2022 Volume 59 Issue 1 Pages 12-18
T-cell acute lymphoblastic leukemia (T-ALL), which affects 10–15% of pediatric ALL patients and approximately 25% of adult ALL patients, has clinical characteristics and genetic alterations that are distinct from those in B-cell precursor ALL (BCP-ALL). Improvements have been achieved through treatment intensification strategies using drugs, such as dexamethasone, L-asparaginase, and nelarabine, in addressing the issue of cranial radiotherapy omission. Furthermore, for adolescent and young adult (AYA) patients with T-ALL, pediatric-inspired regimens have been adopted, leading to improved outcomes. Regarding prognostic factors in pediatric T-ALL, age and white blood cell count at diagnosis have been found to have little prognostic value compared with the case of BCP-ALL. Moreover, minimal residual disease (MRD), rather than genetic alterations, has been recognized as the most reliable indicator. Therefore, recent clinical trials have been designed to use MRD-directed treatments. Additionally, new recurrent fusions involving SPI1 have been recently identified to be associated with poor outcomes. Progress in the knowledge of tumor biology will certainly lead to the development and use of new targeted therapies, such as the recombinant monoclonal antihuman CD38 antibody daratumumab, the Janus kinase inhibitor ruxolitinib, and dasatinib, potentially improving the outcomes of pediatric and AYA patients with T-ALL.
