T细胞淋巴母细胞瘤（T-LLy）和T细胞急性淋巴细胞白血病（T-ALL）一直被认为是同一种疾病的谱系。然而，最近的证据表明，对化疗的不同反应可能表明T-LLy和T-ALL是不同的临床和生物学实体。在这里，我们检查了这两种疾病之间的差异，并使用具体案例来强调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，包括使用奈拉巴林和硼替佐米，诱导类固醇的选择，颅脑放疗的作用，以及危险分层标志物来识别复发风险最高的患者并进一步完善当前的治疗策略。因为复发或难治性T-LLy患者的预后较差，我们讨论正在进行的研究将新型疗法，包括免疫治疗，纳入前期和挽救方案，并探讨造血干细胞移植的作用。版权所有© 2023美国血液学会。

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 two diseases and use illustrative cases to highlight key recommendations on how to best treat newly diagnosed and relapsed/refractory T-LLy patients. 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.Copyright © 2023 American Society of Hematology.