Response to Plasma Exchange and Steroids as Combined Therapy for Patients with Thrombotic Thrombocytopenic Purpura

Abstract

We describe our experience in the management of 11 consecutive patients with thrombotic thrombocytopenic purpura (TTP) treated with a combined therapy of plasma exchange (PE) and steroids. Nine patients (82%) achieved complete remission (CR) after a median of 6 rounds of PE (range 2–22). There were 3 early relapses managed in the same way as the initial episode. One patient relapsed 23 months after diagnosis achieving CR with standard therapy; another patient suffered several relapses, and splenectomy was performed after the last one. Three patients died, 2 of them with resistant disease 9 and 38 days after diagnosis, and the remaining one died due to AIDS-related complications while he was in CR. Eight patients are alive in CR with a median follow-up of 38 months (range 8–74). The combination of PE and steroids is a well-tolerated and effective treatment of TTP, but improvements in therapy are needed to manage refractory patients.

Thrombotic thrombocytopenic purpura in southern Turkey: a single-center experience of 29 cases

“…However, overall mortality was higher in the present study compared with those in the literature which may also correlate with delays in treatment (Shumak et al. , 1995; De la Rubia et al. , 1999; Dervenoulas et al.…”

“…In general, corticosteroids are being used in combination with PE as a frontline therapy (Bell et al. , 1991; De la Rubia et al. , 1999).…”

“…Complete response to treatment was defined by a platelet count >150 × 10 9 /l, partial response (PR) as a platelet count >50 × 10 9 /l or doubling of the initial platelet count and no new clinical events (De la Rubia et al. , 1999).…”

“…Recurrence of any of the following after a complete remission; the initial symptoms, microangiopathic hemolytic anemia, thrombocytopenia, deterioration of clinical status, and laboratory values is defined as relapse (Bell et al. , 1991; De la Rubia et al. , 1999).…”

Role of methylene blue‐treated or fresh‐frozen plasma in the response to plasma exchange in patients with thrombotic thrombocytopenic purpura

“…Seventeen patients had idiopathic TTP and three cases presented post‐partum TTP. Diagnostic criteria have been previously reported (De la Rubia et al , 1999). Briefly, diagnosis was based on a platelet count < 100 × 10 9 /l, microangiopathic haemolytic anaemia with a negative direct antiglobulin test and increased serum lactate dehydrogenase (LDH) concentration.…”