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Leucemii, Coagulare
Leucemii, Coagulare
LEUCEMII......................................................................................................................................................1
Clasificare........................................................................................................................................................3
Clasificare
LEUCEMII ACUTE........................................................................................................................................3
Definitie.......................................................................................................................................................3
Clasificare....................................................................................................................................................3
Simptomatologie..........................................................................................................................................3
1. Sindrom anemic...........................................................................................................................................3
anemic
2. Sindrom infectios.........................................................................................................................................3
infectios
3. Sindrom hemoragic......................................................................................................................................3
hemoragic
Simptome de localizare................................................................................................................................3
Simptome de localizare................................................................................................................................3
Examen hematologic.......................................................................................................................................4
hematologic
A. Sange periferic.........................................................................................................................................4
periferic
B. Maduva osoasa.........................................................................................................................................4
osoasa
LAL..................................................................................................................................................................4
LAM................................................................................................................................................................4
LEUCEMIE MIELOIDA CRONICA..............................................................................................................4
Definitie.......................................................................................................................................................4
Simptomatologie..........................................................................................................................................4
I. Examen hematologic................................................................................................................................5
II. Alte investigatii.........................................................................................................................................5
LEUCEMIA LIMFATIC CRONICA............................................................................................................5
Definitie.......................................................................................................................................................5
Simptomatologie..........................................................................................................................................5
I. Examen hematologic................................................................................................................................6
II. Alte investigatii........................................................................................................................................6
BOALA HODGKIN........................................................................................................................................6
Definitie.......................................................................................................................................................6
Simptomatologie :............................................................................................................................................6
1. Determinari in organe limfatice...............................................................................................................6
limfatice
2. Determinari in organe nelimfatice...........................................................................................................7
nelimfatice
3. Manifestari generale :..................................................................................................................................7
:
Investigatii paraclinice.................................................................................................................................7
I. Biopsia ganglionara.....................................................................................................................................7
I. Examen hematologic....................................................................................................................................7
3. Alte investigatii...........................................................................................................................................7
investigatii
Stadializare:..................................................................................................................................................7
LIMFOMUL NON HODKIN..........................................................................................................................8
Diagnostic si stadializare.............................................................................................................................8
MIELOMUL MULTIPLU...............................................................................................................................8
Simptome.....................................................................................................................................................8
Diagnostic paraclinic....................................................................................................................................8
SINDROAME HEMORAGIPARE.................................................................................................................8
