Croizier Carolyne, Guièze Romain
Service d'Hématologie Clinique et de Thérapie Cellulaire, CHU Estaing, 1, place Lucie et Raymond Aubrac, 63000 Clermont-Ferrand, France.
Service d'Hématologie Clinique et de Thérapie Cellulaire, CHU Estaing, 1, place Lucie et Raymond Aubrac, 63000 Clermont-Ferrand, France; EA 7453, CHELTER, Université Clermont Auvergne, CHU Estaing, Hématologie Biologique, 1, place Lucie et Raymond Aubrac, 63003 Clermont-Ferrand, France.
Bull Cancer. 2021 May;108(5):521-527. doi: 10.1016/j.bulcan.2021.01.014. Epub 2021 Apr 23.
Richter syndrome (RS) is defined as the occurrence of an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL) and rarely Hodgkin lymphoma (HL), in a patient with prior or concomitant chronic lymphocytic leukemia (CLL). RS is estimated to occur in 0.5-1 % per year and is associated with adverse outcome. In the vast majority of patients (80 %), RS is clonally related to the prior CLL. Those with unrelated RS appear to have better outcome. The therapeutic approach is based on those of de novo DLBCL or HL. However, even with modern immunochemotherapy regimens, response rate remains low. In eligible patients with related RS, a consolidation by autologous or allogeneic stem-cell transplantation must be proposed. Combinations including therapies targeting BCR or BCL2 and effective in CLL are currently being evaluated in RS. Novels immunotherapies could be promising approaches based on preliminary results.
里氏综合征（RS）定义为既往患有或同时患有慢性淋巴细胞白血病（CLL）的患者发生侵袭性淋巴瘤，最常见的是弥漫性大B细胞淋巴瘤（DLBCL），很少见霍奇金淋巴瘤（HL）。据估计，RS的年发生率为0.5%-1%，且与不良预后相关。在绝大多数患者（80%）中，RS与既往的CLL存在克隆相关性。那些RS与CLL无关的患者似乎预后较好。治疗方法基于原发性DLBCL或HL的治疗方法。然而，即使采用现代免疫化疗方案，缓解率仍然较低。对于符合条件的相关RS患者，必须建议进行自体或异基因干细胞移植巩固治疗。目前正在RS中评估包括靶向BCR或BCL2且对CLL有效的疗法在内的联合治疗方案。基于初步结果，新型免疫疗法可能是有前景的治疗方法。
