Expert Review Redefines ETP-ALL Management with MRD-Guided Risk Stratification
This clinical review addresses the management of early T-cell precursor ALL in children, a subtype initially associated with very poor prognosis. Contemporary regimens have largely closed the survival gap with non-ETP T-ALL, shifting focus to MRD-based risk stratification for transplant decisions. The authors discuss the interplay between immunophenotype and genomic markers of immaturity, and position MRD response as the key determinant of treatment intensification.
The original study
How I treat ETP-ALL in children.
- Authors
- Summers RJ, Teachey DT, Hunger SP
- Journal
- Blood
- Type
- Journal Article, Case Reports, Review
- PMID
- 38364183
Original abstract
Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a unique subtype of immature T-cell ALL that was initially associated with a dramatically inferior prognosis compared with non-ETP T-cell ALL (Not-ETP) when it was first described in 2009. Analyses of larger patient cohorts treated with more contemporary regimens, however, have shown minimal survival differences between ETP and Not-ETP. In this manuscript, we use representative cases to explore therapeutic advances and address common clinical questions regarding the management of children, adolescents, and young adults with ETP-ALL. We describe our recommended treatment approach for a child or adolescent with newly diagnosed ETP-ALL, with an emphasis on the prognostic significance of induction failure and detectable minimal residual disease and the role of hematopoietic stem cell transplant in first remission. We discuss the interplay between the ETP immunophenotype and genomic markers of immaturity in T-cell ALL. Finally, we review novel therapeutic approaches that should be considered when managing relapsed or refractory ETP-ALL.