An update on lymphoma in children and young adults

Abstract

Lymphoma accounts for approximately 10% of childhood cancers, but 17% in teenagers. It is divided into Hodgkin and non-Hodgkin lymphoma, with the vast majority of non-Hodgkin lymphoma in children being Burkitt lymphoma, lymphoblastic lymphoma and anaplastic large cell lymphoma. Lymphoma usually presents with painless lymphadenopathy, most commonly in the cervical region. ‘B’ symptoms are associated with more advanced disease. The diagnosis is usually made by histological examination of an excised lymph node, but can be made on pleural fluid or a bone marrow aspirate. Emergency management, particularly of the airway, may be necessary, especially in non-Hodgkin lymphoma. Lymphomas are treated with multi-drug chemotherapy regimens, stratified by risk group, with radiotherapy in selected cases. Rituximab (anti-CD20 monoclonal antibody) is used in conjunction with chemotherapy for poorer risk groups and in the relapse setting. In Hodgkin lymphoma, positron emission tomography scans are prognostic at assessment of response to therapy. The high cure rate for lymphomas (more than 90% for Hodgkin and Burkitt lymphoma) and the need to reduce late effects in children and adolescents has led to the current drive to tailor treatment according to risk group and response to initial therapy.