The role of splenectomy in the treatment of relapsing thrombotic thrombocytopenic purpura

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a serious disorder of unknown etiology. Clinical findings are the result of vascular occlusions by platelet aggregates. Treatment with plasma exchange, often used in combination with corticosteroids, vincristine, aspirin, and dipyridamole, has reduced mortality to 20%. Relapses may occur even after long disease-free intervals. In this report we describe our experience with splenectomy in patients with relapsing TTP. Between July 1978 and March 1994, 16 patients with TTP were treated in our hospital. Five of the 13 patients surviving the first episode of TTP had relapses. Most relapses were treated as the first episode of TTP with plasma exchange with fresh-frozen plasma, followed by plasma infusions, corticosteroids, and vincristine. Sometimes aspirin and dipyridamole were added. Splenectomy was performed after five relapses in the first two patients and after two and three relapses in the other patients. Before splenectomy the disease-free interval varied from 3 weeks to 27 months and the incidence rate of relapses was 1.5 relapse/patient/year. None of the patients had relapses after splenectomy. The mean follow-up after splenectomy is 39 months with a range of 9-62 months. We conclude that patients with relapsing TTP can benefit from splenectomy, since it seems to increase disease-free intervals. Further investigation is necessary to understand the role of the spleen in the pathogenesis of TTP.

New strategies in diagnosis and treatment of thrombotic thrombocytopenic purpura: case report and review

“…Steroids [1], intravenous immunoglobulins [33], vincristine [21,29], antiplatelet agents [7,32] and other drugs have been tried with variable success. Occasionally, splenectomy proved bene®cial for patients with recurrent disease [17,42] but has never been regarded as the therapy of choice. For the last 30 years most regimens consisted of plasma infusion or plasma exchange with replacement by FFP.…”

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

“…In fact, plasma therapy is costly and entails risks due to exposure to blood products of numerous donors. Thus, alternative therapies like splenectomy may play a role in the management of this subset of patients [6, 12, 23, 24, 25]. In general, when performed in remission, splenectomy does not seem to be associated with severe complications [12, 24]and should be considered for those patients who present several relapses.…”

A case of thrombotic thrombocytopenic purpura in an adult treated with vincristine

“…Relapsing TTP still carries a significant mortality. Various therapeutic procedures have been used for resistant or relapsing patients, including splenectomy [24], antiplatelet drugs [6], high-dose immunoglobulins [7,9], and combined chemotherapy [21]. The resemblance of TTP to disorders in which vasculitis is prominent, e.g., systemic lupus erythematosus, prompted the use of cytotoxic agents such as vincristine sulfate in TTP patients.…”