Department of Haematology, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom.
Oxford Molecular Diagnostic Centre, Oxford, United Kingdom.
Blood Rev. 2024 Mar;64:101163. doi: 10.1016/j.blre.2023.101163. Epub 2023 Dec 10.
Richter transformation (RT) represents an aggressive histological transformation from chronic lymphocytic leukaemia, most often to a large B cell lymphoma. It is characterised by chemo-resistance and subsequent short survival. Drug development has struggled over recent years in light of the aggressive kinetics of the disease, lack of pivotal registrational trials and relative rarity of the phenomenon. In this review we will highlight the diagnostic and therapeutic challenges of managing patients with RT as well as taking a look to the future therapeutic landscape. Highly active therapies developed across B cell malignancies are starting to impact this field, with T-cell activation therapies (CAR-T, bispecific antibodies), antibody-drug conjugates, and novel small molecule inhibitor combinations (e.g. BTKi-BCL2i) being actively studied. We will highlight the data supporting these developments and look to the studies to come to provide hope for patients suffering from this devastating disease.
里希特转化（RT）代表慢性淋巴细胞白血病的侵袭性组织学转化，最常见于大 B 细胞淋巴瘤。其特征为化疗耐药和随后的短期存活。近年来，由于该疾病的侵袭性动力学、缺乏关键注册试验和相对罕见的现象，药物开发一直面临挑战。在这篇综述中，我们将重点介绍管理 RT 患者的诊断和治疗挑战，并展望未来的治疗前景。在 B 细胞恶性肿瘤中开发的高度有效的治疗方法开始对这一领域产生影响，包括 T 细胞激活疗法（CAR-T、双特异性抗体）、抗体药物偶联物和新型小分子抑制剂联合治疗（如 BTKi-BCL2i）正在积极研究中。我们将重点介绍支持这些进展的数据，并期待即将开展的研究为患有这种毁灭性疾病的患者带来希望。
