12例伴单克隆免疫球蛋白Mκ血症的自身免疫性溶血性贫血患者临床分析

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. CONCLUSION: Mostly, CAS patients had monoclonal immunoglobulin IgMκ, but warm AIHA patients with monoclonal IgM were fewer.