Management of adults with T-cell lymphoblastic leukemia

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 similar to 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 gamma-secretase inhibitors, in adult patients with T-cell ALL. (Blood. 2017; 129(9): 1134-1142)