopathy.

Labs ViZ: protcinuria: microscopic hematllria: red cell cmtq. Elevated

BUN and creatinine. CBC:: micronngiopathic l~enlnlpticailcmii~.
ECG: lefl-axis rlrviation with Ieft h m e rtticl~lal-
l hyertrophy

Imaging Echo: ronccntric left vrn~ricl~lal- hypertrophv tvi~11i.eciureri

ejectinn fraction. LTS, abdomen: presence of parenchymal renal
disease in norrnal+ized kidneys (unlike that of henign
~vlvheretllcr-c are I>ilatrt-a1contracted kirlnevc).
I I P ~ E I I-OSCI~I-osis,

Micro %thology PatIloIogic changrs includc fibrinoid necrosis of arterioles

.
( N I:< ROTl71VC 4RTERlr31 m s ) hyperplastic arteriolosclemsis
( " 0 ~ 1 0SU;INNIN~;*),
~ ~~ a s s o c i a ~ e dwith
and n e c r o l i z i ~glotner~llitis
a thrombotic rnici-crangiopa~hy.

Treatment Reduction o f diastolic blood presstlre to at least 100 rnmHg;

maintain urine output > 20 rnL/horw.

Discussion Sodirun nitmprusside i.i the cafest ancl most efcctive drug for
usc in hypvrtcnsive errirrgencies: I3ecarl~cit docs not irl~pair
inl imrlrl-lying
mvuca~-dialbloclrl flnw, it iq especiallr: u s c f ~ ~
i~chenlichexrr discasc. Howrvet; i t ir; inerrtl~olizrclto c y r i i c i t '
xnd thio~!a~lale; thrt-ef'ore. proloilgcd us(: rnnily lead LO r!anide
toxicity or to thiocyanate tnxicitv. Blood tlli4 ~ cana y re Ievcls
should be cteterminert frequentlv.
 ID/CC ,I 2-vear-old white male romplaills of rec~zrser~l epi~nrlesof
"bloody h e ' ' that laslril Tor several clays in conjunction with a
URI.

HPI Hc was well lrt~tilthe onset of'wtnplnmq.

PE Pallor; slight palpebrnt cdci-na.

Labs Zr;ZI: ~rrnteinllria;red cell casts in urine; gross henlaillria.

Increased serum IgA.

Micro Pathology Focal glotneri~lnnel~hritis

involvirlg otjlv sclected glc3meruli wit11
mesandal proliferation and segn~eiltalnecrosis with crescents:
immunofluorcscheucerypically reveals mcsarigial TgA deposits
with w m c IKIW. TgG, and C3.

Treatment S~ppol-t ive; IgA clep~sitsr o r n r ~ l ~ l treappear

ll~ fvllowi tlg kidney
I ransplantation.

Discussion IgI:4ncpkxopa~hyis idiopathic hut associated with rlppcr respil-a-

trlr y cji- GT infections lacking a laserlcy period (VF. postrtreprococcal
glomcrulonrphritis). Lt.4orls are variable and may he mesangic-
prolifurdtive, focal ptnliSe~;itivu,or possil>lyct-ewendc glomeru-
larlephritis. The glomerular pathology seer1 in B~rger'sdisease is
similar lo that wen in Henoch-Schiinlein purpura. ~vliichis seen
in children. It is scrn with illcreased f;cqlre~lc!,in patients with
t r l i a c disease and liver disuase (due rIefectivc Ig14uImr.aiice).
Chronic rend failure may ultimately develop.
 ID/CC Pc 30-year-old black woman preqcnts with pain in both her knee
joints and in t l ~ esrn;1ll-jnint5oCthr h a n d logether wit11 milt1
fever; atwl-&a, weight loss, and 1099 or hair.

HPI She alqn has a history of recurrent oral ~~lcemtions

a13d a
phoiosemihe skin rash.No,joint deformities art: reported.

i i . oral aphthous ulcers uoted; cry lhernatnus

PE VS: h y p e r ~ e ~ ~ s i oPE:
photosensitive skin rash: "butterfly raqh" nver 111alar area of
hce; pallor; n o ahdominaI o r renal I>ruitq hrarrl.

La bii C;BC:: normocyt is, 1 1 0 r ~ t l o c h r o ~ nanemia.

ic FA: ~nicroscopic
h e m a u ~ r i azvi t h RaC casts in aclclitit,n to pl-oteiuuria. Elevated
RUN and creatinine; antinuclear antibodies positive in high
titel-: LE cell phenomenon posi~ive;anti-Sm antibody and anti&
DNA anFibody positive; VDRL positive but FTA-JIBS negative.

Micro Pathology Rcn;~!I ~ i o l ~reveal5

w 1eatu1-csof diffuse proliferative glomeru-
lonephricis. Klertmn microscop\ reve;lls immune complex
deposit9 that are typicall! subendothelid nnd form "'wire loops.'"

Treatment Cni.ticost~r.oirls; cyrotoxic ill-11gs(cyrlopho~pharniclc,iuathio-

p r i n ~a, n d c11Ior;~nlbucil);Inng-term hcrnodialysis nl- transplant.

Discusslion Tl1ei.e are five pattcrns of I i~pu.;nephritis. Class I i s ~iorrnalIly

light. EM, and i~nmt~nofluoresccncc luicroscopy. Clam I1
pl-ewnt c; as rnesangial lupus gZomeruloncphritis and is foun rl in
ahout 25% ofpatients; i t is ;isw~ciaterlwith minimal Ile~natitria
or prnteinuria. Class I11 is chamtesizccl hv focal proliferative
glomc*rl~lo~~el~h a nf 'di ~isi sassnciatccl wit11 r-eclu-renr l a c n ~ a h ~ r i a
arlcl tnilrl r e n d i~ls~rfficiency. !.Claw I
S' is clcscril>eclin tllis rase
and is hv far thu rrlosl common I'orrn. Class V pre5ent5 as
membranous glomerulonephritis ancl i'i seen in 15% or cases; i t
iliclt~cessevere protrin~rria(11.nephrntic qndrunrc=.

LUPUS NEPHRITIS
 IDJCC AIT1 I-year-rllcltvlii~egirl is brought to the perliatrician hucausr
of hc:tdaclic. cheri palpitalions, and ringing in llcr ears t o ~ e t h e r
~ i 2 hgeneralized edema,

HPI She 11x7 nn hiqtorv nF dvspnea, Fore throat, skin infec~innlq, ot-
lcvrr. C a r e f ~ que4tionin~
~l reveals that shr has a h had hematuria.

PE ( HP 140/ 1 00). PE:: g e n e d i e d (including

S'S: t~\.per~~ension
prriorl~ital)pitting ederna;JrP nnrn~;~l; lung hases clcar; neither.
P pleu tal eIr115iorlor ascitcs.
liidnpy palpnhle; no P V ~ E ~ E ' I I Cor

Labs Elrva~etlBUK and serum crei~~init~c: clecreased s~t-unia l h ~ ~ m i n :

clcv;t tcd wl-iim ~righcerides;serum hypocomplementemia;
anrirlt~clearatltihorly (ANA) negative; normal A S 0 titcrs. UA:
fatty casts and oval bodies in addition to proteins,

Micro Pathology Dii'fusc glornc.r~rl;lrinvolve~rlrntwith ~hickenedcapillary walls

or) lighr rnictnscopv.
and FohuIar ~ n u . w ~ ~ gprcdiferatic~n
iaI
SpIitting of has em en^ membrane causing railroad-back
appearance with ! 'AS reagent or silver ~ t ; ~ i prominent
n: granular
immanofluorescence;mrs;tngi;-11 arid sohenrI(>tEirlial tlepositr of
inin~iinecornpleseh,

Treatment Cort icosteruids; renal transplan~atiori .

Discussion Ven~hnnopr.oliferxtiv~ glr~tner~alonephritis (MPGK) is idiopathic

hut mav he aqsnciated ~vitliiuflcrited dcficicncic.; of conlple~nent
compo~lrn It is subdivided into two
ts arld partial lipudvs~r.ophy,
I Y ~ P S : npe I MPGN (hot11 classic a n d alternatir~ccomplerncr~t

pathways activated) ancl type I1 MPGK (clcnsr deposit diseasr;

a c t i ;I~tion of nltrrn;~tecomplcmrn t p;lthwav). Apprn~iniatelv50%
of psticnrs w i ~ h3ZPC;K will go on In rlev~lopchronic renal failure.
Thrr-c is a high rrpcurrence rate Lollrwing rer~;~l ~rat~rplantation.

MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS (MPGN)

 h 47-war-old black diahetic Ternale complains ofwcight luss,
shortnew of 111-path,anrl swelling of the lower leg
pl-ng~-es~ive
and arrn5.

Hrr past meclical Ilistor), is tiii~-en~ar-kal~Ie.

Pallor; pitting erlen~ain exmerniaics; clecrmsorl lung sounds with

cracklcs hilatcriill~in Ic~wcrlr~ngIirlcls: periorbital edma; mites.

Labs UA: proteinuria (> 3.5 g/24 hr); lipiduna with o'i~:~lfat hodie5
a n d Iatw and was? casts in lit-inary sediment. Hypoallbumhernia
(< 3 g /dl,) ; hyperlipidemia (serum cholesterol 250 r n ~ J d L ) .

Gross Pathology Iiidilevs r tllargecl, p a k , and 1-11bherv; renal t~cinthronlbosiq may

be p ~ e s e r ~ t .

