Objective: To explore the clinical features of autoimmune hemolytic anemia (AIHA) with monoclonal gammopathy IgMκ.
Methods: The clinical and laboratory features of 12 AIHA with monoclonal gammopathy IgMκ were retrospectively analyzed.
Results: 12 cases with monoclonal immunoglobulin IgMκ were found in 85 patients with AIHA by immune-fixation electrophoresis from June 2012 to June 2014. There were 4 (5.7% ) cases of warm AIHA and 8 (80.0% ) cases of cold agglutinin syndrome (CAS). The 4 warm AIHA were primary type, and 4 CAS cases were secondary to lymphoproliferative disorder (small-cell lymphocytic lymphoma) and the other 4 CAS were primary type. Positive TCR gene rearrangements were detected in 2 warm AIHA patients; IgH rearrangements positive were detected in 6 CAS patients, and TCR/IgH rearrangements positive were seen in 1 CAS patient. Four warm AIHA cases received glucocorticoid treatment, three cases of complete remission, one case of partial response. Three CAS cases were treated with low-dose of rituximab, two cases of partial response and one case of invalid. Two CAS patients received chemotherapy of COP regimen, one case of partial response and one case of invalid. Two CAS patients of normal hemoglobin were suggested to keep warm, and one case died of infection after splenectomy.
Conclusions: Mostly, CAS patients had monoclonal immunoglobulin IgMκ, but warm AIHA patients with monoclonal IgM were fewer.
目的: 探讨伴单克隆免疫球蛋白Mκ（IgMκ）血症的自身免疫性溶血性贫血（AIHA）临床特征。
方法: 回顾性分析85例AIHA患者临床资料，观察伴单克隆IgMκ的AIHA患者一般临床特征、实验室检查特点及转归。
结果: 85例AIHA患者中12例（14.1%）单克隆IgM κ阳性，其中4例温抗体型AIHA, 8例冷凝集素综合征（CAS）。4例温抗体型AIHA患者均为原发病例，免疫表型未见异常；8例CAS患者中，4例继发于小B细胞淋巴瘤，4例为原发CAS，免疫表型未见异常。4例温抗体型AIHA患者中2例TCR重排阳性；8例CAS患者中6例IgH重排阳性，1例TCR/IgH重排均阳性。4例温抗体型AIHA患者均给予糖皮质激素治疗，3例达完全缓解，1例部分缓解。8例CAS患者中3例给予小剂量利妥昔单抗治疗，2例部分缓解，1例无效；2例给予COP（环磷酰胺、长春新碱、甲泼尼龙）方案化疗，1例部分缓解，1例无效；2例HGB正常患者给予保暖对症治疗；1例院外行脾脏切除术，术后死于感染。
结论: CAS常伴有单克隆IgMκ，而温抗体型AIHA伴单克隆IgMκ较少见。