Tulburari hemostaza Flashcards

1
Q

Teleangiectazia ereditara Rendu-Osler

A

transmit AD, b a peretelui vasc
exces de VEGF –> prolif endoteliala => subt perete vasc => dilatari = TELENAGIECTAZII cut-muc –> hemoragii cr
trat - corect anemie, cauteriz

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2
Q

Purpura Schonlein-Henoch

A

purpura imunologica, la copii si tineri
purpura cut simetrica + manif dig + artrita +/- afect renala
patogen: dep complexe imune cu IgA, pos rol al strep b hemolitic
trat - evol spontana fav, eventual corticoizi, dializa, erad strep

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3
Q

Trombocitopenia - def, clasif

A

PLT <150.000/mm3
centrala (def Mgk)
perif - imune/ neimune (sechestrare sau consum)

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4
Q

Purpura trombocitopenica idiopatica/imuna (PTI) - patogen

A

distrugere PLT prin AutoAc anti plachetari (GP IIb/IIIa) sau Ly T
trombopoieza ineficienta

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5
Q

PTI - clinic, lab

A
hemoragii cut-muc cu debut brusc
hlg - PLT f mici, izolat
mo - Mgk N/hiperplazice/displazice
Auto Ac anti plachetari
!excl cauzelor de trombocitopenie sec
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6
Q

PTI - dg

A

de excludere
ac anti PS, excl cauzelor de trombocitopenie sec (LES, Ac anti HP, imunodef, HIV, HCV)
la pacientii >60 ani, PTI refract/recidiv - medulograma

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7
Q

PTI - trat

A

I: PLT <50.000, disf PS, chir, trat anticoag, comorbiditati
linia 1 - corticoterapie
linia 2 - splenectomie, imunosupresie, agonisti TPO-R, danazol
linia 3 - combinatii linia 1 si 2, polichimioterapie, TCSH

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8
Q

PTI - sarcina

A

! risc de hemoragie intracerebrala fetala
corticoizi inaintea sarcinii
recolt sg cordon ombilic (prepartum)/vena centrala a sclapului (intrapartum) -> PLT <50.000 –> cezariana

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9
Q

PTI - prognostic

A

favorabil

mortalitate scazuta chiar si la cei cu PTI refract

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10
Q

Purpura trombocitopenica trombotica (PTT) si sindromul hemolitic uremic (SHU)

A

apar dat agreg plachetare diseminate
PTT - mai frecv la adulti tineri
SHU - la sugari si copii, frecv afect renala

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11
Q

PTT - patogeneza

A

AutoAc anti clivaza FvW => persist multimerilor FvW (forma activa) => activ exagerata a aderarii PS => trombi plachetari diseminati (TROMBOZE) –> trombocitopenie prin consum => HEMORAGII

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12
Q

PTT - clinic

A

debut brusc

hrg cut-muc, manif neuro-psihice tranzitorii, IR, febra

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13
Q

PTT - lab

A

trombocitopenie severa

anemie, reticulocitoza, schizocite (hemoliza microangiopatica)

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14
Q

PTT - trat

A

! urgenta
plasmafereza, transfuzii masive de plasma si MER
corticoizi, ASS
dializa

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15
Q

PTT - prognostic

A

mortalitatea a scazut

pot persista sechele neuro

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16
Q

Boala von Willebrand

A

b congenit dat anomalii cant/calit a fvW

17
Q

BvW - clinic

A

hemoragii cut-muc - sfera ORL (loja amigdaliana)

18
Q

BvW - tip 1

A

transmit AD
deficit moderat cant de FvW
se poate asocia cu deficit de FVIII (>10%)

19
Q

BvW - tip 2

A

transmit AD - defecte calit a FvW
2A - defect de polimerizare -> abs polimerilor din sg si PLT
2B - defect de polimerizare -> abs polimerilor din sg
2M - defect de leg de PLT
2N - defect de leg de FVIII

20
Q

BvW - tip 3

A

transmit Ar
deficit cant sever de FvW
asoc cu deficit de FVIII 2-10%

21
Q

BvW - lab

A

TS prelungit, APTT poate fi prelungit (tip 1,3)
deficit de agreg la ristocetin
scad FVIII in tipurile 1,3

22
Q

BvW - tratament

A

plasma, criopp
concentrat de FvW si FVIII
desmopresina, EACA

23
Q

Hemofilia A,B - def

A

b eredit X linkate
A - deficit de fact VIII
B - deficit de fact IX

24
Q

Hemofilie - clinic

A

hemoragii prof - hemartroze, hematoame
3 forme
severa - hrg spontane (activ <1%)
medie - hrg la traumatism minor (activ 1-5%)
usoara - hrg la traumatisme mari si interventii chir (activ 5-25%)

25
Q

Hemofilie - lab

A

APTT prelungit

activitatea FVIII/IX scazuta

26
Q

Hemofilie - trat

A

imobiliz mb si articulatiei, antialgice, fizio-kineto terapie
tratament substitutiv on-demand/profilactic - criopp, plasma, concentrat de fact VIII/IX
hemofilia dob (autoAc anti fact coag) - cresterea dozelor de concentrat de fact, corticoizi, FVII activat

27
Q

Hemofilie - complicatii

A

artropatie hemofilica

boli virale

28
Q

Trombofilii congenitale

A

fact V Leiden
protrombina G20210A
deficit de proteina C, S, ATIII

29
Q

Trombofilii - tratament

A

in episoade trombotice - anticoagulant
profilaxie anticoagulanta - ep trombembolice rec, eventual FV Leiden homozigot, cond cu risc trombotic (sarcina, imobilizare, interventii chirurgicale)

30
Q

Trombofilii sec

A

cc, leucemii, limfoame - trat b de baza
sdr AFL - corticoterapie + imunosupresie, profilaxie anticoag in sarcina in dupa avorturi spontane recidivante
sdr nefrotic