Richter transformation in 16 of 149 Chinese patients with chronic lymphocytic leukemia
Richter transformation in 16 of 149 Chinese patients with chronic lymphocytic leukemia
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149 例中国慢性淋巴细胞白血病患者中 16 例进行里克特变换
DOI：
10.3109/10428194.2012.664845
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发表时间：
2012-09-01
影响因子：
2.6
通讯作者：
Xu, Wei
中科院分区：
文献类型：
--
作者：
Fan, Lei;Wang, Li;Xu, Wei
摘要目前还没有确定的危险因素可以预测里希特综合征（RS），临床诊断指标在慢性淋巴细胞白血病（CLL）中的应用价值也没有得到充分的证实。本研究的目的是提供中国CLL患者RS的证据和CLL转化为Richter综合征的危险因素。在149例中国患者中，自CLL诊断起的中位随访时间为43个月，16例（10.7%）患者进展为弥漫性大B细胞淋巴瘤（DLBCL）。根据相关分析，高水平的乳酸脱氢酶（LDH）和CD 38阳性被认为是转化为RS的独立预测因子。生存分析显示，RS、Binet分期、高LDH、高β2-微球蛋白、高TK、ZAP-70和CD 38表达、IGHV基因突变和del（17 p13）是影响总生存的不利因素。多变量考克斯回归分析显示，只有del（17 p13）与生存率强相关。转化后的中位OS为16个月（95%置信区间，5.6-26.4个月）。结果支持RS与不良结局相关，携带转化风险因素的患者需要密切监测和仔细活检。
Abstract No confirmed risk factors are known to predict Richter syndrome (RS), and the value of clinical prognosticators conventionally applied to chronic lymphocytic leukemia (CLL) is not firmly established in this setting. The objective of this study was to present evidence for RS in Chinese patients with CLL and risk factors for CLL transformation to Richter syndrome. With a median follow-up of 43 months from CLL diagnosis in 149 Chinese patients, 16 (10.7%) patients progressed to diffuse large B-cell lymphoma (DLBCL). According to correlation analysis, a high level of lactate dehydrogenase (LDH) and CD38 positivity were found to be independent predictors of transformation to RS. Survival analysis showed that presence of RS, advanced Binet stage, high level of LDH, high level of β2-microglobulin, high concentration of thymidine kinase (TK), ZAP-70 and CD38 expression, unmutated immunoglobulin heavy chain variable (IGHV) gene status and del(17p13) were adverse factors in determining overall survival (OS). Only del(17p13) was strongly associated with survival by multivariate Cox regression analysis. Median OS after transformation was 16 months (95% confidence interval, 5.6–26.4 months). The results support that RS is associated with a poor outcome, and a policy of close monitoring and careful biopsy is needed in patients carrying transformation risk factors.