伴双克隆M蛋白的淋巴浆细胞淋巴瘤一例并文献复习

Lymphoplasmacytic lymphoma with biclonal M protein:report of one case and review of literature

目的探讨伴有双克隆M蛋白的淋巴浆细胞淋巴瘤(LPL)的临床特点、诊断、治疗方法。方法回顾性分析江苏省盐城市第一人民医院2018年1月收治的1例伴有双克隆M蛋白的LPL患者的临床资料,并进行相关文献复习。结果该患者为老年女性,临床主要表现为淋巴结肿大、肾脏损害、贫血、骨破坏等,依据淋巴结活组织检查、免疫固定电泳、骨髓细胞学、基因突变检测(MYD88 L265P突变阳性)明确诊断。采用硼替佐米为主的方案治疗4个周期,达到部分缓解。结论伴有双克隆M蛋白的LPL临床主要表现出一种M蛋白特点,免疫球蛋白IgM及IgA双克隆的LPL罕见,采用硼替佐米为主的治疗方案有一定的效果。

Objective To investigate the clinical features,diagnosis and treatment of lymphoplasmacytic lymphoma(LPL)with biclonal M protein.Methods The clinical data of one LPL patient with biclonal M protein at Yancheng First People's Hospital in January 2018 was retrospectively analyzed,and relevant literature was reviewed.Results The patient was an elderly woman with clinical manifestations of lymphadenopathy,kidney damage,anemia,and bone destruction.The diagnosis was confirmed based on lymph node biopsy,immunofixation electrophoresis,bone marrow cytology,and genetic mutation testing(MYD88 L265P mutation-positive).Partial remission was achieved after 4 courses of treatment with bortezomib-based regimen.Conclusions Clinically,LPL with biclonal M protein shows one characteristic of M protein,and the immunoglobulin IgM and IgA biclonal LPL is even rarer.The treatment scheme based on bortezomib has a certain therapeutic effect.