Advances and updates in pediatric anaplastic large cell lymphoma Open Access

Anaplastic large cell lymphoma (ALCL) is a rare form of mature T-cell non-Hodgkin lymphoma. In pediatric patients, the majority of cases are Anaplastic Lymphoma Kinase (ALK)-positive. Despite intensive multi-agent chemotherapy regimens, treatment failure rates remain at 25-30%. Recent advancements in targeted therapies, notably ALK inhibitors and the anti-CD30 antibody-drug conjugate brentuximab vedotin, have demonstrated substantial activity in relapsed and refractory settings. Molecular detection of minimal disseminated disease (MDD) and minimal residual disease (MRD) offer improved prognostic stratification. For patients with relapsed or refractory disease, targeted therapies have increased treatment options, but more work needs to be done to define optimal treatment regimens, duration, and need for hematopoietic stem cell transplantation in this group. Immune therapies such as checkpoint inhibitors or Chimeric Antigen Receptor T-cell therapy provide additional therapeutic options. Incorporating targeted therapies and MDD/MRD assessments into clinical trials could significantly improve outcomes for pediatric and adolescent patients with ALCL.