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首页> 外文期刊>British Journal of Haematology >Diffuse large B-cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): A cohort study of newly diagnosed patients
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Diffuse large B-cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): A cohort study of newly diagnosed patients

机译：慢性淋巴细胞性白血病（CLL）患者的弥漫性大B细胞淋巴瘤（Richter综合征）：一项新诊断患者的队列研究

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Nearly all information about patients with chronic lymphocytic leukaemia (CLL) who develop diffuse large B-cell lymphoma [Richter syndrome (RS)] is derived from retrospective case series or patients treated on clinical trials. We used the Mayo Clinic CLL Database to identify patients with newly diagnosed CLL between January 2000 and July 2011. Individuals who developed biopsy-proven RS during follow-up were identified. After a median follow-up of 4 years, 37/1641 (2·3%) CLL patients developed RS. The rate of RS was approximately 0·5%/year. Risk of RS was associated with advanced Rai stage at diagnosis (P < 0·001), high-risk genetic abnormalitites on fluorescence in situ hybridization (P < 0·0001), unmutated IGHV (P = 0·003), and expression of ZAP70 (P = 0·02) and CD38 (P = 0·001). The rate of RS doubled in patients after treatment for CLL (1%/year). Stereotyped B-cell receptors (odds-ratio = 4·2; P = 0·01) but not IGHV4-39 family usage was associated with increased risk of RS. Treatment with combination of purine analogues and alkylating agents increased the risk of RS three-fold (odds-ratio = 3·26, P = 0·0003). Median survival after RS diagnosis was 2·1 years. The RS prognosis score stratified patients into three risk groups with median survivals of 0·5 years, 2·1 years and not reached. Both underlying characteristics of the CLL clone and subsequent CLL therapy influence the risk of RS. Survival after RS remains poor and new therapies are needed.
机译：几乎所有有关发展为弥漫性大B细胞淋巴瘤[Richter综合征（RS）]的慢性淋巴细胞性白血病（CLL）患者的信息均来自回顾性病例系列或接受临床试验治疗的患者。我们使用Mayo诊所CLL数据库来识别2000年1月至2011年7月之间新诊断为CLL的患者。确定了在随访期间发生了活检证实的RS的患者。中位随访4年后，有37/1641（2·3％）CLL患者出现了RS。 RS的发生率约为0·5％/年。 RS的风险与诊断时的Rai晚期（P <0·001），荧光原位杂交的高风险遗传异常（P <0·0001），未突变的IGHV（P = 0·003）和ZAP70（P = 0·02）和CD38（P = 0·001）。接受CLL治疗的患者中RS的发生率翻倍（1％/年）。刻板的B细胞受体（奇数比= 4·2; P = 0·01）而不是IGHV4-39家庭使用与RS风险增加相关。嘌呤类似物和烷化剂的组合治疗会增加RS发生三倍的风险（比值= 3·26，P = 0·0003）。 RS诊断后的中位生存期为2·1年。 RS预后评分将患者分为三个风险组，中位生存期为0·5年，2·1年，未达到。 CLL克隆的基本特征和随后的CLL治疗都会影响RS的风险。 RS后的生存仍然很差，需要新的疗法。

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