ANTICARDIOLIPIN ANTIBODY-RELATED THROMBOCYTOPENIA - PERSISTENT REMISSION AFTER SPLENECTOMY

The lupus anticoagulant (LAG) and anticardiolipin antibody (ACA) syndr omes require particular therapeutic approaches: thrombotic accidents a re an indication for oral anticoagulant therapy (OAT), whereas severe thrombocytopenia may require the special treatments used for immunolog ic thrombocytopenic purpura (ITP). We describe the case of a 21-year-o ld male who presented with axillary vein thrombosis associated with LA C and ACA at high titers in December 1990. OAT was begun and, due to r epeated episodes of thrombocytopenia, high-dose steroid therapy was la ter added with success. The daily steroid dose was reduced because of patent hypercortisolism, but the platelet count fell to 4x10(9)/L. A b one marrow biopsy was characteristic for ITP. Splenectomy was performe d in June 1993, and the platelet count rapidly normalized. Platelet an tibodies were always detectable before and after splenectomy. The pati ent is currently asymptomatic, with platelet counts above 300x10(9)/L at one and a half years after splenectomy, This case indicates that AC A-associated thrombocytopenia, Like ITP and HIV-related thrombocytopen ias, can be successfully treated with steroids and splenectomy, even t hough different pathogenetic mechanisms are responsible for the antibo dy-induced platelet consumption.