Department of Pediatrics, University of Hawai'i, Honolulu, HI.
Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE.
Blood. 2023 Jun 22;141(25):3019-3030. doi: 10.1182/blood.2022016503.
T-cell lymphoblastic lymphoma (T-LLy) and T-cell acute lymphoblastic leukemia (T-ALL) have historically been considered a spectrum of the same disease. However, recent evidence demonstrating differential responses to chemotherapy raise the possibility that T-LLy and T-ALL are distinct clinical and biologic entities. Here, we examine differences between the 2 diseases and use illustrative cases to highlight key recommendations on how to best treat patients with newly diagnosed and relapsed/refractory T-LLy. We discuss results of recent clinical trials incorporating use of nelarabine and bortezomib, choice of induction steroid, role of cranial radiotherapy, and risk stratification markers to identify patients at highest risk of relapse and to further refine current treatment strategies. Because prognosis for relapsed or refractory T-LLy patients is poor, we discuss ongoing investigations incorporating novel therapies, including immunotherapeutics, into upfront and salvage regimens and the role of hematopoietic stem cell transplantation.
T 细胞淋巴母细胞淋巴瘤（T-LLy）和 T 细胞急性淋巴细胞白血病（T-ALL）在历史上被认为是同一种疾病的不同表现。然而，最近的证据表明对化疗有不同的反应，这增加了 T-LLy 和 T-ALL 是不同的临床和生物学实体的可能性。在这里，我们检查了这两种疾病之间的差异，并使用实例来说明如何最好地治疗新诊断和复发/难治性 T-LLy 患者的关键建议。我们讨论了最近的临床试验结果，这些试验纳入了使用奈拉滨和硼替佐米、诱导类固醇的选择、颅放疗的作用以及风险分层标志物，以确定复发风险最高的患者，并进一步完善当前的治疗策略。由于复发或难治性 T-LLy 患者的预后较差，我们讨论了正在进行的研究，将新型疗法，包括免疫疗法，纳入一线和挽救治疗方案以及造血干细胞移植的作用。
