T-Cell Acute Lymphoblastic Leukemia-Current Concepts in Molecular Biology and Management

T-cell acute lymphoblastic leukemia (T-ALL) is an uncommon, yet aggressive leukemia that accounts for approximately one-fourth of acute lymphoblastic leukemia (ALL) cases. CDKN2A/CDKN2B and NOTCH1 are the most common mutated genes in T-ALL. Children and young adults are treated with pediatric intensive regimens and have superior outcomes compared to older adults. In children and young adults, Nelarabine added to frontline chemotherapy improves outcomes and end of consolidation measurable residual disease has emerged as the most valuable prognostic marker. While outcomes for de-novo disease are steadily improving, patients with relapsed and refractory T-ALL fare poorly. Newer targeted therapies are being studied in large clinical trials and have the potential to further improve outcomes. The role of allogeneic stem cell transplant (HSCT) is evolving due to the increased use of pediatric-inspired regimens and MRD monitoring. In this review we will discuss the biology, treatment, and outcomes in pediatric and adult T-ALL.

Automated summary

T-cell acute lymphoblastic leukemia (T-ALL) is a rare but aggressive leukemia with CDKN2A/CDKN2B and NOTCH1 being the most common mutated genes. While outcomes for de-novo disease are improving, patients with relapsed and refractory T-ALL have poor prognosis. New targeted therapies show potential to further improve outcomes, and the role of allogeneic stem cell transplant is evolving with the increased use of pediatric-inspired regimens.