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

J Font, José A. Font, S Jiménez, Sònia Jiménez, Ricard Cervera, R Cervera, Mario García-Carrasco, M García-Carrasco, Manuel Ramos-Casals, M Ramos-Casals, Jaume Campdelacreu, J Campdelacreu, M Ingelmo, Miguel Ingelmo
Annals of the rheumatic diseases · 2000 · vol. 59(11) , pp. 920–923 · doi:10.1136/ard.59.11.920 · PMID:11053074 · PMC1753027 · W2146177772
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This report describes two cases of refractory Evans' syndrome associated with antiphospholipid antibodies that required splenectomy after failing steroid and immunosuppressive treatments, alongside a review of previously reported cases.

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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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MeSH descriptors

Anemia, Hemolytic Antibodies, Antiphospholipid Antiphospholipid Syndrome Splenectomy Thrombocytopenia Adult Anemia, Hemolytic Anemia, Hemolytic Antibodies, Antiphospholipid Antiphospholipid Syndrome Antiphospholipid Syndrome Female Humans Immunosuppressive Agents Immunosuppressive Agents Middle Aged Thrombocytopenia Thrombocytopenia Treatment Outcome

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