Robertson L E, Pugh W, O'Brien S, Kantarjian H, Hirsch-Ginsberg C, Cork A, McLaughlin P, Cabanillas F, Keating M J
Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
J Clin Oncol. 1993 Oct;11(10):1985-9. doi: 10.1200/JCO.1993.11.10.1985.
The incidence, clinical features, laboratory findings, and treatment results of 39 patients with Richter's syndrome (RS) are reported.
Thirty-nine of 1,374 patients with chronic lymphocytic leukemia (CLL) developed RS.
Features associated with RS included systemic symptoms (59%), progressive lymphadenopathy (64%), extranodal involvement (41%), elevation of lactate dehydrogenase (LDH; 82%), and a monoclonal gammopathy (44%). Analysis of the CLL karyotype showed no specific chromosomal abnormality that conferred increased risk; however, multiple abnormalities were common. Patients at all Rai stages and in complete response (CR) were at risk, including three CR patients with no residual disease at the level of detection by dual-parameter flow cytometry or restriction analysis for immunoglobulin (Ig) gene rearrangements. The incidence was not higher in patients who had received prior fludarabine or chlorodeoxyadenosine. The median survival duration was only 5 months, despite multiagent therapy. Patients who responded had prolonged survival durations (P < .001). Three of eight patients who survived more than 1 year had a de novo presentation of both CLL and large-cell lymphoma (LCL). Comparison of surface light-chain analysis from both low- and high-grade components demonstrated isotypic light-chain expression in 12 of 15 patients. Ig heavy- and light-chain gene rearrangement analysis showed identical rearrangement patterns in five of five patients.
The clinical, laboratory, and survival characteristics of our RS patients were similar to those reported in earlier studies. Ig gene rearrangement and light-chain isotype analysis support a common origin for CLL and LCL. Despite progress in the treatment of CLL, the development of LCL remains a serious complication and continued surveillance in all CLL patients is warranted.
报告39例Richter综合征（RS）患者的发病率、临床特征、实验室检查结果及治疗效果。
1374例慢性淋巴细胞白血病（CLL）患者中有39例发生了RS。
与RS相关的特征包括全身症状（59%）、进行性淋巴结病（64%）、结外受累（41%）、乳酸脱氢酶（LDH）升高（82%）和单克隆丙种球蛋白病（44%）。对CLL核型的分析显示，没有特定的染色体异常会增加风险；然而，多种异常很常见。所有Rai分期及处于完全缓解（CR）的患者均有风险，包括3例通过双参数流式细胞术或免疫球蛋白（Ig）基因重排的限制性分析在检测水平无残留疾病的CR患者。既往接受过氟达拉滨或氯脱氧腺苷治疗的患者发病率并未更高。尽管采用了多药联合治疗，中位生存时间仅为5个月。有反应的患者生存时间延长（P <.001）。8例存活超过1年的患者中有3例初发时同时患有CLL和大细胞淋巴瘤（LCL）。对低级别和高级别成分的表面轻链分析比较显示，15例患者中有12例存在同型轻链表达。Ig重链和轻链基因重排分析显示，5例患者中有5例的重排模式相同。
我们的RS患者的临床、实验室及生存特征与早期研究报道的相似。Ig基因重排和轻链同型分析支持CLL和LCL起源相同。尽管CLL治疗取得了进展，但LCL的发生仍然是一种严重的并发症，所有CLL患者都需要持续监测。