Micro Pathology Thickened basement membrane; .;lthc.pitheli;~ldepclri ts of IgG

l d along h a ~ c m c n tnlcrnhranr srcn i1-r "spike and dome"
i ~ ~ CS
pattern un m c t h c ~ l a m i n esilver qrain; i m m u n e cleposits in a
"lumpv-bumpy" (disconrinurrr~s)pattern o n ~~nrnunofl~!crr~ercrtice.

Treatment Corticr~stcrnlrls;yclophosphamiclc; renal transp1;;lntat inr1; ACE

inhibirors redricu urinary prmteiri Ir,ss.

Discussion h'cphrotic m~cIrumcmay hc idic~p~thic or cauqetl Ijy m e n i b ~ a -

nous gl:lonrcr~ilr~i~rphriti.; (the m(w1 cnmmnn ca~tsc.in adults).
rriinimal change cfiqeasr ( I . I I Y ) I I V) I . E ~ I - I K O ~(the
I \ ) most common
in cl~ildrrr~), IbcA glomer~l toscler(3~is.or mcmhranoprolifera-
live glorner~~loiiephri~i~. Patiet~~.: w i ~ hnephtncic wndt-rrme have,
hpercoaplabiity sccondnrv to locs of antithrombin I11 in t h e
trine ( p . ~ .inrreaspd
, incidence pcrip11e~-;ll vein thl.nmhnsir).

" MEMBRANOUS GLOMERULONEPHRITIS (MPCN)

 ID/CC i2 5year-olcl white rnale presents \v<tl? generaked edema and
ahdnminal clistcntion , producing respiratorv embarrassment.

HPI agn.
T h c child had a URZ 1 u ~ r k

PE VS: 13P normal. PE: generalized pitting edema; frce asdtic fluid
in prrironeal ca~ity;shifiing dullncs~and fluid thrill present;
nortnal rund~~scopic exam.

Labs L J k .I t. proteinuria ( > 3 g/24 1). Hypoalbuminemia;

hypercholesterol~mia: hvpet-triglvccriclenlia: rlrcreasccl s e i - I I ~ I
ic111iccalcium: normal CS levcls; normal serum crcatii~ine
and BVN.

Gross Pathology slight2y enlai-zed, soft, ancl vcllowi5h.

liid~lt>\~s

Micro Pathology I .igl~rmicrascopv and immunofluorescen t shldies normal on

renal biopsy ( n a ~\<rLcnceof immune complex deposition). EM
rcrcals uniform and diffuse l n s s or the poclocytic foot processes.

Treatment Corticosteroids; salr-restricted dict: dilirei icq: eIccnoIyte therapy

and rnonitclr-ing.

Discussion Xlsn caller1 Lipoid nephrosis, nlinimal change disease is tlze most
common r m s c of idi oIjarf~icnephrotic r;yndrome in children
ancl is associate(1witla ini'cctiuns or vaccinations. It carries a
good prognosis.

Atlas Links fIFZI PM-P2-062 I IM2-036

 A tj8-vear-old black male complains nf dysuriia, pr~~gressively
increased u h a r y Frequency, and hack pain 11iat has lasted
several months.

IJe report5 high animal-fat intake.

Nodulal; r o c k - h d , irrep4ar area of indrrratinn irl peripheral

lobe of prostate on d i ~ i t arectal
l rxilm: midline fwrrow between
prosta~iclobes obscured; extension to serninal vesicles detected.

Labs Markedly elevated prostatespecific antigen (PSA) and acid

phosphatase.

Imaging Transrectal VS, prostate: hypoechoic masses in periplleral zone

with extensioi~tn seminal vesicles. NIIC,brine scan: hot lesions
of spine, sacrum, and pelvic bones (axial skeleton). C T / M R:
prostare mass with capqular pener ration ancl ~tilargedseminal
vesicIes.

Gross Pathology Irregularly enlarged, firm, nodular prosla ie.

Micro Pathology Gore needle biopsy of prostate reveals single layer of malig-nant
neoplastic cells arranged haphxardly in ;trlt.rloplastic struma.

Treatment Proqtatectorny with radiation; or-chicctomy: leupralide:

antiandrogens such as flutamirle.

Discussion A primary malignant neoplasn-i o f the prastare commonly

arising from the peripheral zonc (70%).prostatc carcinoma is
thc most common male cancer. Its prognoqis ancl rrr;3ttncnt
depend heavilv on stage. Most case5 are diagnosed in
asymptomatic mcn un digital rectal exam. Prostale cancer
exh i hi ts hernatogenous dissemination. Inosr row mon lv to bone,
forming osteohlastic lesions. Thr tumor can a l ~ oinvade sacral
nerve rOOF& causing significant pain.

Atlas Links
 ID/CC X tifl-year-old white inale complains of r i g h ~flank pain and
hcmaturia.

MPI H e Elas heen n heavy smoker for the past 24 years: he lost
5 pounds over the past month and i s net on a diet.

PE VS: To.~v:r-ade fever; moderaw hypertrnsion. PE: pallor: pdpable

mass in right flank.

Labs Elcvatcd ESR. CRC/PBS: norrnoqtic. normochromic anemia.

t'A: gross hemamria.

Imaging n T J C T J U S :mass in upper pole of right kidney. MR: no

invasion uf renal vcin o r infcrior vena cava (RTC).

Gross Pathology Yellowi~hareas of necrolic [issue with fncal areas of hemorrhage

within rcnal parenchyma.

Micro Pathobogy PoIy~onalclear cells ( c r l t ~ l atling

i glycogen) with evidence of
rytologic sepia invading renal parenchyma.

Treatment Right nephrectomy; cunsidcr renal-sparing partial ncphrcctomy.

Discwssion The most common rend tumor, renal cell carcinoma is

Crcqticntl~speradic hut is, seen in association with von Hippel-
k d a u syndrome and djalysisrelated acquired p o l p t i c kidney
disease. I 1 rreqr~enOyinvades the renal vein and TVC arid
mctastasi7es to lungs and hone ~ i Iletnatoget~ou~
a dissemination.
Ic can also cause paraneoplastic syndromes (secondary to the
.
pr-c3rI11rtion r )f erythrnpoietin para thyroid-like hormone, ACTH,
and renin).

Atlas Link PM-P2-064

h RENAL CELL CARCINOMA

 ID/CC A 63-!car-old white male compIains of sudden-onset pain in the
right flank lugether tvirll gross hernaturia, nausea. and vomiting.

HBf He is overweight, has !>ernrlial~~ticTor 15 years. is a hcar??

smoker and drinker, atid 1-tas Iwrn surgicallv treated for
aortofernoral occluskve disease grid^).

PE VS: nn fever; mild hvpcrtension (BP 150/10O). PE: acute fislress:

pilllor; swc-ating; st-vtre right flank p;~in;xanthelasma in hot11
r-vclirls.

Labs Normal RUN and creatininr. UA: hernaturia. FCC;: old silent
~'ilI~
an zrrior l ~~yo~il~ Elevated LDH.
i~ifarctiotl.
-dial

Imaging CT, a1,domr.n: wedgeshaped, nonenhancing lesion in right

kidney, US. rei~al:edeinatous kidney with Focal region nf
dpcreaserl color flow.

Gross Pathology Pale. yellouish-white, wedge-sl~apeclarea wit11 hernorrllagic

necrosis in renal cortex.

Micro Pathology Cr,;~guI;ttionnecrosis irlvolving renal cortical nephrons

cxtcncling into cortitomed~~lla~junction.

Treatment Remove arterial obstr~~crion

IIV thromholvsis; Iwparin an tico~gu-
lation ro prevent recurrence.

Discussion Risk Fitcror~Inr emholic events inclr~dcatherosclerosis and

r n ~ ~ rthrombi
al in the Iieart and aorta. infuctious enrtocarditiq
~egetatinnq.and atheromatous plaqiics in the atma.
Crwnplic:\tion- fr-urn I-rrlalartery rmhnliqtn incll~rlerenal failure,
hvpel-rensiotr, acute pycIonephritis, and rcnal abscess.

RENAL INFARCTION
 ID/CI: ,4 30-year-old &it? female iq fmind tn be hypertensive on rolltine
physical vxam.

HPI S h r claims lo have no history of hypertension 3 r d denies any

changes in lifestyIe or excessive strcss.

PE VS: hypertension (13P 1 75/ 105). PE: loud S2; hlnrlt~rcopicexam

normal; abdamind bruit present.

Labs Elevated plasma renin: hvpokale~nia.

Imaging A n ~ i orcnal:
, cnniirmator~;unilateral lcft renal artery stenusis
in a "string of pearls" pattern.

Gross Pathology In fihromu5cular dysplasia. the rei-ial artery lumen is decreased

dur. ~uhvpcrpfastic fibrotic wall thicken in^.

Micru Pathology Mi~scularhygerplasi;~with fibrosis and segrnetltal s!enosis.

Treatment ACE inhib'itors (contraindicated in hilateral renal artury stenosis).

s z e n ~ n g~unjcal
Ik~lloona t~ginpla.itv; ; corrrction.

Discussi~n Renovascul;u- I~ypertcnsionis secondarv wsteinic hyp-pertension

rai 1xe~1 ion of r.enin ti-om Fiypnperfuwtl kidiwy(s).
11)' I~y~el-secret
I t is mast ohen cauwd by fibromuscular dysplasia (young
Caucasian women) or atherosclemsis (older men) ancl accountq
for < .5%, cof all caucc-s of hpc-rrrnsic~n.

Atlas Link T-1PG-PZ-066

RENOVASCULAR HYPERTENSION
 S&yeardd wliitc male prcscnts wit11 progressive painless
enlargement of the left testicle of' 2 mnr-frhs'rltlration.

He also cumplains of a w i s e or hrsviness in his scr-oturn. He

nf p a i n or ir-auma a t the ~ i t e .
t l r i i i r s i i r l y histnl.~

Walni~r-~ized. nontender. s111uot11. firm mass at upper end of left

testicIe: mass does not bansilluminate: epididymis and vas clerer-
ens normal on palpation: prostate a n d srminal vesicle5 t~ol-inal
oil digital rectal w a r n : xhdnlninal lvlnph nocles not pnlpahlu: no
hepa~ornegza2v.

tabs h'cjrrnal lcvels or hC:G; normal levels of serum a-fetoprotcin and

LDH; histologic ( i i i t q n m i s hased o n postopurative specimen
stuclv.

Imaging CSR: n o met;lst;~ris.US, ahdonlurl and pelvis/scrotum: 5olirl

intratc5ricular m a w CT: no 1neti1st;tsi.s.

Grass PathoIogy

Micro Pathology Shepts of germ ceIls ctin~aintngclear cyoplaxm wi rh lymphocytes

in f i b r o l ~ sstrclma.

Treatment OrcIiicctomv ui th rctrc~prrilonralItmph nocle diswcrion;

chcm orhcrapv ni th cispl:~tir~;
rilciiot het-apv.

Discussion Seminnma i q tlre most cornmon Cpc tjf germ cell rumor.
Dvsgpl-lninnmaq in nvarie~are histnlngicnlly similai: T11rnni-s iivp
extrcmc-IT radiosensitive. It is assr,ci;~ted rvi th ;I good pl-ngnosis.
Cryptorchidism prcdi5poscs 'to the decelupmcn~of lev ic~llar
tltrnol-~.

Atlas Links
 ID/CC A 30-yrar-old man cornplaiils of a small painless nodular swelling
ever his right testicle that he noticrd a few mo11~hqago, coupled
wit11 increaqing growth of his breast tissue.

HPI He also compIains of mild rhortncss of breath on exerrion

( n m p u ~ x )cough,
, and hlnorl-streakccl sptl~um.

PE \rS: normal. PE: bilateral gyneco~nastia(breast tissue paIpahlc) ;

srnaI1, pea-shaped swelling involr.ir~gthe right testicle; testicular
sensation lost: no Iransillurninatiot~;left s~~praclavicular
lymphadenopathy; h epa!nmeply.

I Labs CRC;: mild anemia. Serum 0-hCG elevated.

Imaging CXR: I I ~ O''cannonball" parenchynlal masses (due to metastases).

T=T, aabclornrn: erilarged rernvptritonral lvrnph riodes and multiple
hepatic nletastasw. US, scrotum: complex. solid r i ~ h resticular
t
mass.

Gross Pathology Small. p~a-shapedhenrorthqic mass seeti in right testicle.

Micro Pathology Polvgunal, co~nparativelyr~nifclrlncytotrophoblastic cells with

clear cytoptasm growing in s h ~ e and
~ s corrls. mixed with
multin~~cleatesyncytiotrophoblasticcells that have eoslnopMc
vacuolated cytoplasm with readily d e r n ~ ~ bhCG;l e no well-
dcreIoped villi seen.

Treatment Chemotherapy with cisplatin, etoposide, and blromycin in qome

cornhin;~tion.Fnllowecl lrry radical inguinal orchiectomy ancl
retroperitoneal lymph node dissection; gynecomastia rrgres3es
once rhc source nf hCC; ( t h t~~ i ~ ~ ii~o removecl.
r)
I
Discussion Choriocarcinoma is thr most malignant of all ~ ~ s t i c u l aturnurq:
r
i t metaqtasizcs relarively early via bolh the fymphatics and she
bloodstream even w h e n i t remains very small locally. Follow up
with P-h CG levels.

TTESTICULAR C H O R I O C A R C I N O M A
 I'O/Ct A nelvhol-n hahy is uv;duatt.cl for ambiguous external pnitalia.

HPI The baby was delivered v;iginally a1 fill1 term without any
pw-. inrra-, or. posrnar~!complications; the mother did not take
hormones or. any o~hel- d r u g during p r e p n c y .

PE Incompletely virilized external genitalia: Ii!~ospadia5;bilateral

inpina1 swelling.

Labs Ki~i-vurttw: 46,XY. Miillerian structures absent: inguinal

swelling4 prmed lo he maldescended dysgenetic testes.

Imaging US: absence of miillesian si rticrl tres and presence of dysgenic

trstes.

Micro Pathotogy hv seminiferous tubule degeneration and

Testes rh:1rilr1~l-i7erl
i~~vasionhy connuctivc tissr~carranged in wberlx.

Treatment Conadectomy to protect againsr increaqecl risk nT testicular

tumor; hormone replacement ~lier;~py given at puhertv.

Discussion T ~ intitlcnce
C uF gonadal tumors in d~geneticgonads rnav
reach 1123 tn SOW, i n a k i n ~ot-chirttr>myand s ~ ~ h w q u e n t
hormn~ler s p l a r e r n ~ttthe
~ hest tl~erapeuticoption.

I
Ir9 TESTICULAR PYSGENESIS
 ID/CC A ?hear-nld white male is seen I)? his Faillilr;physician because
or dmnea. bilateral enlargement of the breasts (I:~~KC:OMASTL~),
arid a painless lump in the right testis of approximalely 2 monchs'
di~ration.

HPI H P denies anv history uf STDs, genital ulcers, drug use, or

Il'illltllit.

PE Bilatcral nonterlder gyiiecornastia (duc iucreaserl hCG); left

supraclalicular I y n p h a c l ~ n o p ~ t lk~ ym
; hard mass palpablc on
righi testis, distorted shape; no1.1nal rectal exam.

Labs Markedly elevated blood hCG and a-fetoprotein ( A m ) .

Imaging US/MR, 1e.ites: solid inmtesticular mass with some Coci of hem-
t).
orrhage (iiltratessicr~larmasses usually ~r~aligrlan

Micro Pathology Cvtotl-ophehlastic and syncytintrophohlastic ceIls with h C G

dcmonstralhe within cytoplasm.

Treatment High I-adicalingttinal orchicctomyt foIlowerl by cisplatin-based

con1billation chemotherapy.

Discussion Testirulitl-cancer rnav he pure or niixerl (mixed germ celI

neoplasm) and is higl~ly~~lalig~nant with early xnrt widespread
metastaris. It is thr most coinmon neoplasm in men aged 20 to
35.Wk sac ~~~~~~s ptnduce 011ly AFP, wlier~aqrhoriocarcirlo-
tnas procluce oi1Iy hCG.

" TESTICVLAR TERATOMA (MIXED)

 ID/CC A 9-yeat~)lclldack male is hrougt~tin to the errlrrgrncy room
hecause of sudden-onset severe pain that he expericncud in the
lower at>domen and scrotum while p l a ~ i n gsoccer.

HPI He has no relevant medit;ll history. r p o n admission, he llecame

n;l~isenieclar~rlvomited three times.

PE Tsl-itakility;righl testicle tender, swollen, and e1cv;ttcd; palpable

r~orrnalepididvmis antcriorlv; increased pain with elevation of
mass (PRI.I IV'S SI(:N); 1 1 0 her-nia palpalde: 1 1 0 transfIlurnination
nl tnass.

Labs Uh: mild le~tkocytn~is.

Imaging in tel;zicle.
U S , scrcllrtiii: aqvinrri~trirrlecreaserl color flow
Nirc-Tc9S: doughnut s i p (due to ccntr;ll lec~ic~tliir-
ischemia ancl
circl~mferentialco1l;ltur;~lflow).

Gross Pathotoqy Tcsticlr rnarkcdlv cnl;t~-gcd will1 hrmtlrrf~agicnecrosis; scrotlrm

may bc p ~ i ~ p l i scol-rl
h ; twisted.

Micro Pathology Severe venous congestion : interstitial hemorrhage: hernorrl~agic

necrosis.

Treatment Immediate surgery (clttclrsion ;md fixation of tesris to scmtum)

dnc to risk of iusiicle loss (less than 4 hours); contr-alatcral
orc11iopc.x):propliylaci ically (high incirlenre or bilatcrality) ;
atrophic testicle should hc rcmu~ccIdue tr) puwihlr autnimmune
rlestrucl ion of' con il-alaterxl testis.

Discussion Tcrticular torsion is a s l ~ r ~ i c ernel-genet;

al 1ha1 needs to hr
clirk~-enti;~zed TI-oinorcl~iti~,
epidirlymitis, and s t r a n p ~ l a t ~ d
I-lertlia. I r i s seen more Frequentlv in an ondescended testide
(( ~wroirt:i-rinrs~).

TESTICULAR TORSION
 ID/CC A 45j-ear-old man with a high-grade nan-Hodgkin's lpmphoma
develops olipiria, severe malaise, and fatigue :$ti hours foIlowing
chemotherapy eeament.

PE Carpoperlal spasm prcsunt; n e i ~ h e rkidnev is palpable: urinary

hladdel- is empty.

Labs Lytes: h?.purkalemia,hyperuricemia. ,ind hyperphosphatemia

with scco~idxyhypocalcemia. BUN and matinine eIevated. IrA:
acidic urine with numerous rhomboid crystals: no casts or cells
wen.

Treatment Main tcnance of good bydriltivn. b~iskalkaline diuscsis. and

are keys to preventi13nof this
pretreatment with allop~~rinol
syndrome; once acute renal failure has developed. fIuid and
clecuoly~ebalance must bc maintained m i d clialvsis may be
necessal-):

Discussion Tumui- lysis syndronle is tnost often seen in patierits wit t i

lymphoma or leukemia hut is also scen in patients with a. mriety
of solid tumors. The presencc uf ;i large turnor burden, a high
growth fraction, an increased pretreatment LDH level and uric
acid level, or preexisting renal insufficienq~increases the likeli-
hood that a. patient will develop tumor Iysis uyt~drotne.Incrrased
Ic-vels o f uric acid, xanthinc, and p hosphatc may restdl in
precipitation c ~ these
f subsratlres in the kidney Renal sludging
and acutc renal insufficienry or failrire Flirt1 1 ~ 1 aggravate
. tlle

F?a URATE NEPHROPATHY

 A 3yearald male is brought to his pediatrician for cvalualjon of
an abdominal maw that his parents noticed.

HPI The chiId has heen well ;ill his life.

PE Slight pallor: weight and hcigh; within normal range;

smooth intl-a-ahdoininal mass to
nontcnder, large, fii-m, ; ~ n d
right of midline: sight cryptorchidism and aniridia.

Labs UA: ~nicroscopichemaiwia; normal llrinarv vanillylmandelic

acid (VM.4); BUN increased: serum crythwpoiedn clcvated.

Imaging hT:displacement and distortion af r i g h ~pelvicaliceal sysrem.

CT,abdomen: mmnr at-isirrg from right kidney with areas of
low densirv (due to necrosis) ; persistent ellipsoid arm of-
enhancemen t (dile t o compressed renal par~nrhynla); no
widence of vasculal- i r ~ v a q i o ~ ~ .

Gross Pathotogy Whitish, solid rumor wit11 awaq nF hemorrhagic ~lucrosis

distorting normal renaI parenchyna comprew~dinlo narrow
rim; may. he involvement o f perirenal fat; metastasis 11sualIyto
lunp.

Micro Pathology Glomcruloid and t~thulnrstructure5 enclosed within spindlr cell

stroma; areas of carrifage. bone, or striated muscle tissue.

Treatment Surgical rcmov~lof kidnev con raining tnmnr: chemotl~erapv

with actinomvcin D and vincristine; radiorhelapv.

Discussion Ncphroblastoma (WILMS' TL~STOR) is a malignat~ctumor of

embryonal origin. 11 is associated with deletions on chromosome
I lp involving the WT-1 gene and should he diffcrcntiated from
~ ~ e u r o h l a ~ t oar n da malignant Ivmphomn, which are other small
cell turrlnrs of childhood. WAGR syndrome consists of Wilms'
tumor, aniridia, genitor~rinaryal~normalitics, and mental
retardatinn.

WILMS' TUMOR
 IQ/CC A 4.5-vc.ar-old whitc remale is r r ~ s l ~ utod the OR because of shock
rlne 50 postoperative hlccding; during in tl~hatiorl,she vomit.;
and aspirates thar day7shreilkfast,

H P I She had 11nde1-gonea cholcn~stectorny2 d;~vsbefore atid had

presrn tcd with postoperative blecding requi~ingsurgical
explorr~tio~~.

PE 1's: tachycardia: tachypnea; fever; hypotension. P E central

cyannsis; warm, moist skin: intercostal retraction; inspiratory
crepitant rales heard over both lung fields.

Labs CRC/T)BS: marked leukocytosis with neuh-ophilia; hagtnented

TU3Cs: thrornbocytopenia. ABGs: severe hypoxemia with no
improvement on 100% oxygen. Inrl-e.~qerl131TN and ct-eatinine;
i ncr-eas~rlAST and ALT.

Imaging CXR: typical diffuse and symmetric parahilar ("hat-wing"

pattern) alveolar fitling process suggestive of noncardiogenic
pulmonary edema.

Gross Pathology Fomatioll of hyaline membranes wirh proteinaceous deposits in

i~lveoli:pulmonary edema will] red, heal? lungs which,
comhirter? 1vit11 widespread atelectasis, produce stiff lung with
fiht-045.

Micro Pathology 1711dr)t I-rrIial and ;~lr.eolocapillaryclilmagr rvirh edema, hyaIi t ~ e

memhrane formation, and inflammatory infiltrate: loss of
surfactant with fihsohlasr activity in later 5tages.

Treatment .Mecha~iicalven tila~ion,a n tihiotics, steroids, close monitoring of

I l e m o d ~ ~ a r nfi~nction.
ic

Discussion Adult respirarorv diqtl-ess synrtrome is a corldition that i q

;issociatecl with high mortality; i t is caused by gram-negative
sepsis, mawive trauma;burns, dissemina~edin travascu1;lr
coaguIat inn ( DIC) , acute pancreat i ti?. r~arcotic overdose, m d
n e a r - r l r w n i t ~ g .I t i~ characterized 1 3 ccliCF~rw
~ alveolar capillary
in,jurv. ~ I - t i c hIcads t o a n incrcasc in vascular pcx-mcitbilivand
p ~ l t t ~ o t ~ aeld-eyt r ~ a .

Atlas Link

? ADULT RESPIRATORY DISTRESS SYNDROME (ARDS)

 ID/CC A 65-year-eld male prrsents with pmgreasively increasing c011gh
and dyspnea r)n exel-tion.

HP I We i~a retired construction worker anrl has a nca1.1~

L OO-pack-year
smoking historv.

T
PE VS: normal. PE: pxtle TI dubbing; lirte c ~ a c k l eauscu1t:rtt.d
~ C
hilateri~llyover lung bascs. 5
0
z
Labs CRC normal. PIT<:niixetl ohrtrllctivc anrl restrictive diseiise pat-
t ~ r t l reT111ced
: Dl+tl- M i ~ t - n s ~ ocxam
p i ~ t ~ sputtlrr~
f re\+ealsgolden-
brown beaded rods (~szsn~srt,s onnres) composerl of asbcstor
G ~ P I - Fcoated wit11 an iron-con raining protei~l;+cer>t~q rnaterinl.

Imaging CXR: ir~reglilar.lirtear, interstitial infiltrates in 1owc.r lo1)es wi 111

. CT
cirr l t tnscri hed racliopaqiae cl~nsitirs( ~ ' i . ~ u ~PL~QLIES)
,it.
(high resolutia~l):posterior anrl Iatcral p l c ~ mthickened~ with
calcifiecl plaques sren bilaterally.

Grass Pathology D i f t i ~ s epulrl~or~ary

interstitial fibrosis with bilateral pleural
calcification and ~ l ~ i c k ieng
n and i~~volvcmcn I c ~ thu
f rliaphraatn.

Micro Pathology Calci~iinconrainingdmsc plruraI opaciries ancl plaqltes of

cnll;lge~i
; askesrc~ql~nrlies.

Treatment Suppnl-tive anrl syrnptnt~~at

ic treanncn t (oxycn. I~~-onchodilators,
antihiocics):prevention of h t h e r exposlue: smoking cessation:
counseling re~ardir~g high risk orbranchogenic carcinoma and
malignant mesotheliorna.

Discussion Prolonged rxposr1r.c to asbestos in ~igt~ificar~

tlv curnulatir~close.;
results in pulmonary parenchymal scarring. This process is seify
pc-rpetuating, 1x11cessaiior) 01 eupuullr may slow rliseastl prc-
gre~sion. Cnmplica tion5 itlcl~idebranchogenic carcinoma,
malignant rnesothelioma, cor pulmonale, arxl death.

* ASBESTOSIS
 ID/CC A 10-year-nlcl girl is brought illto the ER in acute respiratory
distress.

HPI The patient is known to 11r allergic to cats and pollen: her
rnotller stales that she had a recent URT. She also c-omplai~~s
of a
hil;tot-vof moderate intermittent dpspnea that is exacerbated by
exercise.

PE VS: n o fever: hchypnea (KR 322);RP: nor~nal.PE: inspiratory and

expiratory wheezes (due to hronchoconstric t i m , small airway
i nflammatini~)
;h n ~ g y
a n d pale nawl mucosa; accessory m d e
use during hreathi ng; enlarged ch esz AP diameter; hyperresonant
to percu~sion.

Labs .kRC;s: primarv respiratory al kalosiq (h per-uentilarion). CBC:

eosinophilia (1 3 % ) .PFTs: low FEV, /FLrC.

Imaging C a hypesinflation with flattened d i a p h r a p s (incrcasecl residual

vrdurne rlnr to air trapping);peribrontliial ct~ffit~g.

G T ~ S SPathology Hyperinflation with air trapping in alveoli; plugs of inspissated

mucus: erIenla of m ~ ~ c o slin
a l itlg.

Micro Pathalogy Inilammatory i ~ i i l t r z t eor bronchial epithelium. m;tir~ly

cosinophilic: plugging of airways with thickened mum5
(CL~RSC:HM.~NN'SSPIRALS) ; I~ypertraphv of rnllcolls ~ ~ a n d s :
~longatertrhomboid crysmls derived from ~ o s i n n p l ~rytopIasin
il
(CI~PX(:D.I-LFM)FN I : R w ~ . ~ L:. hyperplasia
s) oC smooth muscle of
bronchi.

Treatment Tnl~aled,01-al, and parenteral hronchndila~or~;

steroirls:
r l - o m o l y : ralirlltkast.

Discussion Bronchial asthma is characterized hy hyperreactivity of the

a i r w a ~and ohstrr~ctiondue to hronchospas~n,edema, a n d
mucus. I t is also knowm as reactive airway disease.

7 ASTHMA
 ID/CI: A 5l).yea.-old white male de~~elops
a fever 24 holm aFter surgery.

HPI He underwent an emcrgenq lapamtomy for n perforated

~ ~ tintraoperativc or immcdiatc postopera-
peptic ulcer ~ i t i l o any
tive complicationq.

-0
PE VS: fever; BP normal; tachypnea; tachycardia. PE: no cyanr,sis; C
scattered rales and decreased breath sounds;n o calf tendcrncss; 5
-
0
no liematoma or discharge Crnm woiuld: n o inflamm;~tionof Z
lV linr veins: n o urinary svmptoms. X-
70
<
Labs ABGs: mild hypoxemia. GBC: sl iff11 t nea trophilic leukocvtosis.
R h o d and spliturn culture sterile. ECG: sinus ~achycardia.

Imaging CXR: dense opacity in right lower lobe (collapsrd lobe) with
elevillion o f right hemidiaphragm (due to volume 10s.;).

f reatment Chest phyqiotherap); (incentive spiromrtry): dcep in~pirations;

m~tcalvticagents.

Discussion Poxtnperat i1.e atelectasis is i h e mnqr roinlnoti cause nf postaper-

ative river- in the first 48 hours; alveolar collapse is produced hy
o c c l ~ ~ s i odue
n to viscid secretions hvorcd by rccumhcr~q,
hypovon tilalion, and oversedatior~.Other-causes of pnstopet-a-
l l v later in the postoperative period, include
tive fever. l ~ s ~ ~ aseen
UTI, IV catheter infection. deep venous thrombosis, wotlnd
infection, and clrug reac~ious.

ATELECTASIS-POSTOPERATIVE
 A 1.1-ycal+-oldmale pi-esents with complait~wof exertional
dyspnea, chronic productive cough, and occasional hemoppis.

I-lc was d i a p o s c c l wit11 cystic fibrosis at agc 4 and !ins Iiad

recurrent pulmonary infections r c q ~ ~ i r i nf rge q r ~ c n t
hospitalizatinns.

1's: Ir>w%ratle fever. (38°C); ~ a c h y c x r l i a(H R 1: 113): t a c h y n e a

(RR 28). PE1: pallor and grade IJ clubbing nored: coarse crackles
a ~ ~ s r ~ ~ l t river
a t e r l110th I11119 iielrl~.

Labs CRC: norrnocytic, norrnochromic anemia: low hernatoctil.

S p ~ ~ t trultrlre
nl reveals S ~ ~ J I ) J ? / T I ~nI -r rI rTnIr,~I .Pms: clecrtlascd
F W ,JFC'C; ~uggestiveof oh~truc tive pathnlov.

Imaging XR. chest: incrcasecl l~ronchc)vascularm a r k i n ~ s honeycoml)

:
l c e I(>PIICI-(111~hadow'iof dilated hrr~rlchinles);loss
a p ~ m ~ r a ~(dur
of lung volrmle (atelectasis). CT (high resolutioi-i). chcst: dilated
bronchi0Itz-s with "'signetring" appearance ( duc ti3 adjacent
hranrh of puI~nonal-7artery).

Gross Pathology Lot~g,tubelike, irre\~ersihlyrlilated bronchides extending to the

plcrlra with loss of l n n g parenchyma.

Treatment Supportive measures; antibiotics; bmnchadilators, cxpeccorsnts,

a11d physical therapy lo p r o ~ n n tIworlr
~ hial dl-ainage. Surgery
may hr indicatccl lor localizccl or segmcn till hronchicctasis or
when medical flel-apy hifs.

Discussion Dilatation of thc I~ronchialtrer Icads ro infections and to fnr-

illel- il-reversil>ledilamt ion. Under-Fving callses inclt~rleobstruc-
tion clue m tumor, foreign hodics, anrl r n ~ ~ c impaction; z~s
congenital disorders such as k t a g e n e r ' s syndrome. 1~'iIliam~-
(hr~~pl>pll S Y I ~ ~ ~ O ~ ancl
I I C ,cystic fibrosis; i ~ n dinfections due to
Ilm?~dr*~r~/lri ~ tnpvit.1 IS.RW. n1ea4 es. and R'lymt)rzr/priwm 111-
/ ) m / ? ti+,
I ~ ~ ~ t r l n rComplications
i.~. iticludc lung abscesses, metastatic brain
abscesses, arnyloidosis, and cor pulmonale.

BRONCHIECTASIS
 ID/CC X 60-year-old male is rukrrud to arl i~llrrgistfor late-onset
asthma rhai has been unresponsive to bronchodilators and
antibiotics.

HPI tlc 1x1s also been hming chcst pain ( n ~ c i ~ nf i)l i, g ~ wanorexia.
.
and pain in both calves ( c : r , \ r ~ n r r m c ~(111
~ )exer-lion that are of
r c c c n t onsrt.

PE a ; fever; mild hypertension (I3P 15n/ 100)

VS: ~ a c l i y i ~ emild
(secourlal-y to renal vascular involrcment). PE: mai-kcd
respiratory diqrrew; rvidespread wheezes hilatcrallv; numt.sc>lis
purpuric lesions on feet (rI~rrto clrtancotis small vessel x~wulitis).

Labs CRC:: mild at~enlia:leltkocyto5is (> 10,00O/pL); Hct < 35%:

( > ?P00.000/pL); eosinophilia (> 1000JpL).
r hrnml~ocvtnsi~
Elevated BUN and creatinine: P-kVCA positive. LTA: proteinwia:
prewnct of RRCs, SITRCs. and granular casts. PWs: FEV,/WC.
ra~ictreduced (obstructivepulmonary disease). EGC.T. hll1os -'

tachycardia.

Ernaging CXR: bilateral upper and fewer lobe infiltrates i ~ t ~noncavimt-

d
ina nnclirles.

Gross Pathology t,unq ~ h n w she~nol-rhapyicinfarcts secondary to thrombi in

afecwrl artel-ieq.

Micro Pathology Tran~bmnchiallung biopsy shows ganulomatol~s;lesions in

vascular and extravsrsclrlar sites accompanied by intense
eosinophilia: skiit biopsy nf ~ > l ~ rt-ic
p ~lesions
't shnws vasculitic
lesion-fil~rinoid necrt,sis of mcdia ~ i mixttiru ~ h of irlflamtna-
lory cells extending along arlvetltitia; octxcional :Inenr~smsand
seranrlary thmnihnses wen; tltc artcrinl in tcrnal clastic lamina
i~ clestrovrrd and intima and mcclia arc thickened.

Treatment Pcednisolone effective in i n r l ~ l c11i g remiwion: cyrlophospharnide

l those refractory to srcroids; monitor disuaso c o t w e
u ~ e rin
l~sirlgESR leveLs.

Discussion C;h~~rg-Strxusssvndrolne is an idiopathic systemic small- and

medium-vessel p u l o m a t o u s vasculitis (grouped ~ vthi
pnlval-teritis nnrlaca [PAN].ivflic1l cloc?i not i~~vr)lrre
Iizngsl thal is
cliararterizrrl hp a triad of Fate-onset asthma. a fluctiii~ting
eosinophiIia, and an extrapulmonary vaqc~ilitis.

CHURG-STRAUSS SYNDROME
 ID/CC A 5(T-year-oldwhite male smoker presents with productive
cough, copious sputum, shortness of breath, and fever.

HPI The parient has a 40-pack-yearsmoking hiqtory. Hc has also

.experienced chrnnic dyspnea on exertion; chronic productive
cough. usually in the mornings. for several years; and niultiple
colds each winter.

PE VS: fevcr. PE: stocky build with plethora; whee7es

Labs CBC: elevated IVBC cout~l(14,000); neutrophits predominant;

secondary polycythemia. Str+tnrocrix~ p n m moniw o r Hnmophib~a
i?lflurmwo n Gram stain of sputum sample. Al3Gs: decreased
Po?; ~ l e m f e cPcn,.
l PlTs: decreased vital capacity; decreased
mv,.
Imaging CXR: increased bmnc11ovascuIar markings in lower lung fields.

Gross Pathology Thick mucous secretion: cdema of hmnchial mrlcosa.

Micro Pathology Incceawd size and number of mucous gFands (Reid's index
> 50); inflammation: fibrosis; sqlramous metaplasia.

7T ' *
COPD-CHRONIC BRONCHITIS

- - -. -
 A 55-)~eawldninle complains or progl-essivt=l~, incl-c-asing
shortness o f breath on exertion for t h e past few ~nonths.

Hr x l w ~romplains of a nonpl-oductivc mild cough ;111d11as a

40-pack-year smoking history hut 113s no hiqtory or hemoprysis
or uccupa tiunal esposm-e lo inorganic or rrl-ganic dusts.

15:mocIcrate rachvpnea. PE: moclel-ate respiratorv distress;

using accessory muscles of respiration; full ncss of neck veins
during expiration; chest barrel-shaped pemzssinn nole
hyperresonant; cardiac and Iiver duIlness are obliterated;
scattcrccl rhonchi 1ljlater;llly;heart sound* heard distant hut
normal.

Labs XBGs: mild llr~oxiawith respiratorv ;~lkalosiu.PFTr;: ir-tcreaserl

r c ~ i d u a~l ~ o l u ndecreased
~e; FEV, /FVCratio (oncr 1r1 11.1.:IYISFA~E
PATTI:RN) ; decreased DL.,,.

Imaging CXR (PA view) : hyperlucent lung fields with a few b~~llae:
flattening of diaphragm ancl elongaied tlthular I~eat-tshadn~v.

Gross Pathology Air space5 dilared; tapper lobes most affected.

Micro Pathology Pattern of centrilohular emphysema: alveolar sepra arc visiblv

clilrlinishrd in number along with increased air spaccs.

Treatment Ccssatiun of smoking, brc)t3chntlilatois. s~ptmidsit1 resistant

caws, ar~tibinticqcturing acute esacrrhations, and h a m r u x y g c ~
t herapv.

Discussion Ernpkyselna is defined as ahi~ormalpel-n~anent enlargement of

t h r air spaces distal to the turmii~alhmnchiolr arcnmpanierl
tllr desfruction of the alveolar wallf; ~mphvseman ~ a yinyolye
r he acinus arid the lohu te unifnrnilv in a pattern cal1c.d parmcinar,
or i t m w primariIv iilvolve t h e respiratorv hronchir~les,termed
cell triacin;~r,Pi~naci tlar enlphvqerna iq C O I I I I ~ O Iin
~ patie 11ts with
a,-antitrypsindeficiency. Centsiacinar cmphyiiema is con111ionlv
fout~rlin rigzarette smokers and is rare in nonsrr~nkers:i~ is
~15ualEymnrc rxtcnsivr and qcvcre in tlie upper lohes.

Atlas Links

COPD-EMPHYSEMA
 I DJCC A 37-year-old male in t h c ICU devrlops petechiae, altered
sensorium, and marked dyspnea that pl-nve rprracta1.y to oxygen
thcrnpy.

HPI Twenty-Fourhours a5o311u %$asaclnlittrd ro the hoqpital with

fractures of the shafts of both femurs, the pelvis, and the ri@t
humerus, rl~stainedfollowing a fall from a 20-fool-high stepladder.

PE marked <dv.ipnea.PF,: delirium; tend cyanasis; u?ing

VS: rt=~~t.r;
accessor-v ~ ~ ~ u s r01'l erespiration;
s wheezing heard over both Iirtlg
Cielrls.

Labs ABGs: profound arterial hypoxemia with hypercapnia.

CRC:/PRS: i1ii.ntn bocyrop~nia.Fat demonstrated in urine and
sputum: normal PT a n d PTT.

Imaging CXR: earlv, normal; later, bilateral perihilar ("R.%T-M'IKc;'*)

appra~'ancenf pulmonary infiltrates withnut carcliomugaly (due
pulmonary edema). r;R.plain: Ion%l3one
tn r~oncardinge~iic
rr:ici~~res.

Micro Pathology Obstruction of pulrnonar y vessels by far glnh~r!eq;chemical

p ~ ~ r u r n o niq.i l

Treatment Intrrmittrnt positive pressure vcr~tilationwit11 190%nxxygen,

supportivr managurncnt.

Discussion Fat cmbolizatinn usually occurs 24 to 72 hours after fractures of

the shafts of the long bones.

Atlas Link PM-P2-082

FAT EMBOLISM
 IDJCC 2 1 .in-year-nlcl farmer p r t ~ ~ e nwith
t s sei7vrVshortness o f breath
( I ) W P K F , ~a11d
) fatigue.

HPI Hr also complnins of'n dry cough anrl mild fever. His synptoms
a1.r ex;icei-bated when h e wnl-kl; in tile fields. c~pr~ciallv
T ~ . ~he
I~II
cortles inlo canracr rviih moldy hay. He does not smokc and
T
drinks alcnhol occasionallv. C

tabs CEC: Icukncytosis ~ i t shifth to IrSt. Elcvi~trdESR; serum

antibodies against thermophilic A c t i n o rnp ups organisms:
hronchonlrcr~larl a n g r sllr~~cu ni:lr kctl I~rnpliocvtnqir, primarily
rirppl-rc.;oi--cytrhr)xic T r.ell5. P l T q : restriccivc lung disease pattern.

Imaging G S R : bilateral retimlanodular inFi1trates with fihrnsis. CT: al-eas

of grour~d-glassabr~orirlalitiesw i t h c r n ~ r i l o b ~ ~perihrnnchial
lar
nodule.;.

I GTOSS Pathology

Micro Pathology
Fil~rosiswit11 honeycombing.

B~-oncl~osct~pic I~iufiI>ir>psvr c ~ e i ~ interstilia1

ll; piier~tnr~iiia
with
I \ - m p h o ~ i canrl
s plnslnii c ~ l l sin nl\,eolal-walls as well as
sca ctei-cd g r a n ~ ~ l o l nwith
a < Coreign body ~ i a tnccllq.

Treatment Srricr ay(>idanccof cour;~ctwith a ~ p e r g i l l spores;

~~s stesoiris.

Discussion Ftypei-sensitivity pneuri~urtitis(;rllei.pjc.;~lverstitis) rrfrrq In

in tcrstiti~~l Iung dircnsc thnt results 1rt)m inhali~tiouc ~ organic f
a ~ ~ t i g r nHyersmrilivizy
s. p i ~ e ~ ~ r n icq ~i ci !>clievrcf
~ir tr) h a w a n
irnrri~~r~olugic hasil; (t..g., qlutc~xic.irnmtule cvinplr?r, aild cell-
t~ie~liarerl reartinns) ; the most common Corm of hypersensitivity
pneumonitis, called farmer's lung, is caused by inhalation o f a
thermophilic A ctisnmjlces organism present in moldy hay and
grain. C)t her cornrlro 11 cauqes nl t ~ p p ~ r s e nt ivi s i ry pnrumonitis
i n c l ~ ~ rpigeon
le Im-errIer's rlirease anrl Iil-cl Cancier'~clisrasc, in
which in halcd qcr-run protcir~sfrom pigeons or ~,a~-akt-eb ind!~re
thc svi~rlmme.Huinirlilicr luilg rtiwasc ~ - c s u lii-om t ~ cxposurc to
cont;~rninaterl Forced-air svrtrliil;.

* HYPERSENSITIVITY PNEUMONITIS
 A Ei5-yrar-c1ld ir~alecolnplai~irnT pl-ojircssivc. shortness r l t ' I~reath
on exer-tion and a chronic dry cough.

"The prjtient hns never smoked cigaretrr~and has n o hirtory of

exposure tn occupational dusts or fitnles; he has tint had a
procluctivt. cough or-hrmoprysis.

VS: rvann but cyanosed: tachycarrlia (HR 108); tac!l)ipiiea: RP

nor-ni;\l.PE: clubbing present; Jl'P not elevated: heart sounds
ilormal with no a d c l i ~ i o ~sotincIs
~al or 1nltr.rnul.s;respiratory
examination reveals presence nf hilatcral basal fine inspiratory
crepitations.

Labs hl3Gs: hypoxernia. P!LT5: decreased DL,,: clesaturatinn with

exurcisc: p~-oporlionalely redt~cerlFEV, ancl FVC so that ratio
rc=rriairlecl~lnchangecl(due t o rertrictivc cli~ease).
l~rnnrhoalrenlarla~ngepredominiinnrly neutrophilic; serum
c;~lci~inl ancl /ICE levels Eo~v.

Imaging CSR: reticiilo~lodnlai- shadow in both lower lung fields with

ncc:isir~nalarea5 of "honeycombing." CT (high I-esolt~tion) :
lihrosis in Inwer lung 1ol)es suggestive of lislial interstitial
pne~unonitispattern of FPF.

Micro Pathology Rronchoscopically obtained lurig biopsy rwealc; presence of

6hro3is, inflan~matoryround cell infilrrare, ant1 thickening of
chc alvcolai- sepba.

Treatment

Discussion T h e rnain differential diagnoses to consicler are lung fibrosis

aswciatetl with a cnilnective tissue disorder (rule nu1by history
; ~ n dclinical c-xam), extrinsic alveolitis due to organic dusts, left-
sided Elcart failure, sarroidosis {rule nil1 otr the haris of ahscnce
of anv othrr svsrern invofvei~~enl, nnrmal calcium and ACE
lcwls, ncgative Kvei~n'stest, a n d lack nC hilar 1yrnph;idcnopatliy
ohrerved on CXR), lymphangitis carcinomatosa (rtite o u ~o n
biopsy 2nd CT). a r ~ pneurnoccrnic>sis.
l The onset of idiopathic
pulinonat-v Fihmsis i s typically in the fifth or sixth rlecacle.

Atlas Link

I D I O P A T H I C PULMONARY F I B R O S I S (IPF)
 lD/CC ,4 .?Rye-ar-oEd trlalc prcsentr with shortness of breath (rnl;tlut,~)
hoarseness, cough ancl hemoptysis.
:h,

H PI H p has an 80-pack-year.smoking history. O v r r - thc past 2 mor ~ t s,h

hc has a l ~ oharl a significant loss of appetite and weight.

T
PE Marker1 pallor: cachexia; clubbing: mild whet-zing at r r s t : c h r s ~ C
barrel shaped (ernphysrrnato~t~) ;mrl nlovemrnlq rliniiniuhed o n 5
right; dullness to percussion rtyel- ~.iqht111iilclle Inl>c;no breath o
z
sounds Ilea1d oyrr r-igh t middle Ir~he:vncal freiiiitlis rerlucrd in B
\:1111e ;il'eil.
7
tabs CBC: normocytic, normochrornic anemia. Clixrn a n d ZN r t x i n ~
rlf' spltturn fbr acid-f*ilstI~ilctlli neg:u ive: ylzlturn c l ~ c d rcveals
o ~
presence of malignant squamow cells.

Imaging CXR/C:T: i r r c ~ i 1 a 1hililr

- nlass on tigfll side. pi~odiiring:III
ohsunttiun iitrlec~asiurlf r-ieht micldlp Inhe. Rroi1rhosct)pr:
righl-sided liilar mass nhauucting right middle h r o n c l l ~ ~ s .

Gross Pathology Pnnnu-gical sperimeil 1-eveaI4an irl-cpl;lr inv;tsivr m;ics o f

tumor spreiidillg 0111 Fr-oril rig111tniddle hronchns
g~~"yi$li-tan
I a i ~ du l ~ ~ t r u c t i nil.g

Micro Pathology Diop~yrevcaIs prcccncc oC n~alignancsquamaus rc511$,ccllit!:*r-

qtmtificatior~,intercell~llarbridges, anrl "keratin pearls."

