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.

Relapse of Thrombotic Thrombocytopenic Purpura: Is It a Continuum of Disease?

“…In patients who have experienced multiple relapses, splenectomy has been shown to be beneficial in > 50% of patients in several single-institution series. [24][25][26][27] The benefit of splenectomy may not be immediate for many patients, and platelet recovery may be delayed several days to a week after surgery. In a recent series, one institution reported their experience of 14 patients who underwent splenectomy for refractory or relapsed TTP.…”

Minimal evidence of platelet and endothelial cell reactive antibodies in thrombotic thrombocytopenic purpura

“…Similarities between TTP and autoimmune thrombocytopenic purpura have provided rationale for use of corticosteroids and splenectomy in TTP [7,8]. Associations between TTP and systemic lupus erythematosis (SLE), the antiphospholipid antibody syndrome, and other autoimmune disorders provide further cause for suspicion of immunological mechanisms [1,9,10].…”

NEWASPECTSINTHEPATHOGENESISANDTREATMENTOFTHROMBOTICTHROMBOCYTOPENICPURPURAANDHEMOLYTICUREMICSYNDROME

“…Immunosuppression would seem rational, and could account for reports of therapeutic success in TM patients that have autoantibodies against VWF‐cleaving protease, endothelial cells or other relevant targets. Corticosteroids, vincristine, splenectomy, cyclosporine, combination chemotherapy, staphylococcal protein A immunoadsorption and other immunomodulatory measures have been reported to be of benefit in TM (97, 169–174). New immune modulating treatments, such as the anti‐B lymphocyte agent rituximab and anti‐TNF and TNF‐receptor agents infliximab and etanercept, have become available that could be beneficial in TM.…”