Analysis of unknown circulating anticoagulant cases

我们分析了新发现的未知循环抗凝剂病例。最近的病例为一名61岁妇女，在感染带状疱疹病毒后出现高烧伴贫血和APTT延长。体格检查发现一个巨大的脾肿大，没有淋巴结病。实验室评估显示狼疮抗凝剂(LA)和单抗Ig Mκ蛋白。LA与抗β2GPI抗体、抗心磷脂抗体、抗凝血酶原抗体相关。此外，因子IX、XI、XII和C-反应蛋白、D-二聚体的测定结果受到其测定原理(乳胶凝集试验)的干扰。行脾切除术，确诊为SMZL。所有血液学检查结果均在脾切除后迅速恢复。未经血栓预防的脾切除术后无血栓事件发生。临床过程表明，在实验室测试中，产生SMZL的抗体会导致免疫异常。由于她在脾切除后不久病情复发，我们用利妥昔单抗进行了自体外周干细胞移植。另一例，49岁男性，狼疮抗凝剂治疗重症肌无力合并IgM丙种球蛋白病。患者的Ig M副蛋白对凝血因子IX、XI、XII、前激肽释放酶和高分子激肽原有非特异性抑制作用。他接受强的松龙治疗后，肌无力症状有所改善，但APTT和巨球蛋白血症仍有延长。双滤血浆置换成功地将血清IgM水平从1190 mg/dl降至375 mg/dl，APTT由58秒提高至38秒。重症肌无力经常与其他自身免疫性疾病相关，但与狼疮抗凝剂和IgM丙种球蛋白病的相关性很少。

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.