Richter's transformation: Transforming the clinical landscape

IF 6.9 2区医学 Q1 HEMATOLOGY

Blood Reviews Pub Date : 2024-03-01 DOI:10.1016/j.blre.2023.101163

A. Barrett , N. Appleby , H. Dreau , C.P. Fox , T. Munir , T.A. Eyre

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Abstract

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.

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里克特的转变改变临床格局

里克特转化（RT）是慢性淋巴细胞白血病的一种侵袭性组织学转化，最常见的是向大 B 细胞淋巴瘤转化。其特点是对化疗产生抗药性，存活期短。鉴于该病的侵袭性、缺乏关键性注册试验以及相对罕见的现象，近年来的药物开发工作举步维艰。在本综述中，我们将重点介绍 RT 患者的诊断和治疗难题，并展望未来的治疗前景。针对 B 细胞恶性肿瘤开发的高活性疗法正开始对这一领域产生影响，T 细胞激活疗法（CAR-T、双特异性抗体）、抗体-药物共轭物和新型小分子抑制剂组合（如 BTKi-BCL2i）正得到积极研究。我们将重点介绍支持这些发展的数据，并期待未来的研究能为这种毁灭性疾病的患者带来希望。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Blood Reviews 医学-血液学

CiteScore

13.80

自引率

1.40%

发文量

期刊介绍： Blood Reviews, a highly regarded international journal, serves as a vital information hub, offering comprehensive evaluations of clinical practices and research insights from esteemed experts. Specially commissioned, peer-reviewed articles authored by leading researchers and practitioners ensure extensive global coverage across all sub-specialties of hematology.

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