Pediatric Aggressive Mature B-Cell Lymphomas, Version 2.2020, NCCN Clinical Practice Guidelines in Oncology

Abstract

Pediatric aggressive mature B-cell lymphomas are the most common types of non-Hodgkin lymphoma in children, and they include Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL). These diseases are highly aggressive but curable, the treatment is complex, and patients may have many complicated supportive care issues. The NCCN Guidelines for Pediatric Aggressive Mature B-Cell Lymphomas provide guidance regarding pathology and diagnosis, staging, initial treatment, disease reassessment, surveillance, therapy for relapsed/refractory disease, and supportive care for clinicians who treat sporadic pediatric BL and DLBCL.

Clinical characteristics and treatment outcomes of children and adolescents with aggressive mature B-cell lymphoma: a single-center analysis

“…The prognosis of pediatric mature B-cell lymphoma has substantially improved over the decades, with good long-term survival. In 2020, the National Comprehensive Cancer Network (NCCN) guidelines for pediatric aggressive mature B-cell lymphomas provided guidance on the pathology and diagnosis, staging, initial treatment, and therapy for relapsed or refractory disease [ 1 ]. Our retrospective study reviewed the clinical characteristics of patients, outcomes of various protocols, and treatments of relapsed patients with mature B-cell lymphoma over 28 years at a single institute.…”

“…They reported markedly improved 3-year EFS and OS rates of >93% in the R-LMB group. On the basis of these reports, the NCCN guideline recommended rituximab-combined chemotherapy in high-risk patients classified as groups B and C [ 1 ]. In our study, 2 patients underwent therapy with the combination of rituximab and the LMB96 protocol, both of whom showed good treatment outcomes without treatment-related complications.…”

“…Children generally present with high-grade lymphomas that tend to be aggressive and fast growing, such as Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), lymphoblastic lymphoma, and anaplastic large cell lymphoma. Among them, aggressive mature B-cell lymphomas account for approximately 60% of NHLs in children [ 1 ]. They include many subtypes and variants, such as DLBCL, BL, and high-grade B-cell lymphoma, not otherwise specified (HGBL-NOS) [ 2 ].…”

“…Increased serum LDH levels and CNS involvement are considered factors associated with a poor prognosis [ 10 ]. For these groups of patients, multiple strategies have been proposed and new therapies are being investigated [ 1 ].…”

“…For these groups of patients, multiple strategies have been proposed and new therapies are being investigated [1].…”