Treatment Surgici~lrcwc tion can hc p o ~ c n ~ i a Icurative

lv in par ien ts wit 11
nun-smalIccll 111112 C C I I C ~ T .

Discussion Lung cancrr is l h r most preventable cancer. Owins In rhe

i ~ i c r e a ~ eincidellce
d r)i srr~r>l;irip.
I i ~ n gcancel- has r x c ~ e r l e d
brrilst carlrer a.i the Iradi~rgc a u s r c~fcancer rteath irl women.
h Pancoast's trlmor i~a lilng nlnlor Ioci~tcdat tllr lu11,q ttpex in
~ h superior
c p ~ r l m r ~ n a r.;t~lc~tr
y tli;~I caltses corn presqintl nC the
crr.vic.al syrtipa~hc~ic plex~ls,resl~ltingin Horner's syndrome
(ptosis. niimis. anI~idrosis):IT well as sci~pt~lar paill a ~ ulnard
ncrvc radiculc~pa~l-ty.

Atlas Links r'lPM-P2-085,PG-P2-085

? LUNG CARCINOMA
 A 67-yrar-nlrt tnak is referrecl to a clinic for evaluatint-i or
pleuritic pain, weight 1 1 ~ 5pacl~ri~Fly
, progre~sivedyspnea, and a
nonproductive cough of a few rnon t h ~ rlz~ration.
'

H e worker1 in ;I shipyard ror 20 years bcforc retiring. an

orcupation that involved asbestos exposure.

VS: normal. PS: clubbing of fmprs: mild c!lannsis; reduced chest

expansion; end-i~~spiratory rnles auscultated over bath lung fields;
c l d percussion, reduced breath sotmdq, and egophony in right
side { ~ t lto~ p1~1lml
r errtlsion).

C:RC/PRS: pnlycyt hernia; marked eosinophilia, PFTs: restrictive

pattern observcd (dccrrased vital capacity and decreased total
l t r r g capacity i\.ilIinr)rmal FEV,/F'VC; razio). Rcduced diffusion
capacity; pleural efftl~ionbloody a11dshorvs acidic pH (< 7.3).

Imaging CXK: rigllc-sided pleural effrisiou; diffuse bilaleml interstitial

fibrosis; parietal pleural calcifications. T=T: highly i r i - u p l a r
p lel~r-al-hafedrnasseq; 11 e n ~ o s s h a g i ceffusion.

Cross Pathology Thick, fibrous pleural plaques with calc=cation; diffi~se

interstitial fibrosis: asbestos cc~rnpol~nd\
Form nesr for further
c?epnuiiir,n nf il-or1 saltq and glvcoproteins ( m ~ l i r " r . r ~ c >ASRPSTOS
us
l3( ~IIIT:~).

Micro Pathology Epi tlrelioicl pattrrn of pletu-al malignant sarcomatous

t l - a n s f i l r m a t i o n with cellular atypia and high mitotic itldex.

Treatment r y : prognosis.
S l ~ s ~ ~poor-

Discussion Occlipationd exposure to asbestos is found in 80% :,C cases of

malignant mesotheliorna; it produces lung fibrosis wit11 a:
restrictive pattern. Ashcstos and tobdcco expnwr 1-e syn~rgisticallv
i n c i - c a s c thc risk of lung ~ ~ L ' I I O C ; I ~ C ~ T ~ O I I ~ ~ .

Atlas Links

MALIGNANT MESOTHELIOMA
 ID/CC ,;\ 57-yeal--old remale comes ro the ernergcnv room complaining
OF pIeuritic pain o n the r i g h ~side nf llcr clievt i111t1dyspnea
logether- will1 fever- ; n ~ d;ip r c ~ d l l c t i ~co~lgh.
e

HPI T I i ~ r eis n o hemnptvsis. T h e pail? i q nqicallv sharp and stabbing,

a n d it nriws when 511e takes a deep Iwearh I K I ~ ~ ) .
(r31.r:t

PE Decreased chest movement dtuing inhalation oil right side; f

0
dullness 011 percus5ion orright Iung base: reduced or absent z
breath sounds ores right lnng haw: I~ronchialI>rcath sounds g
<
i n ~ s c ~tatctl Irrar ir )II r lf dullness
t l 011 rig11t side; fr-iction ri~l>:
moves with respiration; decreased tactile fremitus ovei- right
lung.

Labs CBC: clevatrcl U'EC count with prerlorriinancr oi'neutl-nphils.

Gram-poriiive tliplr~coccinn qplltllrn smear and clzlt~lre:elevated
protein, decreased glucose, and many neakophils in pleural
exudate.

Imaging CSR: c o n ~ l i d a t i o nof ri$t lower lobe: p1eur;il rff'i~sintlon right

side. XI<. lateral ~ I e r ~ l ~ i layering
i l ~ s : of fluid (tllereforc not
loculated).

Treatment Antibiotics ailcF ncecllc drainagu nf cffitsic~rl( T H O I L I ~ : I ~ ~ K ~ ~ - F . ~ I ~ } :

tc~inrrtimerol>litrl.a~i o t ~nf plrllral space.

Discussion Plctrri~lcfft~sionsrrlav be d u e lo iril'cc~ion(viral. har rerial,

nlyrolmctc-rial, h11iq1):o t h r r ciillsrs are ttlal ig11:111cies,
congestive heart fililw-e, cirrhosis, nrphrotic synclrvnlr. tmirna,
p a ~ i c ~ - ~is,a tcollagen
il tfi5e;irt.r. atlrl tf!-~lqreactioils. E1T11sions111m
Ile transudative (< 5 ~ / r l LaT PI-otein) or- exudative (> 3 g JdL of
prolei 11). F,I~vatedpleural flilirl LDIH Irvcls mar. he s~~ggustive of
n-lalignarrcv.Tms~adativepleural efrusions arc cornmonty
citusrd hy conges~i\.ehyart failure, cil.rI~n\is,ant-1 nel~hrotic
svndmmc, wherea~exudative pleural effi~sionsare c;titsed by
TB, infections. malignancr: panel-ca~itis. pulmonary emlmll~s,
a n d chvlothnrax (milky pleural fluid).

PLEURAL EFFUSION
 ID/CC h 2-i-yrar-r>Idu-hitc male complains or sudden pIe~triticchest
pain and shortness of breath that awakens him at night.

HPI I-Ie smokes onc pack of cigaretteq a clay and states that his
patcrnaI uncle once had a similar episode.

PE Tall, thin par i e n ~diapl~oretic

; and fuels wcali; lrft chest expands
poorly o n inspiration; trachca and apex heat dirrplaced to right;
left qide hyperresonant to percussiorl: decreawd breath sounds;
demeaqed tactile fremitrrs.

Labs r\RC;s: rlecl-eased Po,; elevated Pc:o,.

Imaging CXR: partial collapse of left 111119with no lung markings excupt

thin line parallel to chest wan; costaphrenic sulcus a b n o m d y
radiolucent ( ' ' I ~ E PP S I ~ L . C L ~SICS)
S" in supine film.

Gross Pathology Types: rraumatic, sponmrieous, lension. open: common causes:

s~irgicalpuncture, rLiplilrr of eniphvsematous bullae, positive
pressure mechanical vet~tilatinn.hro~~choploural fistula.

Treatment Pnrumothorax wacuatiori via p l e ~ ~ r a

catherer
l (r,~-rr:s~
TUBE).

Discussion TI he (rsual cause of spnn taneous pneumothoras is rupture of a

subplerd ble b .
 fD/tC X -10-vrar-old III;IIP is Ijrn11g111to [ h e ER nit11 cornplaintu c l f
sudden-onset. severe right-sided chest pain followed by sevcre
dirfic~lltybreathing.

HPI Hc i\ ;I c h ~ n t l i rsmoker a n d has prerlolninal~tlyemphysematous

COPD.
-D
C
PE I'S: .;cvurt=t;~chrcal-dia:t;~cliypnea;l~yporrnsinn:n o few-r. PE: 5
0
cyanosis;trachea shifted to left; cheqi exam r ~ v c a l s z
l~yprrresonantpercussion note on right, d t n i s h e d breath >
7~
sounds. ;inti decreased tactile fernih~s. <

Labs 1\113Gs: h!puxcmia: rc.;piriltor alkatosis. F.C:C;: nnrmal.

Imaging C X R (afttr- paticrzl 41 ahil i;.es) : right pneurnothorax compressing

Ir~ngparenchyma and shifting of mediastinurn toward left.
Flattrnecl lcft h c m i d i a p t ~ ~ + a ~ m .

GKOSS
Pathology Plcnral 5piI.c.c i u lilletl with air anrl 1n1tgis aaelecta~ic( t o
r l r r n o ~ ~ ~ t ~r ~ atl -aurupqv,
i ~~~eeu l n c i t h o a~ thc c11c.ut r.a\rilv is
npentd illnrlcr watcr, lettiilg ail huhl~lesrrcape).

Micro Pathology S~ctir,noI I i ~ n aslinws collap~ecla1vrc)lar spnccs.

Treatment Immerlintc- Tife-saving trcxlment conqihrs of'illsrr~ing- a wiclv-horc

If' c;~nr \ l t l ; i
o i l I l ~ ai7kriecl
e sirlr I(> rlecor~~prcss
thc pleural ci~tqtv
if ;I ~ I I P C Idrain is n o r irnmcdiatelv availaMe; rhr wiclr-hnr~
neetlle call then hc rep1:lcetl h~ a che\r drain cot~ilectedto an
nncln'watrr ~ r ; l l .

