Treatment of Thrombotic Thrombocytopenic Purpura (TTP) Diagnosis of TTP: • Rare and severe, ADAMTS13 deficiency • Autoimmune >> genetic • Distinguish TTP from other TMA: Thrombotic microangiopathy without other condition (vs. secondary TMA), Platelets < 30 G/L (vs. HUS), Serum creatinine <200 umol/L (vs. HUS) Treatment of TTP: • Consider admission to the ICU (age, LDH, troponin) • Contraindication to platelets transfusion • Daily plasma exchanges (PLEX) +++ • Rituximab (scheduled on day 1, 4, 8 and 15) • Caplacizumab as soon as TTP is diagnosed, in combination with PLEX and RTX • Short-term glucocorticoids • Folic acid supplementation (5-10 mg/day) By Dr. Raphaël Cauchois #TTP Thrombotic #Thrombocytopenic #Purpura #management #treatment #rheumatology #hematology
