Tsimberidou Apostolia-Maria, O'Brien Susan, Khouri Issa, Giles Francis J, Kantarjian Hagop M, Champlin Richard, Wen Sijin, Do Kim-Anh, Smith Susan C, Lerner Susan, Freireich Emil J, Keating Michael J
Department of Leukemia, Blood and Marrow Transplantation, The University of Texas M.D. Anderson Cancer Center, Houston, TX 77030, USA.
J Clin Oncol. 2006 May 20;24(15):2343-51. doi: 10.1200/JCO.2005.05.0187.
The purpose of this study was to assess the incidence, presenting characteristics, and treatment outcomes of Richter's syndrome (RS) and factors predicting response and survival.
An electronic database search of patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) who presented at The University of Texas M.D. Anderson Cancer Center (Houston, TX) between January 1975 and June 2005 was performed, and patient medical records were reviewed.
Of the 3,986 patients with CLL/SLL, 204 patients (5.1%) had possible RS, and 148 patients (3.7%) had biopsy- or fine-needle aspiration-proven RS. Treatment included chemotherapy alone and chemoimmunotherapy with rituximab. The overall response rate for the 130 assessable patients was 39% (chemotherapy, 34%; chemoimmunotherapy, 47%; P = .2). In multivariate analysis, factors predicting prolonged survival were Zubrod performance status 0-1 (P = .006), lactate dehydrogenase < or = 1.5x the upper limit of normal (P = .003), platelet count > or = 100,000 (P = .01), tumor size < or = 5 cm (P = .02), and fewer than two prior therapies (P = .02). The five adverse factors predicting shorter survival were used to design a model to predict an individual patient's risk of death: the RS score. A total of 20 patients underwent stem-cell transplantation (SCT). Patients who underwent allogeneic SCT as postremission therapy had longer survival than patients who achieved remission and received no additional therapy or patients who underwent allogeneic or autologous SCT as salvage therapy (P = .019).
A score to predict an individual patient's risk of death is proposed. Chemotherapy and rituximab combinations are effective in RS. Patients with available donors may be considered for allogeneic SCT as postremission therapy.
本研究旨在评估 Richter 综合征（RS）的发病率、临床表现特征、治疗结果以及预测反应和生存的因素。
对 1975 年 1 月至 2005 年 6 月在德克萨斯大学 MD 安德森癌症中心（休斯顿，德克萨斯州）就诊的慢性淋巴细胞白血病（CLL）/小淋巴细胞淋巴瘤（SLL）患者进行电子数据库检索，并查阅患者病历。
在 3986 例 CLL/SLL 患者中，204 例（5.1%）可能患有 RS，148 例（3.7%）经活检或细针穿刺证实患有 RS。治疗包括单纯化疗和联合利妥昔单抗的化疗免疫治疗。130 例可评估患者的总体缓解率为 39%（化疗组为 34%；化疗免疫治疗组为 47%；P = 0.2）。多因素分析显示，预测生存期延长的因素包括 Zubrod 体能状态 0 - 1（P = 0.006）、乳酸脱氢酶≤正常上限的 1.5 倍（P = 0.003）、血小板计数≥100,000（P = 0.01）、肿瘤大小≤5 cm（P = 0.02）以及既往治疗次数少于两次（P = 0.02）。利用五个预测生存期较短的不良因素设计了一个模型来预测个体患者的死亡风险：RS 评分。共有 20 例患者接受了干细胞移植（SCT）。作为缓解后治疗接受异基因 SCT 的患者比达到缓解但未接受额外治疗的患者或作为挽救治疗接受异基因或自体 SCT 的患者生存期更长（P = 0.019）。
提出了一个预测个体患者死亡风险的评分系统。化疗与利妥昔单抗联合治疗对 RS 有效。有合适供体的患者可考虑在缓解后进行异基因 SCT 治疗。
