Analysis of unknown circulating anticoagulant cases

我们分析了新发现的未知循环抗凝病例。最近的病例是一名61岁的妇女，在感染带状疱疹病毒后出现高热、贫血和APTT延长。体格检查显示脾肿大，无淋巴结病变。实验室检测显示狼疮抗凝血剂（LA）和单克隆IgM κ蛋白。LA与抗β 2gpi抗体、抗心磷脂抗体、抗凝血酶原抗体存在相关。此外，因子IX、XI、XII和c反应蛋白、FDP -二聚体的检测结果受到其检测原理（乳胶凝集试验）的干扰。行脾切除术，诊断为SMZL。脾切除术后所有血液学指标均迅速恢复。在未预防血栓形成的情况下，脾切除术后未发生血栓事件。临床过程提示产生SMZL的抗体在实验室检查中引起免疫异常。由于她在脾切除术后不久疾病复发，我们采用利妥昔单抗进行自体外周干细胞移植。另一个病例，49岁男性，狼疮抗凝剂重症肌无力与IgM伽玛病相关。患者IgM副蛋白对凝血因子IX、XI、XII、prekallikrein和高分子量激肽原均有非特异性抑制。给予强的松龙治疗，肌无力症状有所改善，但APTT和巨球蛋白血症仍持续。双滤过血浆置换成功地将血清IgM水平从1190降至375mg/dl， APTT从58秒降至38秒。重症肌无力常与其他自身免疫性疾病相关，但与狼疮抗凝剂和IgM γ病相关的情况很少见。

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.