Haema 2017; 8(1): 54-63
by Vasilios Papadakis1, Vasilios Dendrinos1, Kalliopi Stefanaki2
1Department of Pediatric Hematology-Oncology, Oncology Unit Marianna V. Vardinogiannis – ELPIDA “Aghia Sofia” Children’s Hospital Goudi, Athens, Greece,
2Haemopathological Laboratory, “Aghia Sofia” Children’s Hospital, Athens, Greece
Abstract
Burkitt lymphoma is a rapidly progressing lymphoproliferative disorder, that mostly affects children and young adults, with lymph node but mostly other organ involvement (jaw, GI tract, kidneys, CNS, bone marrow). Endemic (African) Burkitt lymphoma (eBL) most commonly involves the jaw and facial bone (orbit) (>50% of cases). Sporadic Burkitt lymphoma (sBL) most often presents with abdominal tumors with bone marrow involvement. Immunodeficiency-related Burkitt lymphoma cases (HIV related or other) usually present with nodal involvement with frequent bone marrow infiltration. The World Health Organization (WHO) classification of lymphoid neoplasms identifies Burkitt lymphoma and Burkitt leukemia (>25% involvement, L3 ALL) as two different manifestations of the same disease based on genetic and immunohistochemical similarity and treatment is identical. Burkitt lymphoma can be Epstein-Barr virus related, especially in its endemic/African form. Usually, C-MYC oncogene (on chromosome 8) dysregulation and mutation is found, resulting in translocations with the immunoglobulin heavy and light (κ or λ chain) genes on chromosomes 14, 2 and 22, respectively [t(8;14), t(2;8) and t(8;22)]. Typical morphology and immunophenotyping of the cells establish the diagnosis, even in the rare occasion where typical translocations are not found. Burkitt lymphoma patients are treated according to prognostic treatment groups, based on stage, age, and biological parameters (i.e. diagnosis serum LDH values). Intensive combined poly-chemotherapy bears the best outcome results. The treatment is usually short length, but adult patients could be treated with longer duration treatment, ALL type. Recently, addition of rituximab to the already established treatment protocols appears to further improve treatment outcomes for all patient groups. Historically, pediatric patients have the best outcome, while inferior outcome is known for older patients, patients with extensive disease, CNS and bone marrow involvement. Extensive surgery and radiation therapy have no place in Burkitt lymphoma treatment. Relapsing patients usually have resistant disease and following remission reinduction stem cell transplantation appears advantageous. Under investigation, but with initially promising results, is the minimal residual disease evaluation of the patients. Thus, early non complete remission status and subclinical, early stage relapse can trigger appropriate therapeutic interventions. New drugs and immune based antibody and cellular treatments are under investigation with the intent to further improve treatment outcome of Burkitt lymphoma patients at diagnosis or at relapse.