伴单克隆IgM增高的淋巴结边缘区淋巴瘤1例并文献复习  

Nodal marginal zone lymphoma with elevated monoclonal IgM:report of 1 case and review of literature

作　　者：蔡伟欣 周美玲 舒凌 卢锦波 王玲玲 徐亚 徐浩 程月新 Cai Weixin;Zhou Meiling;Shu Ling;Lu Jinbo;Wang Lingling;Xu Ya;Xu Hao;Cheng Yuexin(Department of Hematology,Yancheng Clinical College of Xuzhou Medical University,Yancheng No.1 People's Hospital,the Fourth Affiliated Hospital of Nantong University,Yancheng 224006,China)

机构地区：[1]徐州医科大学盐城临床学院、盐城市第一人民医院、南通大学第四附属医院血液科,盐城224006

出　　处：《白血病．淋巴瘤》2022年第12期725-729,共5页Journal of Leukemia & Lymphoma

摘　　要：目的探讨伴单克隆IgM增高的淋巴结边缘区淋巴瘤的临床病理特征及诊断、鉴别诊断和治疗方法。方法回顾性分析2020年7月于盐城市第一人民医院诊治的1例伴单克隆IgM增高的淋巴结边缘区淋巴瘤患者的临床资料,并结合相关文献进行分析。结果患者为57岁女性,因乏力、左胸肋骨疼痛就诊。血清免疫固定电泳提示IgM-κ型M蛋白血症;骨髓形态学检查可见少量浆细胞样淋巴细胞,骨髓活组织检查和免疫组织化学检查提示B细胞非霍奇金淋巴瘤,骨髓基因检测MYD88 L265P和CXCR4均阴性;腹膜后淋巴结穿刺活组织术后病理提示边缘区淋巴瘤(成熟小B细胞类,倾向淋巴结边缘区淋巴瘤),免疫组织化学:CD3、CD5、CD138、κ、λ、CD10、Cyclin D1均阴性,CD20、Pax-5、CD23(FDC)、bcl-2均阳性,Ki-67阳性指数<5%。综合检查诊断为伴单克隆IgM增高的淋巴结边缘区淋巴瘤。采取减低剂量的CHOP方案化疗8个周期,疾病部分缓解;予沙利度胺维持治疗,截至2021年8月,病情平稳,持续随访中。结论伴单克隆IgM增高的淋巴结边缘区淋巴瘤较少见,诊断时应注意和华氏巨球蛋白血症、其他惰性B细胞淋巴瘤相鉴别。目前尚无标准治疗方案,遵循个体化治疗原则可以改善患者的预后。Objective To investigate the clinicopathological features,diagnosis,differential diagnosis and treatment of nodal marginal zone lymphoma(NMZL)with elevated monoclonal IgM.Methods The clinical data of one NMZL patient with elevated monoclonal IgM treated at Yancheng No.1 People's Hospital in July 2020 were retrospectively analyzed,and the related literature was analyzed.Results The patient was a 57-year-old female and the main clinical manifestations were fatigue and bone pain in left rib.Serum immunofixation electrophoresis showed IgM-κtype M proteinemia,bone marrow cytology showed a few plasmacytoid lymphocytes,bone marrow biopsy and immunohistochemistry showed B-cell non-Hodgkin lymphoma,bone marrow genetic testing showed MYD88 L265p and CXCR4 were both negative,postoperative pathology result of retroperitoneal lymph node biopsy was marginal zone lymphoma(mature small B type,prone to NMZL),and immunohistochemistry results:CD3,CD5,CD138,κ,λ,CD10,Cyclin D1 were negative,CD20,Pax-5,CD23(FDC),bcl-2 were positive;Ki-67 positive index<5%.The final diagnosis was NMZL with elevated monoclonal IgM.Partial remission was achieved after 8 cycles of reduced-dose CHOP regimen;thalidomide was used in the maintenance treatment,the disease condition was stable until August in 2021 and the follow-up was continuing.Conclusions NMZL with elevated monoclonal IgM is relatively rare.Its diagnosis should be differentiated from Waldenström macroglobulinemia and other inert B-cell lymphomas.Currently,there is no standard treatment and following the principle of individualized treatment can improve the prognosis of patients.

关 键 词：淋巴瘤 B细胞 边缘区 淋巴结 抗体 单克隆 免疫球蛋白M 

分 类 号：R733.1[医药卫生—肿瘤]