Purpose of Review

The treatment paradigm of chronic lymphocytic leukemia (CLL) has dramatically changed with the advent of novel targeted agents over the past decade. Richter transformation (RT), or the development of an aggressive lymphoma from a background of CLL, is a well-recognized complication of CLL and carries significantly poor clinical outcomes. Here, we provide an update on current diagnostics, prognostication, and contemporary treatment of RT.

Recent Findings

Several genetic, biologic, and laboratory markers have been proposed as candidate risk factors for the development of RT. Although a diagnosis of RT is typically suspected based on clinical and laboratory findings, tissue biopsy is essential for histopathologic confirmation of diagnosis. The standard of care for RT treatment at this time remains chemoimmunotherapy with the goal of proceeding to allogeneic stem cell transplantation in eligible patients. Several newer treatment modalities are being studied for use in the management of RT, including small molecules, immunotherapy, bispecific antibodies, and chimeric antigen receptor T-cell (CAR-T) therapy.

Summary

The management of patients with RT remains a challenge. Ongoing trials show enormous promise for newer classes of therapy in RT, with the hope being that these agents can synergize, and perhaps supersede, the current standard of care in the near future.

中文翻译：

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慢性淋巴细胞白血病的里氏转化——我们正在取得进展吗？

审查目的

过去十年，随着新型靶向药物的出现，慢性淋巴细胞白血病（CLL）的治疗模式发生了巨大变化。里氏转化 (RT)，即 CLL 背景下发展为侵袭性淋巴瘤，是一种公认​​的 CLL 并发症，临床结果显着较差。在此，我们提供有关 RT 的当前诊断、预测和当代治疗的最新信息。

最近的发现

一些遗传、生物和实验室标记已被提议作为 RT 发展的候选危险因素。尽管通常根据临床和实验室检查结果怀疑 RT 的诊断，但组织活检对于组织病理学确诊至关重要。目前 RT 治疗的护理标准仍然是化学免疫疗法，目标是对符合条件的患者进行同种异体干细胞移植。正在研究几种用于 RT 管理的新治疗方式，包括小分子、免疫疗法、双特异性抗体和嵌合抗原受体 T 细胞 (CAR-T) 疗法。

概括

放疗患者的管理仍然是一个挑战。正在进行的试验显示出新型放疗疗法的巨大前景，希望这些药物能够在不久的将来协同作用，甚至可能取代当前的护理标准。