Discussion 111 ~eilsionp~wrimntllorax,air entPrs thc pleural spacc d ~ ~ r i i i g

inupiratinn anrl i s pl-eve11~ r from d cqr-aping cl~rringcoxpir-alio~l
{ l > e r s ~at1~ . ;airwily
~ o r ~ ~ S F I Iflap
C valve): rhel-ei s
acts ;IS ;-I onc--wa~~
a pnq~,?-ossivt. incrc-nw it1 [ ~ l e u ~air:
x l whir11 is unrlcr pressul-c (i.c.,
t e i i ~ i o ~Tl )e. t ~ ~ pne11inot1lcrr-a~
i~n occllrs in onlv 1 tto 2% o r

common manifestntiun of ilic barnts;inma. [ha[ m:m orcllr rluring

prnitivr prcshure mechanicitl ~ e n ~ i l a t i nKt s~k. L:lr;rt)l-s for
~poiltxr~eotl?p r ~ c ~ ~ r n o t h o rincludc-
+ax COPD, c y t i c Cil~rclril;.

7 PNEUMOTHORAX-TENSION
 ID/CC A 34year-old white obese female complains of shomess of
breath. dizzirloss, and near-hinting spells.

HPI Shc has bee11 lltkirlg prescription medication for approximateIv

6 won ths in order to lose weight.

PE Obeqitv; ~ ~ l iqanosis;
ld large "a" wave in jug-uIar venous pressure;
parastcrnal heave; loud S2: narrow splitting of 52; mles on both
bases; hepatornega lv.

Cabs CBC: polycythemia. ECC:: right-axis deviation; right ventricle

and right atrial hypertrophy, rLBGs: hvpoxemia.

Imaging CXR: enlarged light vcntriclc.: rnlarged tnain pulmonary artery

with peripheral pruning.

Gross Pathology Enlargccl right ventricle with mvocardial fiber hypcrtropky;

atherosclerosis or pulmonary artery; narrc3wing of al-terioIes.

Micro Pathology Atheromas in main elastic arteries. Thickening of the media and
intima in rnedilinl size m u s c ~ ~ larteries.
ar causing near-oblit~ration
uf the lumen.

Treatment Calcil~mchannel blockers: prostacyclin; inhaled raitric oxide;

phlrhntomy: heart-lung transplaillation can he cnnsidered.

Discussion Primary plxlrnonary hypertension is a p a t h o l a ~ cincr-ease in

pulmonary artery pressme; if long-stanrling, i t raines latit1 right
heart L~ilurc.It may he- primary (irliopathic) or secondary to
intrinsic pulmonary disease.

Atlas Link U C D I PG-P2-090

P R I M A R Y PULMONARY HYPERTENSION
 A ti(Lycar+lrl female who had ~ ~ n d c s g o n e total hip
right
replacement presents on t h e sixth pn5topcrativc day with ccnlral
chest pain and acute-onset dyspnea.

She has been immobile since the wr-gei-y.

13:l o ~ ~ fever: ~ d .tachvcxr-dia:

e tachypnea; hypotension.PE:
r e n tral cyan osis: elevated JVP; right ventricular gallop rhythm
with widely split 52.

Labs .=C;'i:hypoxia and hypescapnia {type 2 respiratory rnilure).

FCC;: SIQST3 pat1ei.n and sirills tachycardia.

Imaging LJS, Doppler: clot in right common femoral vein. CXR: right
lohc atrlcctasis. Y J Q :thrue it1.t.its of ventilation-prrft~sioi~
lo~+,cl-
mismatch in right lung. Anpjv. p l ~ l ~ n o r ~ acor~fir.malol-y:
ry: not
reqnired il'Y/Q can is high probability.

Gross Pathology Large thramhus scrn in pulmonary artrry.

Micro Pathology Large ocrlz~siveI hl-omh ~ seen

n in pi~lmonar-yartcrv wit11 mriabIc
clegrcc of rrcani12ization.

Treatment Sltpy>or-rive; ~lir-~!nboly tic 1hemp?: cot~sirler.ein hrhectoiny;

heparin, Chun~adin,and low-molecular-~vcigl~t heparin
( cnclsapnrin ) insti tu terl for prop11ylaxi.i (monizc>rINR) .

Discussion Pltlmnnal-y erlllboli mosr rommoiilv originate from pruxinlal

deep venot 15 ~ h t - c m hosis. Pulmonary angiography is t i i t gold
stanrl;~rdin tIlc rliawosis of pz~lrnntraryetnholism, h ~ obtain ~ t a
V/Q cran initially if clinically suspcctrd. Vircbow's triad ouilines
111c risk Lictors S i r thrombus forn~atior~ and inc-lucieq hlnocl Fta-
sis f r .g., iriirnol1ili7alior1), enrlor helid rlanlage ( e . ~ .surgery)
, ,
anrl hyperrnag-t~lahlestates ( c . ~ malignancy,
., prcgnarxcynsew-re
I3~1rnsl.Large emholi may c a u s c car~liovasculi~r cullapse I; lid
uudderr r l ~ ; ~ t h .

Atlas Links

* PULMONARY EMBOLISM
 I D/CC A 28year4d black female cotnplai n F of fever, dmnea, arthralgia,
and erythernatouq, tender nodules on hnth legs.

HPI She has no tlistory of Careign traiwl or curltact with a, ~lzherc~~lar

patient.

PE 15: Ccver. PF: tcn clcr, erythematous nodules over extenwr aspects
of both legs (FR\TW.RI,~ vonos~mf):artIir.algia.r,of hot11 knees:
rpleno1nega1y

Labs CBC: Iymphopenia; eosinophilia. L,ytes: elevated serum calcium;

hypercafciuria. ACE levels elevated: 11Errnrl cultlrres negative;
Mantoux test negative: 111ngals e r o l o ~ negativc. PFTs: evidence
of restrictive changes. T ~ - a n s l ~ r c ~ ~1~L cI ~l ~I Xbit
i a ~l p s vordered.

Imaging CXR: hilateraT hilar lymphadenopathy aucl rig11r paratracheal

aclenopathv; interstitial infiltrates: n o pleural eff~lsirsn.

Gross Pathology Firm nt>clulcsonly a frw millimctel-sin size in affecrcd ol-ganq;

can llecnrne con fluent a n d gite lise lo larger nnrlulcl;.

Micro Pathology Lbmp t~ node biopsy reveals noncaseatinig granulomas with

fil>r.oticacell~rlarcore surr-ounded l,v I?mpI tocyles, r pit lrelioirf
cells, ancl Lnt~gerlian'sgiant cells.

Treatment Corticosteroids.

Discussion 111 tlnc United States, the incidence of sal-coidosis is highest in

13Tack wornen, with oTiset behveen 20 and 40 years of age. The
diwast: rrlay ljc asyrnpturnatic: Iiowui-er, syrnptoIrls rnay he
cnnsti~~tintial a n d [nay iinvolvp many di Cf'eren t organ svstems.
including rhc l u n ~ slymph
, nocles, skin, rvc, uppcr respiratory
tr.acl, r~rticu1nendc)tlieliaIsystem, Iiver, kidneys. ilervow qstem,
and heart. tZppl-oximatelp 60% to 713% or sal-coidosispatients
recover with T e ~ vor no residual wrnproms.

Atlas Link mPM-PZ-092

SARCOIDOSIS
 ID/CC t~lalc.presents T ~ ~
115Tb1~ear=>TrE LII
progrc.;sively increasing dyspnea
xrlrl dry cough noT severaI years' rlr~ralion.

HP1 Hc is a ~ ~ o r ~ s r n o kheurt, his o c c ~ ~ l > a ~ i r11, nistot-?

; ~ l inrludes mining
and quarrying.

7
PE N{I l - l ~ ~ l > l ~wanorili,
ing, reduced chest
or IympI~acterlc~patI~y C
expansion 0x1 inqpirnrinn: d r y inspiratory crackles a~iscullatedin f
upper Infwk of ht>th lrmgs. 0
Z
B
m
Labs PITS: cornl,inetl ohsitrxrctive and restrictive pattern of functional
iliip~irin~ I. nRrrjnch o~c~picallv-~uicIcr1lung biopsv cst;~ldi?hes
rlinfi-nnsi~: negative Mantoux i r s t : sputlun cvtr>lov:;u~d stailling
~ O I acid-f'ls~
- bacilli ~ ~ c j i a l i v e .

Imaging CXR. PA: I-oundetls ~ n ; ~opaci~irc

ll in tipper lobes with retraction
anrl hilar Iyrnphadenapathy; "eggshell" calcification of lymph
nodes.

Gross PathoZogy c ~ l ~ u in the IIPPPI.l ~ ~ n ill

Del~ru,small c c ~ l l a ~ r i ~iznrlules g s~ h early
c
T ~ ~ I ~FP~SI :P Xantl
~ h e r o ~ n emore rliff~iceas clistasi~pr.o.qrp'cses.

Micro PathoIogy Hvalirlizetl whorl5 of c n l l a ~ e u~ r . i l hlitlle o r n o inflammation:

pc~lar-iserll i g h ~r1~t~lot1strate.c
silica particles w i l l ~ i itlo~lliles.
~

Treatment S ~ ~ y ~ p o r tavoitlarlce
i\r: expnqllre
of I'r~rtll~r

Discussion Thrrc is an increased incidence of tuberculosis it] rilicmis

~ x ~ i i e n tSilicosis
s. Irxrls to rrstriclivr l u l ~ gtlisease { h a t varie5 in
wvriilv FI.OTII ~iiilcl10 dis:~l>li~jg.

+ SILICOSIS