Clinical and biological aspects of aggressive B-cell non-Hodgkin lymphoma in adolescents and young adults

Abstract

Non-Hodgkin lymphomas (NHLs) are one of the most frequent malignancies in adolescents and young adults (AYA). Among NHLs, Burkitt's lymphoma (BL) represents approximately 40% while diffuse large B-cell lymphoma (DLBCL) accounts for nearly 20% of cases. Primary mediastinal B-cell lymphoma is a variant of DLBCL, which preferentially concerns young patients. Biology of B-NHLs is well known and several pathways involving chromosomal translocations, gene rearrangements, and molecular profiling are the subject of continuous investigations. AYA with B-NHL have inferior survival when compared with children. The reasons for this unfavorable outcome are multifactorial, but disease-related biological characteristics of the tumor represent a powerful factor influencing survival. The choice of optimal strategy in the management of B-NHL in patients of 15-29 years old remains controversial and depends on the treating institution and its physicians. Although children and younger adolescents benefit from pediatric approaches using intensive treatment, older adolescents are often treated with adult rituximab-based chemotherapy. In this review, we focus on the current knowledge relevant to AYA with DLBCL and primary mediastinal B-cell lymphoma.