摘要
目的探讨淋巴浆细胞淋巴瘤/华氏巨球蛋白血症(lymphoplasmacytic lymphoma/Waldenstr m’s macroglobulinemia,LPL/WM)的临床病理学特征。方法回顾性分析14例LPL/WM的临床资料、实验室检查、病理学检查及MYD88 L256P基因突变状况。结果14例LPL/WM中男性11例,女性3例,中位年龄59岁;12例有肝脏或脾脏肿大,11例有淋巴结肿大。13例贫血,13例分泌单克隆IgMκ,1例分泌单克隆IgMλ,14例均有肿瘤性细胞累及骨髓与MYD88 L256P基因突变。淋巴结活检显示淋巴结结构部分存在,均可见开放的淋巴窦。肿瘤性细胞由数量不等的小淋巴细胞、浆样淋巴细胞及浆细胞组成。其中1例在同一淋巴结内可见两类肿瘤性细胞:(1)以浸润淋巴窦为特征的大淋巴细胞;(2)浸润副皮质区和边缘窦的小淋巴细胞、浆样淋巴细胞及浆细胞。免疫表型:11例CD20弥漫阳性,10例限制性表达轻链Kappa,1例限制性表达轻链Lambda;2例CD23阳性,CD10、CD5均阴性,Ki-67增殖指数5%~30%;在同一淋巴结内可见两类肿瘤性细胞:大淋巴细胞中CD20、CD30、MUM1、BCL-2均阳性,Ki-67增殖指数>80%;小淋巴细胞中CD20、CD38、CD138、MUM1呈条索状或灶状阳性,Ki-67增殖指数<20%。骨髓流式细胞免疫表型:14例小淋巴细胞中CD20、CD19、CD22及sIgM均阳性,2例CD23阳性,CD10及CD5均阴性;浆细胞中CD19、CD138、CD38及CD45均阳性,CD56均阴性。结论LPL/WM尚无特异性的诊断指标,属于排除性诊断,需与其他小B细胞淋巴瘤尤其是伴浆细胞分化的小B细胞淋巴瘤鉴别。明确诊断需结合其他实验室检查(骨髓细胞形态学、流式免疫表型特征、免疫固定电泳及MYD88 L265P突变检查)。
Purpose To explore the clinicopathological features of lymphoplasmacytic lymphoma/Waldenstr m’s macroglobulinemia,in order to improve the understanding and diagnosis of this disease.Methods Clinical data,laboratory examination,pathological examination and mutation of MYD88 L256P gene in 14 LPL/WM patients were retrospectively analyzed.Results Eleven patients were male and three were female,with median age of 59 years.12 had enlarged liver or spleen,and 11 had enlarged lymph nodes.The 13 patients were anaemic,and all the 14 patients had different degrees of IgM immunoglobulinemia,MYD88 L256P gene mutation and myeloid involvement of neoplastic cells lymph node biopsy revealed the presence of a portion of the lymph node structure,especially an open sinus.Tumors were composed of varying numbers of plasma cells,small lymphocytes,and plasmacyte-like lymphocytes.Two types of neoplastic cells were found in the same lymph node in 1 patient.Another type was infiltrating paracortical area and marginal sinus small lymphocyte plasmacytoid lymphocyte and plasma cell.Immunophenotyping showed that 11 cases were diffusely positive to CD20.10 cases had light chain restricted expression of Kappa,and 1 case had light chain limited expression of Lambda.CD23,CD10 and CD5 were not expressed in two cases.The Ki-67 proliferation index was 5%-30%.In the same lymph node,two types of tumor cells were found:large lymphocytes expressed CD20,CD30,MUM1,BCL-2,Ki-67 proliferation index was greater than 80%.Small lymphocytes with CD20,CD38,CD138 and MUM1 expression showed cord-like arrangement and Ki-67 index of less than 20%.Flow cytometry of bone marrow showed that CD20,CD19 and CD22 were expressed in 14 cases of small lymphoid cells and CD10 and CD5 were not expressed but CD23 was positive,in 14 cases of small lymphoid cells,but expression of CD19,CD138,CD38 and CD45 was found in plasma cells.No CD56 expression was found in plasma cells.Conclusion There is no specific diagnostic index of LPL/WM,which needs to be differentiated from other smal
作者
张会超
王芃堉
丁雅雯
李宏
宋媛
高社军
李清靖
黄晨
刘丽宏
ZHANG Hui-chao;WANG Peng-yu;DING Ya-wen;LI Hong;SONG Yuan;GAO She-jun;LI Qing-jing;HUANG Chen;LIU Li-hong(Cinical Laboratory,Fourth Hospital of Hebei Medical University,Shijiazhuang 050011,China)
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2020年第2期163-168,共6页
Chinese Journal of Clinical and Experimental Pathology
基金
河北省科技厅大健康服务和生物医药专项课题(162777243)。
