Risk factors and treatment of childhood and adolescent Burkitt lymphoma/leukaemia

Abstract

SummaryBurkitt lymphoma/leukaemia is the most common (40%) form of non-Hodgkin lymphoma that occurs in children and adolescents. The prognosis of advanced (disseminated) Burkitt lymphoma/leukaemia in children and adolescents three decades ago had a 5-year event-free survival (EFS) of <40%, and required combination chemotherapy and radiation therapy over a 1-2 year period. Currently, the prognosis for the same advanced stage has a 5-year EFS of 85-90% with <6 months of chemotherapy only. Radiation therapy has been eliminated for children and adolescents with Burkitt lymphoma/leukaemia except in emergencies, such as superior vena cava syndrome and acute neurological impairment or in patients with relapse/progression. Current risk factors in the prognosis of childhood and adolescent Burkitt lymphoma/leukaemia include: lactate dehydrogenase level 29 the upper normal limit at diagnosis, bone marrow and central nervous system involvement, poor response to cyclophosphamide, vincristine and prednisone reduction therapy and poor risk cytogenetics. New and novel therapeutic approaches include monoclonal antibody (anti-CD20) therapy, targeted cellular immune therapy and small molecule inhibitors. Future strategies should include improved staging and risk classification, reduction of cytotoxic chemotherapy, the investigation of targeted therapy, an increased understanding of the underlying biology of Burkitt lymphoma/leukaemia, strategies for prevention and approaches to reduce acute and chronic toxicities.

Targeting CD20+ Aggressive B-cell Non–Hodgkin Lymphoma by Anti-CD20 CAR mRNA-Modified Expanded Natural Killer Cells In Vitro and in NSG Mice

“…We demonstrated in an international multi-cooperative group study, which comprised the Children's Oncology Group (COG), The Soci et e Française d'Oncologie P ediatrique (SFOP), and the United Kingdom Children's Cancer Study Group (UKCCSG), that children and adolescents with newly diagnosed mature B-NHL [French-American-British (FAB)/Lymphome malins de Burkitt (LMB) 96], have a 90% 5-year overall survival (OS) following treatment with a short, intensive course of chemotherapy (4). Unfortunately, for children and adolescents who relapse or progress with de novo mature B-NHL, the prognosis is dismal due to chemoradiotherapy resistance (4,5). Similarly, the prognosis in adults with recurrent/ refractory Burkitt lymphoma is dismal (6).…”

Oculomotor nerve palsy as a preceding symptom of adult sporadic Burkitt lymphoma: A case report and review of the literature

“…As IgEEs are specifically active in mature B cells, this translocation to MYC results in aberrantly high MYC expression, which enhances B cell proliferation regardless of whether EBV infection has occurred (10). Other chromosomal aberrations may also induce rapid cell proliferation, which results in a highly aggressive clinical course (11).…”

A Case Report of a Metastatic Primary Gastric Lymphoma; Diffuse Large B-Cell or Burkitt Lymphoma?

“…Tumor lysis syndrome (TLS) may happen in the patients with hematological cancers, especially in tumors with high proliferation rates like BL, spontaneously or while receiving chemotherapy agents. Therefore, one of the first steps in management of BL is prevention of TLS by administration of medications such as allopurinol or rasburicase [14]. For prevention of wrong diagnosis, we have to consider the most appropriate approach.…”