instit.oncol.cluj-napoca- sectia hematologie - 2012
instit.oncol.cluj-napoca- sectia hematologie - 2012
instit.oncol.cluj-napoca- sectia hematologie - 2012
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17. PROTOCOL DE INVESTIGATII SI TRATAMENT IN PURPURA TROMBOTICA<br />
TROMBOCITOPENICA (PTT) SI SINDROMUL HEMOLITIC-UREMIC (SHU)<br />
17.1. DEFINITIE<br />
Purpura trombotica trombocitopenica (PTT) si sindromul hemolitic uremic (SHU) sunt<br />
afectiuni rare, caracterizate prin debut brusc, tablou clinic caracterizat prin tromboze (datorate unei<br />
agregari plachetare disseminate), hemoragii (prin trombocitopenie de consum), anemie hemolitica<br />
microangiopatica, tulburari neuropsihice si insuficienta renala.<br />
- PTT intereseaza in special adultii tineri si este in majoritatea cazurilor o boala<br />
autoimuna, mediata de anticorpi anti-enzima de clivare a multimerilor cu greutate inalta<br />
de factor von Willebrand (vWF), enzima cunoscuta sub numele de ADAMTS13.<br />
Actiune inhibitory a acestor anticorpi are ca rezultat scaderea importanta a activitatii<br />
ADAMTS13 (tipic nivele