Epidemiology, clinical manifestations, pathologic features, and diagnosis of Burkitt lymphoma
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jon C Aster, MD
Jon C Aster, MD
- Professor of Pathology
- Harvard Medical School
- Section Editors
- Andrew Lister, MD, FRCP, FRCPath, FRCR
Andrew Lister, MD, FRCP, FRCPath, FRCR
- Section Editor — Lymphoproliferative Disorders
- Professor of Medical Oncology
- St. Bartholomew's Hospital, London
- Julie R Park, MD
Julie R Park, MD
- Section Editor — Lymphoma; Pediatric Oncology
- Professor of Pediatrics
- University of Washington
Burkitt lymphoma (BL) is a highly aggressive B cell non-Hodgkin lymphoma characterized by the translocation and deregulation of the c-MYC gene on chromosome 8. Three distinct clinical forms of BL are recognized: endemic (African), sporadic (non-endemic), and immunodeficiency-associated. Although they are histologically identical and have similar clinical behavior, there are differences in epidemiology, clinical presentation, and genetic features between the three forms as described below.
BL and Burkitt leukemia are considered different manifestations of the same disease in the World Health Organization (WHO) classification of hematologic malignancies; "Burkitt-like lymphoma with 11q aberration" and "High-grade B cell lymphoma with MYC and BCL2 and/or BCL6 rearrangement" have been proposed as separate entities distinct from BL [1,2]. In addition, the WHO provides an overlap category termed "High-grade B cell lymphoma, not otherwise specified." Some tumors in this group were previously classified as "Burkitt-like" lymphoma. (See "Classification of the hematopoietic neoplasms".)
The epidemiology, clinical features, pathology, and diagnosis of BL will be reviewed here. The pathobiology and treatment of BL are discussed separately, as is a general approach to the diagnosis, staging, and prognosis of the lymphomas. (See "Pathobiology of Burkitt lymphoma" and "Treatment of Burkitt leukemia/lymphoma in adults" and "Clinical presentation and diagnosis of non-Hodgkin lymphoma" and "Evaluation and staging of non-Hodgkin lymphoma".)
The exact worldwide incidence of Burkitt lymphoma (BL) is not known, as collection of these types of epidemiologic data is limited by a lack of resources that are needed for case ascertainment and accurate diagnosis in the developing countries that have the highest apparent incidence (eg, equatorial Africa) . For epidemiologic and diagnostic purposes, cases of BL are generally divided into three distinct clinical forms: endemic (African), sporadic (non-endemic), and immunodeficiency-associated. The endemic and sporadic clinical variants of BL differ geographically.
●Endemic – The endemic variant is found in equatorial Africa and New Guinea. The incidence of BL in Africa is approximately 50-fold higher than that seen in the United States (US) . BL accounts for 30 to 50 percent of all childhood cancer in equatorial Africa with an estimated incidence of 3 to 6 cases per 100,000 children per year . The peak incidence occurs in children age four to seven years, and the male:female ratio is approximately 2:1.
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- CLINICAL FEATURES
- Genetic features
- - Translocations involving the c-MYC oncogene
- - Other genetic abnormalities
- DIFFERENTIAL DIAGNOSIS
- Diffuse large B cell lymphoma
- Lymphoblastic lymphoma
- Mantle cell lymphoma, blastoid variant