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



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