INTERHOSPITAL CONFERENCE

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CC: 5 PTA PI: 5 PTA 8 Admit .admit 3 Augmentin,Ceftazidime iv + Pred 2*3 + Bactrim 2*2, Metronidazole 2*3 , CQ 1* hs .

 PH:
. 5 3 PTA Dx:Possible SLE (ANA positive (nucleolar),polyarthritis, Pancytopenia ) with Lupus hepatitis ( transaminitis AST 107 ALT 108) CBC WBC 2200 N36% L47% M2%Band10% Hct 23.9 MCV90 MCHC33 Plt 99000 F/U WBC 2200 -> 7000 -> 50000 with Lt. shift BMA: peripheral destruction,stool occult blood +ve on pred 8*1tapering off 3

 PH:
u/s abdomen ,True hyperechoic mass 7.5*8.9 mm, 8.6*6.5 mm Rt lobe liver likely Hemangioma liver biopsy anti dsDNA neg ,AMA neg, anti smooth m Ab neg, anti HIV neg ,HbsAg neg, antiHCV neg SPEP: polyclonal gammopathy Hb typing EA Bart

Family history : Healthy

Physical examination
A middle age woman,fully conscious ,well cooperate,looked fatigue. BT 38.2 0C PR 102/min RR 22/min BP 120/70 mmHg HEENT : mild pale, anicteric sclera, pharynx and tonsil not injected,no malar rash, no discoid rash, no oral ulcer,old surgical scar at neck,CLN impalpable,no dry lip,tongue HEART: PMI 5 th ICS and midclavicular line , no heaving , no thrill ,normal S1,S2, no murmur

 Lung : trachea midline, fine crepitation at Lt.lower lung Abdomen : soft,not tender,liver and spleen impalpable, active bowel sound Extremities : no petechiae,no rash, no edema

LAB
CBC Hb 9.4, Hct 28 WBC 42000 PMN 34, Lym 7, Band 14 Metamyelo 27, myelo 17 Promyelo 2 Platelet 28000 MCV 85.6 Retuculocyte 0.30

 PBS:
NCNC, few shistocyte PMN predominate with left shift, no blast Plt. decrease

 BUN 5 Cr 0.5 Na 134 K 3.2 Cl 104 HCO3 23.3 Mg 2. Ca 7.8 Uric acid 4.0 UA sp.gr. 1.015 pH 6.0 ,Pro trace ,Rbc 3-5,Wbc1-2

 LFT
TP 5.9 Alb 2.0 Glo 3.9 Chol 138 TB 0.4 DB 0.1 AST 37 53 ALP 159 LDH 167 ALT D-dimer=negative
PT 12.4 INR 1.02 PTT 35.8

CXR

3 PTA

admit

H/C : NG G/S ,AFB,mAFB: no organism Rectal S/C : NG Stool exam : normal Sputum C/S
Numerous Kieb. Pneumoniae (ESBL) Numerous Coagulase Negative Staphylococci Sent : Tazocin, Meropenem,Colistin

Abnormal granule

Flow cytometry

Flow cytometry
CD7=0.7%,CD10=2.3%, CD11b=30.3%, CD13=99.6%, CD15=5.1%,CD19=2.1%, CD33=97.4%CD34=0.8%, CD56=80.9%, CD64=1.2%, CD117=7.4%,HLADR=1.6%, MPO=97.1%,TdT=2.9%

Flow cytometry
CD7=0.7%,CD10=2.3%, CD11b=30.3%, CD13=99.6%, CD15=5.1%,CD19=2.1%, CD33=97.4%CD34=0.8%, CD56=80.9%, CD64=1.2%, CD117=7.4%,HLADR=1.6%, MPO=97.1%,TdT=2.9%

Suggestive of Acute Promyelocytic Leukemia(AML-M3)

Chromosome study
46,XX,t(15;17)(q22;q21)

Molecular study
PML-RARA gene in APL: positive (bcr 1)

Progress case

Am J Hematol. 1980;9(4):413-20. Unusual intracytoplasmic inclusions in acute myeloblastic leukemia. Wolf DJ, Fialk MA, Mouradian J, Gottfried EL, Pasmantier MW. Abstract Unusual intracytoplasmic inclusions within early granulocyte precursor cells from a patient with acute myeloblastic leukemia (AML) are described. Based upon their staining characteristics and electron- and light-microscopic appearance, the inclusions are distinctly different from any previously described. The inclusions display a variety of shapes, including rectangles, squares, circles, ovals, and irregular, globular forms. Most of the inclusions are refractile and crystal-like. The possible composition of these inclusions is discussed. They are compared with inclusions previously described within leukemic and granulocytic cells.

PMID: 6163354 [PubMed - indexed for MEDLINE]

The bone marrow aspirate shows numerous abnormal promyelocytes with prominent cytoplasmic granules, characteristic of hypergranular acute promyelocytic leukemia.

Tallman M S , Altman J K Blood 2009;114:5126-5135

2009 by American Society of Hematology

 Microgranular Variant: In the microgranular variant, M3v, the leukemic cells have a monocytic appearance with clefted angel-wing nuclei and abundant cytoplasm having at best indistinct cytoplasmic granulation.

Acute promyelocytic leukemia (M3)

Bone marrow aspirate from a patient with the hypergranular promyelocytic variant of AML (FAB classification M3). (Wright-Giemsa stain). The cell in the top center and far left are "faggot" cells, with numerous intertwining Auer rods (arrows).

Acute promyelocytic leukemia (M3V)

Blood smear from a patient with the microgranular promyelocytic variant of AML (FAB classification M3V). (Wright-Giemsa stain). The promyelocytes vary in size and degree of cytoplasmic basophilia. The cytoplasm contains abundant, fine, azurophilic granules; nuclei are markedly lobulated and invaginated.

Myeloblasts with Auer rods

Acute promyelocytic leukemia (M3)

APL with DIC

Hypergranular variant

APL, microgranular variant

Auer rod morphology in APL

 APL represents a medical emergency with a high rate of early mortality, often due to hemorrhage from a characteristic coagulopathy

APL
Bone marrow failure syndrome DIC 80% leukopenia -- hypergranular 20% leukocytosis-- microgranular

Flow cytometry
CD 13 ,33 MPO - positive CD11b CD 117 HLA-DR ->negative

Risk stratification
Low risk ---WBC < 10000, plt >40000 Intermediate risk --- WBC <10000,plt<40000 High risk ---WBC>10000

 Without treatment, APL is the most malignant form of AML with a median survival of less than one month treatment of APL is distinct from that of other types of AML and is comprised of several stages Remission induction Consolidation Maintenance