原发性皮肤边缘区B细胞淋巴瘤临床病理特征  

Clinical and pathologic features of primary cutaneous marginal zone B-cell lymphoma

作　　者：张虹[1] 郭华[1] 农琳[1] 李挺[1] 

机构地区：[1]北京大学第一医院病理科,北京100034

出　　处：《诊断病理学杂志》2008年第5期373-377,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨原发皮肤边缘区B细胞淋巴瘤(PCMZL)的临床和病理学特征,诊断和鉴别诊断。方法对2例PCMZL和1例皮肤淋巴组织增生(CLP)病例进行临床特征、组织形态学、免疫表型及PCRIgH基因重排分析。结果免疫组化示2例PCMZL的小淋巴细胞CD20和CD79α(),bcl-2、BOB.1和Oct-2(),MUM-1(+),CD5、CD10、bcl-6和CD23(-);浆细胞CD138、MUM-1(),限制性表达轻链kappa。1例CLP的小淋巴细胞CD20、CD3和bcl-2(),MUM1(+);浆细胞同时表达κ及λ。IgH扩增2例PCMZL呈单克隆性,1例CLP显示多克隆性。结论PCMZL属MALT-L家族,形态学上肿瘤细胞由异源性小淋巴细胞组成,免疫学上显示后生发中心标记。浆细胞轻链限制性和B细胞抗原受体基因克隆性重排对诊断有非常重要的帮助。Objective To discuss the clinical and pathological features of primary cutanous marginal zone B-cell lymphoma （PCMZL）, diagnosis and the differential diagnosis. Methods 2 cases of PCMZL and 1 case of cutanous lymphoid proliferation were studied with analysis of clinical features, histomorphology, immunochemistry and PCR IgH gene rearrangement. Results The immunostaining of case 1 and case 2 showed that the small lymphoid cells were positive for CD20 and CD79a, bcl-2, BOB. 1, Oct-2,and MUM-l, but negative for CD5, CD10, bcl-6 and CD23; the plasma cells were positive for CD138 and MUM-1, monotypic expression of immunoglobulin light chain - Kappa. Case 3 showed that the small lymphoid cells were positive for CD20, CD3, bcl-2, and MUM1 ; the plasma cells expressed both κ and λ. IgH gene rearrangement showed that case 1 and case 2 were clonai, and case 3 was polyclonal. Conclusions PCMZL is a family member of mucosa associated lymphoid tissue lymphomas, composed of a group of small B cells. These are very helpful for diagnosis if the neoplastic cells have the expression of postgerminal centre markers, the plasma cells show monotypic expression of immunoglobulin light chains, and B cell antigen receptor genes are clonally rearranged.

关 键 词：皮肤 原发性皮肤边缘区B细胞淋巴瘤 黏膜相关淋巴组织淋巴瘤 

分 类 号：R733.4[医药卫生—肿瘤]