Treatment of Richter Transformation of Chronic Lymphocytic Leukemia in the Modern Era.
Treatment of Richter Transformation of Chronic Lymphocytic Leukemia in the Modern Era.
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里希特变换(RT)虽然罕见，但却是慢性淋巴细胞白血病的严重并发症。RT的死亡率很高，代表着一种未得到满足的临床需求。随着对其分子生物学认识的提高，新的靶向治疗和免疫治疗有望改善这种疾病的结果。这篇综述概述了关于RT的已知情况以及该领域的发展方向。还包括一个说明性的案例，以方便读者理解所提供的材料。Richter变换(RT)是指在慢性淋巴细胞白血病(CLL)的背景下发展为侵袭性淋巴瘤。虽然RT有许多变种，但弥漫性大B细胞淋巴瘤(RT-DLBCL)是最常见的(80%)，其次是霍奇金淋巴瘤(RT-HL，19%)。诊断基于临床可疑淋巴结的组织学评估。正电子发射断层扫描(PET)可用于选择感兴趣的结节进行活检。虽然克隆性检测不是RT诊断的先决条件，但它对生存有重要意义。克隆性DLBCL预后最差，在使用或不使用抗CD20抗体的联合化疗时代，中位总生存期(OS)不到一年。随着干细胞移植以及靶向治疗和新形式免疫治疗等新药物的使用，预后有所改善。鼓励考虑进行临床试验。本文介绍了我们目前对RT的认识，重点介绍了RT-DLBCL的治疗，包括正在进行的临床试验和正在开发的新疗法。我们还报告了一个与克隆性相关的DLBCL患者在诊断后存活两年而没有使用联合化疗的说明性例子。
Though rare, Richter Transformation (RT) is a serious complication of chronic lymphocytic leukemia. RT carries a high mortality rate and represents an unmet clinical need. With an improved understanding of its molecular biology, there is hope that novel targeted therapies and immunotherapies will lead to improved outcomes in this disease. This review outlines what is known about RT as well as where the field is headed. An illustrative case is also included to facilitate the reader’s understanding of the material presented. Richter Transformation (RT) refers to the development of an aggressive lymphoma in the setting of chronic lymphocytic leukemia (CLL). While many variants of RT are recognized, diffuse large B-cell lymphoma (RT-DLBCL) is the most common (80%), followed by Hodgkin’s lymphoma (RT-HL, 19%). Diagnosis is based upon histologic evaluation of clinically suspicious lymph nodes. Positron emission tomography (PET) may be used to select the node of interest for biopsy. Although clonality testing is not a prerequisite of RT diagnosis, it has significant implications for survival. Clonally related DLBCL carries the worst prognosis with a median overall survival (OS) of less than one year in the era of combination chemotherapies with or without anti-CD20 antibodies. Prognosis has improved with the use of stem cell transplant and newer agents such as targeted therapy and newer forms of immunotherapy. Consideration of a clinical trial is encouraged. This review describes our current understanding of RT and focuses on treatment of RT-DLBCL, including clinical trials in progress and new therapies in development. We also report an illustrative example of a patient with clonally related DLBCL who survived two years after diagnosis without the use of combination chemotherapy.
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DOI：
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