慢性淋巴细胞白血病的Richter's征转化  

Chronic lymphocytic leukemia transformation to Richter' s syndrome

作　　者：王建宁[1] 张平[2] 候艳秋[1] 张柳波[1] 宋敏[1] 孟庆奇[1] 包红雨[1] 傅行财[1] 

机构地区：[1]南京医科大学第二附属医院血液科,江苏南京210011 [2]南京医科大学第二附属医院病理科,江苏南京210011

出　　处：《现代肿瘤医学》2012年第9期1926-1930,共5页Journal of Modern Oncology

摘　　要：目的:探讨Richter's征(RS)的临床特征、预测指标、治疗和预后因素。方法:报告2例慢性淋巴细胞白血病(CLL)向RS转化并结合文献进行复习。结果:患者表现为进行性淋巴结肿大,伴乏力、盗汗,经血细胞形态学、免疫分型确定存在CLL克隆,淋巴结病理1例示弥漫性大B细胞淋巴瘤、另1例为小B细胞淋巴瘤向大细胞淋巴瘤转化。结论:RS为少见疾病,预后较差,诊断时需有病理证实CLL向侵袭性淋巴瘤转化,治疗应采用含利妥昔单抗的多药联合化疗,年轻患者如对初始治疗反应较好且有合适供体应进行异基因移植。Objective：To explore the clinical features, predicting parameters, treatment and unfavorable factors of Richter＇s syndrome（RS）. Methods： Two eases of chronic lymphocytic leukemia（CLL） transformation to RS were re-ported and the related articles were reviewed. Results ： The patients presented the rapid increase in the size of lymph-oid masses with fatigue and drenching sweats. CLL was diagnosed with examinations of peripheral blood or marrow smears, and flow cytometric immunophenotypes of lymphocytes. One case as diffuse large B - cell lymphoma and an-other as small B - cel! lymphoma transformation to large cell lymphoma were confirmed by the biopsy of lymph nodes. Conclusion ： RS is rare and with a poor prognosis. Diagnosis of RS requires pathologic demonstration of CLL transfor-mation to aggressive lymphoma. RS should be treated with a combination of rituximab and polyebemotherapy. The younger patients who respond to initial therapy should be offered allogeneic stem cell transplantation, if feasible.

关 键 词：慢性淋巴细胞白血病 Richter's征 弥漫性大B细胞淋巴瘤 

分 类 号：R733.72[医药卫生—肿瘤]