Tsimberidou Apostolia-Maria, Keating Michael J
Department of Leukemia, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA.
Semin Oncol. 2006 Apr;33(2):250-6. doi: 10.1053/j.seminoncol.2006.01.016.
Richter's syndrome (RS) is characterized by the development of high-grade non-Hodgkin's lymphoma (NHL) in a patient with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma. At The University of Texas M.D. Anderson Cancer Center the incidence of RS is 3.9%. The large cells of RS may arise through transformation of the original CLL clone or represent a new neoplasm. RS may be triggered by viral infections, such as Epstein-Barr virus (EBV). Trisomy 12 and chromosome 11 abnormalities, as well as multiple genetic defects, have been described in patients with RS. These abnormalities may cause CLL cells to proliferate and, by facilitating the acquisition of new genetic abnormalities, to transform into RS cells. The therapeutic strategies for RS typically include therapies developed for NHL or acute lymphoblastic leukemia. The reported response rates with these therapies are 5% to 43%, and the median survival duration ranges from 5 to 8 months. The median overall survival duration at our institution of patients with RS is 9.1 months (95% confidence interval, 7.8 to 11 months), and the median failure-free survival duration is 7.1 months (95% confidence interval, 5.1 to 10.4 months). Patients appear to benefit from cytoreductive therapy consisting of chemotherapy and immunotherapy, followed by allogeneic stem cell transplantation, as postremission therapy. As part of a program aiming to cure RS, we are currently conducting a clinical trial of oxaliplatin, fludarabine, and cytarabine in combination with rituximab and recommend postremission therapy, including allogeneic stem cell transplantation in patients with available donors.
里氏综合征（RS）的特征是慢性淋巴细胞白血病（CLL）或小淋巴细胞淋巴瘤患者发生高级别非霍奇金淋巴瘤（NHL）。在德克萨斯大学MD安德森癌症中心，RS的发病率为3.9%。RS的大细胞可能源于原始CLL克隆的转化，或者代表一种新的肿瘤。RS可能由病毒感染引发，如爱泼斯坦-巴尔病毒（EBV）。12号染色体三体和11号染色体异常以及多种基因缺陷在RS患者中已有描述。这些异常可能导致CLL细胞增殖，并通过促进获得新的基因异常而转化为RS细胞。RS的治疗策略通常包括为NHL或急性淋巴细胞白血病开发的疗法。报道的这些疗法的缓解率为5%至43%，中位生存期为5至8个月。在我们机构，RS患者的中位总生存期为9.1个月（95%置信区间，7.8至11个月），中位无失败生存期为7.1个月（95%置信区间，5.1至10.4个月）。患者似乎从由化疗和免疫疗法组成的减瘤治疗中获益，随后进行异基因干细胞移植作为缓解后治疗。作为旨在治愈RS的项目的一部分，我们目前正在进行一项奥沙利铂、氟达拉滨和阿糖胞苷联合利妥昔单抗的临床试验，并建议进行缓解后治疗，包括为有合适供体的患者进行异基因干细胞移植。
