单克隆IgM升高的B细胞/浆细胞肿瘤3例报告及文献复习  

B-CELL/PLASMA CELL TUMOR WITH ELEVATED MONOCLONAL IGM:A REPORT OF THREE CASES AND LITERATURE REVIEW

作　　者：张婷[1] 黄俊霞[1] 高燕[1] 钟玉萍[2] 冯献启[1] 孟繁军[1] ZHANG Ting;HUANG Junxia;GAO Yan;ZHONG Yuping;FENG Xianqi;MENG Fanjun(Department of Hematology,The Affiliated Hospital of Qingdao University,Qingdao 266003,China)

机构地区：[1]青岛大学附属医院血液内科,山东青岛266003 [2]青岛市市立医院血液内科

出　　处：《青岛大学学报（医学版）》2021年第3期459-462,共4页Journal of Qingdao University(Medical Sciences)

基　　金：重大疾病防治科技行动计划项目(2018ZX-07S-007)。

摘　　要：目的探讨单克隆IgM免疫球蛋白病的临床表现、病理特点、诊断及鉴别诊断方法。方法回顾性分析3例单克隆IgM升高的B细胞/浆细胞肿瘤病人的临床资料,并进行文献复习。结果 3例病人均存在贫血、IgM升高。例1病人骨髓病理及免疫组化检查倾向考虑为小B细胞淋巴瘤,结合MYD88 L265P突变阳性可进一步明确诊断为华氏巨球蛋白血症;例2病人淋巴结病变病理检查示符合边缘区B细胞淋巴瘤伴浆细胞样分化,免疫组化提示滤泡树突细胞网,结合MYD88 L265P突变阴性可进一步明确诊断为淋巴结边缘区淋巴瘤;例3病人根据肾脏损害、骨髓检查、活检提示浆细胞骨髓瘤以及MYD88 L265P突变阴性明确诊断为IgM型多发性骨髓瘤。结论单克隆IgM免疫球蛋白病鉴别诊断困难,需结合骨髓细胞学、骨髓流式免疫分型、骨髓活检、骨髓基因及染色体检查等结果综合诊断。Objective To investigate the clinical manifestations,pathological features,diagnosis,and differential diagnosis of monoclonal IgM-related diseases.Methods A retrospective analysis was performed for the clinical data of three patients with B-cell/plasma cell tumor with elevated monoclonal IgM,and a literature review was performed.Results All three patients had anemia and elevated IgM.For case 1,bone marrow pathology and immunohistochemistry tended to consider small B-cell lymphoma,and combined with the positive MYD88 L265P mutation,the patient was diagnosed with Waldenstrom macroglobulinemia.For case 2,pathological examination of lymphadenopathy showed marginal zone B-cell lymphoma with plasmacytoid differentiation,and immunohistochemistry suggested follicular dendritic cell network;the patient was further diagnosed with nodal marginal zone lymphoma based on negative MYD88 L265P mutation.For case 3,the patient was diagnosed with IgM type multiple myeloma based on renal damage,plasma cell myeloma suggested by bone marrow test and biopsy,and negative MYD88 L265P mutation.Conclusion There are difficulties in the differential diagnosis of monoclonal IgM-related diseases,which requires a comprehensive diagnosis based on bone marrow cytology,bone marrow flow immunophenotyping,bone marrow biopsy,bone marrow genes,and chromosomes.

关 键 词：免疫球蛋白M WALDENSTROM巨球蛋白血症 淋巴瘤 B细胞 边缘区 多发性骨髓瘤 髓样分化因子88 突变 诊断 鉴别 

分 类 号：R733[医药卫生—肿瘤]