原发骨髓弥漫大B细胞淋巴瘤三例报告并文献复习

Primary bone marrow diffuse large B cell lymphoma： three case reports and literature review

目的 通过报道3例罕见的原发骨髓弥漫大B细胞淋巴瘤（DLBCL）患者的诊断、鉴别诊断和治疗经过,提高对该病的认识.方法 分析患者的发病经过、临床特点、治疗反应和转归,对患者的骨髓标本进行细胞形态学、流式细胞术、免疫组织化学和分子生物学检测以助诊断和鉴别诊断.结果 3例患者发病年龄偏大（56、60、70岁）,均以血常规异常起病,呈侵袭性经过,查体及影像学检查未发现淋巴结及肝、脾肿大,完善骨髓细胞形态学、流式细胞术、免疫组织化学等检查后可明确诊断为DLBCL,原发骨髓.予以含利妥昔单抗（R）的CHOP（环磷酰胺＋表阿霉素＋长春新碱＋泼尼松）方案或R-EPOCH（R＋依托泊苷＋长春新碱＋吡柔比星＋环磷酰胺＋泼尼松）方案化疗,近期疗效可达完全缓解,远期疗效仍待进一步评价.结论 原发骨髓的DLBCL非常特殊和罕见,这是国内首次报道,相关的发病机制和治疗策略有待于进一步深入探索.

Objective To report the diagnosis, differential diagnosis and treatment of three rare cases of primary bone marrow diffuse large B cell lymphoma （DLBCL）, and to improve the recognition of this disease. Methods The clinical characteristics, therapeutic course and the outcome of these patients were reviewed. Meanwhile, a series of examinations including morphology, flow cytometry, immunohistochemistry and molecular biology of bone marrow samples were also performed. Results These three patients who were old at the onset age （56, 60 and 70 years old）, primarily revealed as abnormal blood count and experienced an aggressive course of disease. Physical and imaging examination showed no enlargement of lymph node, liver and spleen, the patients were finally diagnosed as primary bone marrow DLBCL by bone marrow morphology, flow cytometry and immunohistochemistry analyses. They were treated with rituximab combined chemotherapy, which achieved a complete response, but still need longer follow-up to fiu＇ther evaluate their survival. Conclusions Primary bone marrow DLBCL was encountered rarely in clinical practice, and this is the first report in China, Further investigation of pathogenesis and therapeutic strategies of this rare disease was warranted.