摘要
目的总结Richter综合征的I临床特点，探讨其早期发现和成功治疗的经验。方法回顾性分析2009年1月至2011年12月中国医学科学院北京协和医学院血液学研究所诊断的5例Richter综合征患者初诊及转化后的临床特点及其治疗情况。结果5例Richter综合征患者中，男4例，女1例，初诊慢性淋巴细胞白血病（慢淋）的中位年龄为47（44—68）岁，诊断慢淋到发生Richter综合征转化的中位时间为52（5—90）个月。4例进行了荧光原位杂交（FISH）检查，3例发现为17p13缺失。Richter综合征转化时主要表现为局部淋巴结进行性肿大者2例，全身多处淋巴结肿大者3例，其中l例伴发热，1例伴多发浆膜腔积液。5例患者均经病理证实转化为弥漫性大B细胞淋巴瘤（经典型转化），其中生发中心来源3例，非生发中心来源1例，1例不详。转化后1例进行了同胞供者的异基因造血干细胞移植，至2012年4月已生存24个月；1例患者进行了自体造血干细胞移植，生存12个月后复发死亡；1例患者为转化晚期因伴严重并发症而放弃治疗；2例患者转化后经治疗获得部分缓解，分别无进展生存20和8个月。结论Richter综合征多发生在慢淋终末期，但从诊断到转化的时间可早可晚。高危细胞遗传学异常17p缺失可能与Richter综合征转化有关。在转化早期及时发现，选择合适治疗可以延长生存。异基因移植有可能治愈该病。
Objective To summarize the clinical characteristics of Richter syndrome and explore the methods of successful treatment and timely diagnosis. Methods Five patients with Richter syndrome in the last three years （from January 2009 to December 2011 ）were analyzed retrospectively at our hospital, including their clinical features and therapy before and after transformation. Results There were 4 males and 1 female with a median age on a diagnosis of chronic lymphocytic leukemia （CLL） at 47 （44 -68）years. The median .duration from a diagnosis of CLL to transformation was 52 （5 -90） months. As for cytogenetic abnormalities, 3/4 patients had 17p deletion by fluorescence in situ hybridization （FISH）. The clinical manifestations on transformation included regional enlargement of lymph node （ n = 2 ） and systemic enlargement of lymph nodes （ n = 3 ） . All diagnoses were confirmed by lymph node biopsy and all transformed into diffuse large B cell lymphoma （classical transformation）. The subgroups were germinal center B-cell like （GCB） （ n = 3 ） and non-GCB （ n = 1 ）. After transformation, one patient underwent sibling allo-stem cell transplantation and survived 24 months until April 2012. Another patient with auto-stem cell transplantation relapsed and died 12 months later. One patient lost the treatment opportunity due to worsening condition. Another 2 patients gained partial remission after therapy and survived 20 and 8 months respectively. Conclusions Richter syndrome may occur during a late stage of CLL. Such a high-risk cytogenetic abnormality as dellTp may be correlated with transformation. Early identification and optimal therapy may extend the survival of Richter syndrome. Allo-stem cell transplantation remains a curable option.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2012年第46期3254-3256,共3页
National Medical Journal of China
基金
国家科技重大专项（2011zX09302-D07-D4）
天津市科技计划项目（09ZCZDSF03800）
