轻链型淋巴浆细胞样淋巴瘤1例并文献复习

目的：提高对淋巴浆细胞样淋巴瘤（LPL）的认识。方法：报告1例轻链型LPL并结合文献进行讨论。结果：患者女性，62岁，无淋巴结和肝脾肿大，贫血、血小板减少，肾功能损害，血涂片浆细胞样淋巴细胞易见，骨髓淋巴细胞弥漫性增多，浆细胞样淋巴细胞浸润，淋巴细胞、浆细胞增多，血清IgG、IgA、IgM水平较低，血清免疫固定电泳示单克隆性K链阳性，外周血淋巴细胞免疫表型为sIg^-CD5^-CD10^-CDl9^＋CD20^＋CD23^-，染色体正常。结论：LPL属于小B细胞淋巴瘤，血清中单克隆球蛋白异常主要为IgM，非IgM型少见并易于误诊，诊断依赖于骨髓中浆细胞样淋巴细胞弥漫性浸润和淋巴细胞、浆细胞增多，M蛋白的确定，淋巴细胞免疫表型．并排除其他CD5-的小细胞淋巴瘤。

Lymphoplasmacytic lymphoma with monoclonal light chain protein. A case report and literature review

Objective：To improve the recognition of lymphoplasmacytic lymphoma （LPL）. Methods： A case diagnosed as LPL with monoclonal light chain protein was reported and the related articles were reviewed. Results：A 62 - year - old female patient was characterized by no evidence of lymphadenopathy and hepatosplenomegaly, anemia and thrombocytopenia, impaired renal function, appearing of plasmacytoid lymphocytes on the peripheral blood smears, diffuse proliferation of small lymphocytes with infiltration by plasmacytoid lymphocytes and plasma cells in the marrow, low concentration of serum Igs（ IgG, IgA and IgM）, positive of a monoclonal serunk light chain band in the immunofixation elecrophoresis, expression at the immunophenotypes of antigens sIg^- CD5^- CD10^-CD19^＋ CD20^＋ CD23^ - in the peripheral blood sample and normal karytype in the cytogentic analysis. Conclusion： LPL is a small B -cell neoplasm and associated characteristieally with monoelonal serum immunoglobulin M （]gM） paraprotein. Non - IgM subtype of this disorder is rare and to be easily misdiagnosed. Establishing the diagnosis with LPL is supported by the infiltration of lymphoplasmacytic cells into the bone marrow with the proliferation of lymphoeytes and plasma cells, demonstration of serum monoclonal protein （ M protein ） and immunophenotypic features of lymphocytes. It is necessary to distinguish LPL from other CD5 - negative chronic B cell lymphomas.