title: Mature B-cell lymphoma and leukemia in children and adolescents-review of standard chemotherapy regimen and perspectives.
abstract: Mature B-cell non-Hodgkin lymphoma (B-NHL) comprises more than 50% of all non-Hodgkin lymphoma (NHL) in children and adolescents. Many B-NHL subtypes frequently observed in adults are rarely diagnosed in children and adolescents. In this age group, Burkitt lymphoma (BL), Burkitt leukemia or FAB L3 leukemia (B-AL), diffuse large B-cell lymphoma (DLBCL), primary mediastinal large B-cell lymphoma (PMLBL), follicular lymphoma (FL), and aggressive mature B-NHL not further classifiable (B-NHL nfc) are the most common subtypes. Diverse clinical trials demonstrated similar results of current combination chemotherapy regimens succeeding in overall survival rates of more than 80%. However, treatment-related toxicity and the poor prognosis of relapse are serious concerns. Furthermore, specific histological B-NHL subtypes are rare in children and optimal treatment is not established. New treatment modalities are urgently needed for these patient groups. Rituximab, a monoclonal antibody that is already established in the treatment of adults with mature B-NHL, demonstrated promising results in pediatric patients. The definitive role of rituximab in the treatment of children and adolescents with B-NHL needs to be evaluated in prospective controlled clinical trials. This review provides a comprehensive overview of chemotherapy regimens and the perspectives for children and adolescents with mature B-cell lymphoma and leukemia.
authors.affiliation: Department of Pediatric Hematology and Oncology, NHL-BFM Study Center, University Children's Hospital, Münster, Germany.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Worch
authors.forename: Marius
authors.initials: M
authors.lastname: Rohde
authors.forename: Birgit
authors.initials: B
authors.lastname: Burkhardt
mesh_terms: D000293:Adolescent; D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000592:standards* / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D015448:Leukemia, B-Cell / Q000145:classification / Q000188:drug therapy* / Q000401:mortality; D016393:Lymphoma, B-Cell / Q000145:classification / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D011446:Prospective Studies; D000069283:Rituximab; D015996:Survival Rate
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab