原发CD5阳性弥漫大B细胞淋巴瘤的临床病理特点

Clinicopathological characteristics of de novo CD5-positive diffuse large B-cell lymphoma

目的:研究原发CD5阳性弥漫大B细胞淋巴瘤(CD5-positive diffuse large B cell lymphoma,CD5^+ DLBCL)的临床、免疫表型、细胞遗传学特点及治疗反应和预后。方法:回顾分析7例通过免疫组化和流式细胞免疫分析确诊的CD5^+ DLBCL的临床和免疫表型特点,细胞遗传学异常及治疗和预后。结果:7例患者中位年龄70岁。3例以淋巴结起病,同时有骨髓累及。2例以淋巴结合并乳腺肿块或胃肿块起病。2例分别以牙龈肿块和胃溃疡作为单纯结外病变起病,免疫组化示免疫表型为CD5^+、CD10^-、CD19^+、CD20^+、CD23^-,Ki-67为50%~80%。Hans分型均为非生发中心亚型。2例累及骨髓者有复杂染色体异常。联合化疗治疗后3例完全缓解,1例部分缓解,3例进展。3例完全缓解病人中2例分别在缓解20和24个月后复发,仅1例持续存活已30个月。结论:CD5^+ DLBCL侵袭性强,治疗效果差,长期生存率低。

Objective：To investigate the clinical,immunophenotypic,and cytogenetic characteristics and effects of the treatment and prognosis of de novo CD5 -positive diffuse large B -cell lymphoma （CD5 ＋ DLBCL）.Methods：The clinical and immunophenotypic characteristics,cytogenetic abnormalities,and effects of treatment and prognosis were reviewed in 7 cases of CD5 ＋ DLBCL confirmed by immunohistochemistry or flow cytometry.Results：The median age of the patients was 70 years.There were 3 cases primarily diagnosed because of lymphadenopathy with bone mar-row involvement,2 because of gingival lumps and gastric ulcer respectively,and 2 because of lymphadenopathy associ-ated with breast or gastric mass.Immunohistochemistry indicated that the immunophenotype of CD5 ＋ DLBCL was CD5 ＋,CD10 -,CD19 ＋,CD20 ＋ and CD23 -,and the proportion of Ki -67 positive cells was 50% ~80%.All cases were classified according to the Hans classification system into non -germinal center subtypes.There were 2 cases that involved bone marrow that were associated with complex chromosomal abnormalities.After receiving combined chemotherapy,complete remission was achieved in 3 cases,among them 1 relapsed in 20 and 1 in 24 months.The third patient survived for 30 months.Conclusion：CD5 ＋ DLBCL is highly aggressive with unsatisfactory results from current treatment.