Splenectomy for the treatment of thrombotic thrombocytopenic purpura

Abstract

SummaryPlasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these conditions but its value remains controversial. We report on a series of 33 patients with TTP who were splenectomised because they were plasma refractory (n ¼ 9) or for relapsed disease (n ¼ 24). Splenectomy generated prompt and unmaintained remissions in all except five patients, in whom remission was delayed (n ¼ 4) or who died with progressive disease (n ¼ 1). Four postoperative complications occurred: one pulmonary embolism and three surgical complications. Median follow-up after splenectomy was 109 months (range 28-230 months). The overall postsplenectomy relapse rate was 0AE09 relapses/patient-year and the 10-year relapse-free survival (RFS) was 70% (95% CI 50-83%). In the patients with relapsing TTP, relapse rate fell from 0AE74 relapses/patient-year before splenectomy to 0AE10 after splenectomy (P < 0AE00001). Two patients died from first postsplenectomy relapse. Although these results are based on retrospective data and that the relapse rate may spontaneously decrease with time, we conclude that splenectomy, when performed during stable disease, has an acceptable safety profile and should be considered in cases of plasma refractoriness or relapsing TTP to reach durable remissions and to reduce or prevent future relapses.

Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab

“…Rituximab has been used as pre-emptive treatment in patients with anti-ADAMTS13 autoantibodies [21], thus rituximab maintenance maybe an option, however possibly related to long term side effects like progressive multifocal 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 leucoencephalopathy [22]. An alternative treatment option would be a splenectomy, which is supported by data provided by Kappers-Klunne et al 2005 [23]. 33 patients with TTP were retrospectively reported who were splectomised due to refractory or relapsing disease.…”

A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura

“…Several small patient series describe various therapies, such as vincristine, 16 cyclophosphamide, or splenectomy. 17 Ciclosporin has been used with beneficial responses within 7-14 days of therapy; however, 14% of patients relapsed while on therapy and 33% relapsed within 3 months of stopping. 18,19 There are no studies adequately powered to prove consistent clinical benefit.…”

Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange

“…However, few patients with refractory TTP treated with splenectomy during the acute phase had a successful outcome, which challenges assertions about its efficacy in the treatment of refractory TTP. 22 However, in a retrospective study, 15 patients with severe TTP underwent splenectomy 20 days after diagnosis. One patient died the day after splenectomy.…”