I. PURPURE VASCULARE.........................................................................................................................8
VASCULARE
II. SINDROAME HEMORAGIPARE PRIN TROMBOPENII SI / SAU TROMBOPATII
TROMBOPATII..............................8
III.
COAGULOPATII..................................................................................................................................8
COAGULOPATII
PURPURA HENOCH SCHNLEIN..............................................................................................................8
Etiologie.......................................................................................................................................................8
Semiologie:..................................................................................................................................................9
Investigatii paraclinice.................................................................................................................................9
I. Examen hematologic................................................................................................................................9
hematologic
II. Alte investigatii.........................................................................................................................................9
investigatii
Page 1
PURPURELE TROMBOCITOPENICE.........................................................................................................9
Cauze............................................................................................................................................................9
Etiopatogeneza.............................................................................................................................................9
Semne clinice...............................................................................................................................................9
1) hemoragii cutanate...................................................................................................................................9
cutanate
2) hemoragii mucoase
mucoase epistaxis.................................................................................................................9
epistaxis
Semne hematologice..................................................................................................................................10
PURPURA TROMBOPENICA IDIOPATICA ( BOALA WERLHOF )......................................................10
Etiopatogeneza...........................................................................................................................................10
Simptomatologie........................................................................................................................................10
Examen hematologic..................................................................................................................................10
TROMBASTENII..........................................................................................................................................10
Definitie.....................................................................................................................................................10
Clasificare..................................................................................................................................................10
Simptomatologie........................................................................................................................................10
Examen hematologic..................................................................................................................................10
HEMOFILII...................................................................................................................................................10
Definitie.....................................................................................................................................................10
Clasificare..................................................................................................................................................10
Istoric.........................................................................................................................................................10
Simptomatologie........................................................................................................................................10
Investigatii paraclinice...............................................................................................................................11
COAGULARE INTRAVASCULARA DISEMINATA.................................................................................11
Definitie......................................................................................................................................................11
Etiologie.....................................................................................................................................................11
Simptomatologie........................................................................................................................................11
I. Faza de hipercoagulabilitate..................................................................................................................11
hipercoagulabilitate
II. Faza de hipocoagulabilitate...................................................................................................................11
hipocoagulabilitate
Manifestri clinice......................................................................................................................................11
Investigatii paraclinice...............................................................................................................................11
LLC................................................................................................................................................................11
Biermer..........................................................................................................................................................12
Hemofilie.......................................................................................................................................................12
LAM..............................................................................................................................................................12
