Adult Bone Marrow Transpant Unit, University Hospitals Bristol National Health Service Foundation Trust, Bristol, United Kingdom; and Department of Haematology, University Hospital of Wales, Cardiff, United Kingdom.
Blood. 2017 Mar 2;129(9):1134-1142. doi: 10.1182/blood-2016-07-692608. Epub 2017 Jan 23.
T-cell acute lymphoblastic leukemia (ALL) is a rare disease in adults with inferior survival outcomes compared with those seen in pediatric patients. Although potentially curable with ∼50% survival at 5 years, adult patients with relapsed disease have dismal outcomes with <10% of patients surviving long term. This review will discuss the diagnosis and management of adult patients with newly diagnosed T-cell ALL with an emphasis on the immunophenotypic and genetic analyses required to assign prognosis, risk stratify, and guide post-remission therapy. The evidence for the main components of complex T-cell ALL treatment regimens is described. The importance of monitoring minimal residual disease is emphasized, with a discussion of the different methods used. The results of hematopoietic cell transplantation are analyzed, and recommendations made about which patients should be considered for this intervention. The treatment of the adolescent and young adult group is delineated, and the role of using "pediatric-inspired" regimens in older adults considered. We also describe the current data and potential future options for the use of novel therapies, including nelarabine and γ-secretase inhibitors, in adult patients with T-cell ALL.
T 细胞急性淋巴细胞白血病（ALL）是一种罕见的成人疾病，与儿科患者相比，其生存结果较差。尽管成人患者有 50%的潜在治愈率，5 年生存率约为 50%，但复发患者的预后极差，长期存活的患者<10%。本综述将讨论新诊断为 T 细胞 ALL 的成人患者的诊断和治疗，重点是进行免疫表型和遗传学分析，以确定预后、风险分层和指导缓解后治疗。描述了复杂 T 细胞 ALL 治疗方案的主要成分的证据。强调了监测微小残留病的重要性，并讨论了使用的不同方法。分析了造血干细胞移植的结果，并就哪些患者应考虑进行该干预提出了建议。阐述了青少年和年轻成人组的治疗方法，并考虑了在老年患者中使用“儿科启发”方案的作用。我们还描述了新型疗法，包括那拉滨和γ-分泌酶抑制剂，在 T 细胞 ALL 成人患者中的应用的现有数据和潜在未来选择。
