Tulburari hemostaza Flashcards
Teleangiectazia ereditara Rendu-Osler
transmit AD, b a peretelui vasc
exces de VEGF –> prolif endoteliala => subt perete vasc => dilatari = TELENAGIECTAZII cut-muc –> hemoragii cr
trat - corect anemie, cauteriz
Purpura Schonlein-Henoch
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
Trombocitopenia - def, clasif
PLT <150.000/mm3
centrala (def Mgk)
perif - imune/ neimune (sechestrare sau consum)
Purpura trombocitopenica idiopatica/imuna (PTI) - patogen
distrugere PLT prin AutoAc anti plachetari (GP IIb/IIIa) sau Ly T
trombopoieza ineficienta
PTI - clinic, lab
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
PTI - dg
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
PTI - trat
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
PTI - sarcina
! risc de hemoragie intracerebrala fetala
corticoizi inaintea sarcinii
recolt sg cordon ombilic (prepartum)/vena centrala a sclapului (intrapartum) -> PLT <50.000 –> cezariana
PTI - prognostic
favorabil
mortalitate scazuta chiar si la cei cu PTI refract
Purpura trombocitopenica trombotica (PTT) si sindromul hemolitic uremic (SHU)
apar dat agreg plachetare diseminate
PTT - mai frecv la adulti tineri
SHU - la sugari si copii, frecv afect renala
PTT - patogeneza
AutoAc anti clivaza FvW => persist multimerilor FvW (forma activa) => activ exagerata a aderarii PS => trombi plachetari diseminati (TROMBOZE) –> trombocitopenie prin consum => HEMORAGII
PTT - clinic
debut brusc
hrg cut-muc, manif neuro-psihice tranzitorii, IR, febra
PTT - lab
trombocitopenie severa
anemie, reticulocitoza, schizocite (hemoliza microangiopatica)
PTT - trat
! urgenta
plasmafereza, transfuzii masive de plasma si MER
corticoizi, ASS
dializa
PTT - prognostic
mortalitatea a scazut
pot persista sechele neuro
Boala von Willebrand
b congenit dat anomalii cant/calit a fvW
BvW - clinic
hemoragii cut-muc - sfera ORL (loja amigdaliana)
BvW - tip 1
transmit AD
deficit moderat cant de FvW
se poate asocia cu deficit de FVIII (>10%)
BvW - tip 2
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
BvW - tip 3
transmit Ar
deficit cant sever de FvW
asoc cu deficit de FVIII 2-10%
BvW - lab
TS prelungit, APTT poate fi prelungit (tip 1,3)
deficit de agreg la ristocetin
scad FVIII in tipurile 1,3
BvW - tratament
plasma, criopp
concentrat de FvW si FVIII
desmopresina, EACA
Hemofilia A,B - def
b eredit X linkate
A - deficit de fact VIII
B - deficit de fact IX
Hemofilie - clinic
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%)