LMC...............................................................................................................................................................12
PTI.................................................................................................................................................................12
INGRIJIREA B.CU BOLI HEMATOLOGICE.............................................................................................12
ANEMII FERIPRIVE....................................................................................................................................12
ANEMIA BIERMER.....................................................................................................................................12
POLICITEMIA VERA..................................................................................................................................13
INGRIJIRE B. SD.HEMORAGIPAR...........................................................................................................13
HEMOFILIA..............................................................................................................................................13
CID.............................................................................................................................................................13
Rolul asistentei...........................................................................................................................................13
Page 2
snge alb
Clasificare
Origine mieloida
Origine limfoida
ACUTE
LAM
LAL
CRONICE
LMC
LLC
LEUCEMII ACUTE
Definitie
- boala neoplazica caracterizata prin proliferarea maligna a celulelor tinere aflate in primele stadii de
diferentiere mieloida sau limfatica si infiltratia maduvei osoase.
Clasificare
leucemie acuta mieloblastica
- leucemie acuta limfoblastica
Simptomatologie
Debut
brusc
febra
alterarea starii generale
sindrom hemoragipar
Stare
1. Sindrom anemic
paloare
cefalee
ameteli
astenie
dispnee
sufluri sistolice
2. Sindrom infectios
febra ridicata
leziuni ulceronecrotice ale - mucoasei bucale, amigdalelor
infectii
cutanate
respiratorii
urinare
3. Sindrom hemoragic
hemoragii cutanate si mucoase
Simptome de localizare
Adenopatii
generalizate
marime redusa
consistenta usor crescuta
nedureroasa
neaderenta la planurile profunde / tegumente
Hepatomegalie moderata
Splenomegalie
moderata
ferma
neteda
regulate
Simptome de localizare
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LAL
Cea mai frecvent afeciune malign la copil
La adult 20% din LA
Afectare frecvent a SN puncie lombar obligatorie
LAM
M0: difereniere minim
M1: leucemie mieloblastic fr maturare
M2: leucemie mieloblastic cu maturare
M3: promielocitic
M4Eo: vatiant: creterea eozinofilelor n mduv
M4: mielomonocitic
M5: monocitic
M6: eritroleucemia(DiGuglielmo's)
M7: megakarioblastic
Specific:
M2 : Cloroma:-prezent ca o mas tumoral tumor of leukemic cells: orbit, sinusuri paranazale,
tub digestiv, ap genito-urinar, pleur, mediastin, sn, peritoneu
M3 : CID
M4/M5 : gingivit hipertrofic, infiltrarea SNC, leucemia cutis (infiltraii blastice cutanate sub
forma unor noduli violacei nedureroi)
Page 4
2)
Splenomegalie
neteda
nedureroasa
mobile
moderata
3) Hepatomegalie moderata
4) Sindrom Mickulicz
hipertrofie simetrica a glandelor lacrimale si parotide
5) Manifestari cutanate
leucemide
eritrodermie (om rosu)
rash
purpura
echimoze
mycosis fungoides
prurit intens ; leziuni de grataj
6) Manifestari pulmonare
infectii bronhopulmonare
7) Semne generale
astenie
subfebrilitate
transpiratii
8) Manifestari oculare ,nervoase , osteoarticulare si renale
I. Examen hematologic
1. Sange periferic
Serie alba
hiperleucocitoza - G.A.= 50.000-150.000/ mmc
limfocitoza - 80%
umbre celulare Gumprecht
Serie rosie - anemie moderata, normocroma, normocitara
Serie trombocitara - trombocitopenie /numar normal
2. Maduva osoasa
infiltatia maduvei cu limfocite - 50-90%
scadere relativa pe celelalte linii celulare
II. Alte investigatii
fosfataza alcalina leucocitara - normala / crescuta
hipo
hipogamaglobulinemie
Manifestri autoimune
Anemie hemolitic
Trombocitopenie autoimun
FR, Ac - antinucleari
BOALA HODGKIN
Definitie
limfom malign caracterizat prin
clinic - poliadenopatie febrile
morfologic - granulom (cel. Sternberg - Reed)
Simptomatologie :
1. Determinari in organe limfatice
a) Adenopatii
Superficial
cervicale asimetrice
Page 6
nedureroase
consistenta ferma
neaderente
nu ulcereaza
mase tumorale
durere dup ingestia de alcool
profunde
mediastinale
tuse iritativa
dispnee
compresiune pe VCS
abdominale - fenomene de compresiune
b) Splenomegalie
prezenta in 50% din cazuri
moderata
2. Determinari in organe nelimfatice
pleuropulmonare
pleurezie
infiltrate pulmonare
cardiace
tulburari de ritm
pericardita
neurologice
osoase - dureri osoase spontane / la compresiune
digestive - in special hepatice
renale
cutanate (ichtioz
(ichtioz,, acrokeratoz
acrokeratoz,, urticarie
urticarie,eritem nodos, leziuni necrotice, hiperpigmentare,
infiltrarecutanata)
infiltrarecutanata)
3. Manifestari generale :
febra (ondulanta)
transpiratii
prurit cutanat
scdere ponderal
astenie
Investigatii paraclinice
I. Biopsia ganglionara
celula Sternberg Reed
giganta
citoplasma abundenta
nucleu mare unic / multiplu
- provine din celula B ntr-un stadiu precoce de difereniere
elemente ale granulomului
limfocite
histomonocite
plasmocite
eozinofile
neutrofile
fibroblasti
I. Examen hematologic
1. Sange periferic
Serie rosie - anemie normocroma / rar hipocroma
Page 7
Serie alba
G.A. de obicei normal
uneori leucocitoza moderata
rar leucopenia
neutrofilie , eozinofilie ,monocitoza , limfopenie
Serie trombocitara - Tr de obicei normale
2. Maduva osoasa - aspect normal
3. Alte investigatii
VSH ; fibrinogen crescute
hipergamaglobulinemie
IDR la tuberculina negative
Stadializare:
Stadiul I : un singur grup ganglionar (I) sau organ extralimfatic (IAE )
Stadiul II : 2 sau mai multe grupe ganglionare (II) sau organ extralimfatic de aceeai parte a
diafragmului
Stadiul III : Interesarea ambelor pri ale diafragmului
Stadiul IV : Interesarea organelor extraganglionare (ficat, mduv osoas)
A. absena simptomelor generale
B. prezena simptomelor generale (febr, transpiraii, scdere ponderal, prurit, astenie)
LIMFOMUL NON HODKIN
Inciden n cretere
NHL>HD
Vrsta medie 65-70 ani
M>F
Celule B-70% ; celule T-30%
Adesea prezint form diseminat la prezentare
Diagnostic si stadializare
Similar cu LH plus,
Imunofenotipare : Ac monoclonali ;
Ag LB ( CD 19, 20, 22);
Ag LT ( CD 2, 3, 5 & 7);
HIV, Ac H. Pylori.
MIELOMUL MULTIPLU
Afeciune a LB
Proliferarea necontrolat a unei clone de plasmocite cu acumularea lor la nivelul mduvei i
supraproducia unei proteine monoclonale, care poate fi identificat la nivel electroforetic
Simptome variabile, 20% asimptomatici.
Vrsta medie 65.
B > F.
Supravieuire la 5 ani 25-30%.
