Diagnosis and management of mature B-cell lymphomas in children, adolescents, and young adults

In resource rich countries mature B-cell lymphoma has an expected cure rate of greater than 90% making it one of the most highly curable malignancies in paediatric adolescent and young adults. Mature B-cell lymphoma incorporates 3 major histological subtypes including Burkitt lymphoma/leukaemia (BLL), diffuse large B-cell lymphoma (DLBCL), and primary mediastinal B-cell lymphoma (PMBL). The chemotherapy for these diseases is short and intense including central nervous system (CNS) prophylaxis with recent data indicating that incorporation of CD20 directed rituximab antibody to advanced mature B-cell lymphoma has “boosted” the event free survival (EFS) by approximately 10–12% into the range of more localized patients. While cure is the primary goal short- and long-term toxicities of therapy are substantial and ongoing efforts should continue to optimize therapy using logical incorporation of immune therapy and reduction (and perhaps elimination) of some cytotoxic chemotherapy.

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