Treatment of choice in TTP is (LQ)
Thrombotic thrombocytopenic purpura (TTP) (AIIMS Nov 07)
It is a pentad of:
2 Fluctuating CNS signs (eg fits, hemiparesis, consciousness, vision)
3. Microangiopathichemolytic anemia (MAHA) (severe, often with jaundice)
4 Thrombocytopenia (severe, often mucosal bleeding)
5 Renal failure. Also: haematuria or proteinuria.
Adult are chiefly affected
Pathogenesis: Adult are chiefly affected due to deficiency of specific metalloproteinase ADAMTS-13
2. Drugs (egclopidogrel, cyclosporine),
3. Pregnancy, HIV, SLE.
Tests: As HUS. In both (HUS and TTP) - PT, PTT, fibrinogen concentration are normal.
DIC is not a feature.
Rx: It is a hematological emergency
1. Urgent plasma exchange is life-saving. It is the treatment of choice.