Simptome
1) Manifestri datorate proliferrii tumorale: dureri osoase, deformri osoase, fracturi pe os patologic
2) Manifestri datorate prezenei proteinei monoclonale
a. Sdr de hipervscozitate (tulburri vizuale, neurologice, insuficien cardiac, hemoragii)
b. Fenomene imunologice (AH, formare fiicuri, trombopenie, fenomene hemoragice, neuropatie
demielinizant)
3) Susceptibilitate crescut la infecii
4) Afectare renal (pr-urie Bence Jones)
Diagnostic paraclinic
Anemie normocrom, normocitar, fiicuri
VSH
Page 8
Page 9
soc endotoxic
leucemii
interventii chirurgicale mari
cancere
emboli gazoase
Simptomatologie
I.
Faza de hipercoagulabilitate
coagulare masiva - formare de microtrombi - infarctizari :
cianoza extremitatilor
nas negru
purpura necrotica
fenomene neurologice
consum exagerat al factorilor coagularii
II.
Faza de hipocoagulabilitate
hemoragii cutaneo-mucoase
hemoragii viscerale
digestive
respiratorii
renale
cerebrale
Manifestri clinice
Investigatii paraclinice
numarul de Tr. < 100.000 /mmc)
TP - scazut ( < 50%)
fibrinogen -scazut ( <2 g / l)
PDF - crescuti
D dimeri crescuti
LLC
B, 53 de ani
Clinic: astenie, adenopatii nedureroase generalizate, eritrodermie
Hb 11,8 g%, Ht 41%
GA 70 000/ mmc
FL: neutrofile 14%, eozinofile 2%, bazofile 3%, limfocite 77%, monocite 4%
Trombocite 200 000 mmc
Biermer
F, 60 de ani
Clinic: cefalee, vertij, dureri precordiale, parestezii membrele inferioare
Ex. obiectiv: paloare, limb roie, neted, dureroas
Hb 4,2 mg%, Ht 21%
GA 2500% mmc, nuclei hipersegmentai
Trombocite: 97 000/ mmc
Fe seric 150 %
Bilirubin 2,7 mg%
Hemofilie
B, 28 ani
Clinic: hemartroz genunchi dup traumatism minim prin cdere, echimoze multiple
Hb 12 g%, Ht 45%
GA 8000 / mmc
Trombocite 210 000 /mmc
TS 3 min
TC 10 minute
LAM
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B, 17 ani
Clinic: vertij, paloare, febr, gingivoragii, adenopatii nedurereroase axilare, inghinale i laterocervicale
Hb 8 g%
GA 67 000/mm3, Formul leucocitar 58 % blati peroxidazo-pozitivi, 13% neutrofile, 3%
eozinofile, 1 % bazofile, 15% limfocite, 7 % limfocite, 3% monocite
Trombocite 56 000/mm3
LMC
F, 56 ani
Clinic: astenie, subfebrilitate, dureri n hipocondrul stg
Hb 10,2 g% , Ht 38%
GA 200 000/ mmc
Formula leucocitar: mieloblasti 2%, promielocii 8%, mielocii 25%, metamielocii 28%,
eozinofile 9%, bazofile 4 %, neutrofile 5%, limfocite 12%, monocite 7%.
Trombocite 550 000/mmc
PTI
B, 28 ani
Clinic: purpur, epistaxis, splenomegalie gradul I
Hb 12,2 g%, Ht 45%
GA 5700/ mmc
Trombocite 28 000/ mmc
TS 7 minute
TC 4 minute
INGRIJIREA B.CU BOLI HEMATOLOGICE
ANEMII FERIPRIVE
oprire sg,dieta bogata in alim cu fe(carne,vegetale frunze verzi)
Fe per os,venofer,ccaptafer,fe haussman
in 3 sapt-recup.50%din deficit Hb
in 2 luni-se recupereaza integral
in 6 luni-reface depozite de Fe
ANEMIA BIERMER
dieta cu alim bogate in vit B12 (carne,lapte,oua,branza)
Trat cauzei ,vit B12 toata viata
nu se asociaza ac.folic
POLICITEMIA VERA
FLEBOTOMII 200-500mlsg pana la Ht 45%
hidroxiuree
radioterapie
hidroxizin ptr prurit
allopurinol ptr litiaza renala
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