Puła Bartosz, Salomon-Perzyński Aleksander, Prochorec-Sobieszek Monika, Jamroziak Krzysztof
Department of Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland,
Department of Diagnostic Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland.
Immunotargets Ther. 2019 Feb 5;8:1-14. doi: 10.2147/ITT.S167456. eCollection 2019.
Richter syndrome (RS) is recognized as the development of a secondary and aggressive lymphoma during the clinical course of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Most of such histological transformations are from RS to diffuse large B-cell lymphoma (DLBCL-RS, 90%) and Hodgkin's lymphoma (HL-RS, 10%). Histopathological examination is a prerequisite for diagnosis. It is crucial to assess the relationship between the RS clone and the underlying CLL/SLL because clonally related DLBCL-RS has a poor outcome, while clonally unrelated cases have a prognosis similar to de novo DLBCL. An anti-CD20 antibody-based immunochemotherapy is hitherto the frontline treatment of choice for DLBCL-RS; nonetheless, the results are unsatisfactory. Allogeneic stem cell transplantation should be offered to younger and fit patients as a consolidative treatment; however, the majority of the patients may not be qualified for this procedure. The HL-RS transformation has better outcomes than those of DLBCL-RS and can effectively be treated by the adriamycin, bleomycin, vinblastine, and dacarbazine regimen. Although novel agents are currently being investigated for RS, immunochemotherapy nevertheless remains a standard treatment for DLBCL-RS.
里氏综合征（RS）被认为是慢性淋巴细胞白血病/小淋巴细胞淋巴瘤（CLL/SLL）临床病程中继发的侵袭性淋巴瘤。大多数此类组织学转化是从RS转化为弥漫性大B细胞淋巴瘤（DLBCL-RS，90%）和霍奇金淋巴瘤（HL-RS，10%）。组织病理学检查是诊断的前提条件。评估RS克隆与潜在的CLL/SLL之间的关系至关重要，因为克隆相关的DLBCL-RS预后较差，而克隆无关的病例预后与原发性DLBCL相似。基于抗CD20抗体的免疫化疗是迄今为止DLBCL-RS的一线首选治疗方法；然而，结果并不令人满意。对于年轻且身体状况良好的患者，应提供异基因干细胞移植作为巩固治疗；然而，大多数患者可能不符合该治疗程序的条件。HL-RS转化的预后比DLBCL-RS更好，并且可以通过阿霉素、博来霉素、长春花碱和达卡巴嗪方案有效治疗。尽管目前正在对RS进行新型药物研究，但免疫化疗仍然是DLBCL-RS的标准治疗方法。
