Diffuse large B-cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients.
Diffuse large B-cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients.
复制标题
DOI：
10.1111/bjh.12458
复制
发表时间：
2013-09
影响因子：
6.5
通讯作者：
Shanafelt TD
中科院分区：
文献类型：
--
作者：
Parikh SA;Rabe KG;Call TG;Zent CS;Habermann TM;Ding W;Leis JF;Schwager SM;Hanson CA;Macon WR;Kay NE;Slager SL;Shanafelt TD
几乎所有关于发生弥漫性大B细胞淋巴瘤（Richter综合征[RS]）的慢性淋巴细胞白血病（CLL）患者的信息都来自回顾性病例系列或临床试验中的患者。我们使用梅奥诊所CLL数据库来确定新诊断的CLL患者（2000年1月 - 2011年7月）。确定了在随访期间经活检证实发生RS的个体。经过中位4年的随访，1641例CLL患者中有37例（2.3%）发生RS。RS的发生率约为每年0.5%。RS的风险与诊断时的Rai分期晚期（p<0.001）、高危荧光原位杂交（FISH）（p<0.0001）、未突变的免疫球蛋白重链可变区（IGHV）（p = 0.003）以及ZAP - 70（p = 0.02）和CD38（p = 0.001）的表达有关。接受CLL治疗的患者中RS的发生率翻倍（每年1%）。定型B细胞受体（优势比 = 4.2；p = 0.01），但VH4 - 39与之无关，与RS风险增加有关。嘌呤类似物和烷化剂联合治疗使RS的风险增加3倍（优势比 = 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，中位生存期分别为0.5年、2.1年和未达到。CLL克隆的潜在特征和后续的CLL治疗都会影响RS的风险。RS后的生存期仍然较差，需要新的治疗方法。
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 (1/2000–7/2011). Individuals who developed biopsy-proven RS during follow-up were identified. After 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 FISH (p<0.0001), unmutated IGHV (p=0.003), and expression of ZAP-70 (p=0.02) and CD38 (p=0.001). The rate of RS doubled in patients treated for CLL (1%/year). Stereotyped B-cell receptors (odds-ratio=4.2; p=0.01) but not VH4–39 was associated with increased risk of RS. Treatment with combination of purine analogues and alkylating agents increased the risk of RS 3-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.
登录
查看更多内容
影响因子：
20.3
作者：
Mauro, FR;Foa, R;Mandelli, F
通讯作者：
Mandelli, F
影响因子：
20.3
作者：
Tam, Constantine S.;O'Brien, Susan;Keating, Michael J.
通讯作者：
Keating, Michael J.
影响因子：
168.9
作者：
Hallek, M.;Fischer, K.;Stilgenbauer, S.
通讯作者：
Stilgenbauer, S.
影响因子：
45.3
作者：
Hillmen, Peter;Skotnicki, Aleksander B.;Mayer, Jiri
通讯作者：
Mayer, Jiri
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
影响因子：
2.6
作者：
Fan, Lei;Wang, Li;Xu, Wei
通讯作者：
Xu, Wei