骨髓多形性套细胞淋巴瘤的临床病理分析

Bone marrow involvement of pleomorphic mantle cell lymphoma:clinicopathological analysis of nine cases

目的 探讨骨髓多形性套细胞淋巴瘤的临床病理特征、免疫表型、诊断及鉴别诊断。方法 对9例多形性套细胞淋巴瘤进行骨髓活检、免疫组化染色、流式免疫表型、骨髓染色体核型分析并与13例经典型套细胞淋巴瘤、26例弥漫大B细胞淋巴瘤进行对比分析并复习相关文献。结果 骨髓多形性套细胞淋巴瘤中有核细胞均过度增生,肿瘤性淋巴细胞累及骨髓的比例均值为80%(70%~90%),呈“弥漫型”浸润模式,肿瘤细胞胞体大、核染色质细致,部分可见核仁,核分裂象小于1个/HPF。免疫表型为:CD20、CD5、CD38、FMC7、CD79b、P53、CyclinD1、SOX11阳性,部分CD71、IgM阳性,Ki-67增殖指数高,CD10、CD23、CD200、LEF1、c-myc、Bcl-6绝大部分阴性。少部分多形性套细胞淋巴瘤可出现复杂核型,而弥漫大B细胞淋巴瘤复杂核型比例高,经典型套细胞淋巴瘤极少出现复杂核型。结论 多形性套细胞淋巴瘤少见,诊断需结合形态、免疫表型及FISH-CCND1综合分析。

Objective To explore the clinicopathological features, immunophenotype, diagnosis, differential diagnosis of bone marrow involvement of pleomorphic mantle cell lymphoma(PMCL). Methods 9 cases of PMCL were studied by microscopic observation of bone marrow biopsy, immunohistochemical staining, flow cytometry and chromosome karyotypes. While the control groups included 13 cases of classic mantle cell lymphoma(CMCL)and 26 cases of diffuse large B-cell lymphoma(DLBCL). The related literatures were reviewed. Results The bone marrows were hypercelluar, the average proportion of neoplastic lymphocytes involving the bone marrow was 80%(70%-90%), and the pattern of bone marrow involvement of PMCL was diffuse. The tumor cells were large, nuclear chromatin was fine, some nucleoli were visible, and mitotic figures were less than 1/HPF. The tumor cells were positive for CD20, CD5, CD38, FMC7, CD79b, P53, CyclinD1, SOX11. Some cases expressed CD71, IgM, but in most cases lacked CD10, CD23, CD200, LEF1, c-myc, Bcl-6. The proliferation index Ki-67 was high.Conventional cytogenetic studies showed complex karyotype appeared in DLBCL cases more than PMCL cases. Conclusion The bone marrow involvement of PMCL is relatively rare. The correct diagnosis is based on clinicopathological features, immunophenotype and FISH-CCND1.