Plasma Exchange and Concomitant Therapy in TTP

Abstract

Since plasma exchange was introduced in the management of thrombotic thrombocytopenic purpura (TTP) in 1977, patient survival rate has increased from 10 to 80%. However, approximately 50 subsequent case reports in the literature provide no consensus as to the optimal therapy. We review here 4 episodes of TTP in 3 patients. In all cases, treatment was started with intensive FFP plasma exchange combined with administration of antiplatelet agents and corticosteroids. Remission was achieved in 3 out of 4 episodes although all required individualization of the medication regimen. In the remaining patient, cytotoxic therapy (vincristine) and ultimately splenectomy were required to achieve stable remission. The variable clinical response to these therapeutic protocols indicates that TTP may not represent a single homogeneous disease entity but rather may involve various underlying pathologies. We conclude that the most effective present therapy for the management of TTP is daily plasma exchange with fresh frozen plasma infusions combined with antiplatelet agents and steroids. Vincristine and splenectomy should only be employed if this protocol proves ineffective.

A Case of Thrombotic Thrombocytopenic Purpura Refractory to Plasma Exchange

“…Since the introduction of PE and plasma infusion, a dramatic change in the prognosis of TTP has taken place. In addition to antiplatelet drugs and prostacyclin, corticosteroids and vincristine are applied in the treatment of patients suffering from refractory TTP to PE (7). We experienced a survived case of TTP without sufficient effect of PE and antiplatelet drugs who required a concomitant use of corticosteroids and vincristine.…”