Analysis of unknown circulating anticoagulant cases

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We analyzed newly found unknown circulating anticoagulant cases. The recent case, a 61-year-old woman, had high fever with anemia and prolongation of the APTT after suffering Herpes Zoster virus infection. Physical examination revealed a large splenomegaly without lymphadenopathy. Laboratory evaluation showed lupus anticoagulant(LA) and monoclonal IgM κ protein. LA was associated with the presence of anti-β2GPI antibody, and anti-cardiolipin antibody, and anti-prothrombin antibody. Moreover, the results of factors IX,XI,and XII assays and C-reactive protein, FDP D-dimer were disturbed by its assay principle(Latex agglutination test). A splenectomy was performed and SMZL was diagnosis. All hematological findings were recovered rapidly after splenectmy. No thrombotic event occurred after splenectmy without prophylaxis of thrombosis. The clinical course suggested that antibodies producing SMZL induced immunological abnormalities in laboratory tests. Since she suffered recurrent of disease soon after splenectmy, we performed autologous peripheral stem cell transplantation with rituximab. Another case, a 49-year-old man, was lupus anticoagulant in myasthenia gravis associated with IgM gammopathy. The IgM paraprotein of patient displayed non specific inhibition to coagulant factors IX,XI,XII, prekallikrein and high molecular weight kininigen. He was placed on predonisolone, which resulted in improvement in his myasthenia symptoms, but the prolongation of APTT and macroglobulinemia remained. Double filtration plasmapheresis successfully decreased the serum IgM level from 1,190 to 375mg/dl, and APTT improved from 58 to 38 sec. Myasthenia gravis is frequently associated with other autoimmune diseases, but the association with lupus anticoagulant and IgM gammopathy is rare.