SINDROAME HEMORAGIPARE

SH DE CAUZA VASCULARA: AI- Purpura alergica Schonlein-Henoch

=purpure vasculare - Purpura medicamentoasa
Inf
Prin alterarea str vaselor si str perivasculare – ereditare- THE Rendu-Osler
- castigate – Scorbut
- Purpura simplex, senila
- GC, PHs
Prin diverse mecanisme

SH TROMBOCITARE: Purpure trombocitopenice –distructie exagerata –imunol- AI- Purpura tromobitopenica imuna- PTI=B. Werlhoff
=purpure trombocitare - sec
-izoimune (transfuzii)
-medicam (heparina)
-HELLP
-neimunologic- CID

-deficit de productie -scaderea prod megacariocite

-trombocitopoieza inef

-tulburari de distributie- sechestrare splenica

Trombocitopatii= afectarea calitatii trombocitelor

-constitutionale –tulburarea adezivitatii= sdr Bernard Soulier (| GpI)
- tulburarea agregarii= trombastenia Glanzmann (|Gp IIb/IIIa)
- tulburarea reactiei release–A- stocarea ADP-Wiskott (+albinism)
-B- expulzia ADP- TP aspririn-like
-tulb metab Tx -|COX
|TX-sintetazei
-dobandite

SH TULB COAGULARII: ch genetic – legate de sex- Hemofilia A (lipsa f VIII:C) –deficit de sinteza a globulinei antihemof A (<20%)
-exces de antitromboplastina
-porozitate capilara crescuta
TR : fVIII purificat, recombinat,
fVII recombinat
Desmopresina
Terapie genica

- Hemofilia B (lipsa fIX Christmas)

 coagp cu mostenire autos- D – Boala von Willebrand (lipsa fVIII: fvW)
- perturbarea aderarii placutelor la str subendot (GpI)
-I/II/III- partial, calitativ, complet

Hemofilie BvW

TC, TH prelungite TC prelungit

Timp de consum al protrombinei scurtat
aPTT usor prel
TS, TQ, TT : N TS prelungit
Tb N, activ fibrinolitica N Scaderea adezivitatii plachetare, Nr Tb N

SH TULB ALE FIBRINOLIZEI

Fibrinoliza primara CID-diateza hemoragica dobandita

CID I= consumul de factori in cadrul unei CID II= fibrinoliza secundara reactionala
CID

Dezech f activ/ inh ai fibrinolizei-> sdr hem sever Scaderea Tb Modificarile CID I+

Alungire TQ, TH, PTT (scaderea V,VIII) Alungirea TQ, TH, PTT
Scaderea Fbg Scade Fbg TARS prelungit
Scurtarea TLCE TLCE normal TLCE scurtat
PDF, prin alungirea TT TMF ++ PDF

TR: Antiplasmine TR : etio

Anti PAI Heparina 300-500U/ora (neutraliz activ trombinei in plasma)
Tratament substitutiv Anti PAI
Perfuzii PPC, masa trombocitara, crioprecipitat