Splenectomy for refractory Evans' syndrome associated with antiphospholipid antibodies: report of two cases

Abstract

The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive Coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed.



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Figure 1  .

Summary of the clinical and therapeutic course of patient 1. Ig = intravenous gammaglobulin; Aza = azathioprine.

Figure 2  .

Summary of the clinical and therapeutic course of patient 2. Ig = intravenous gammaglobulin.