Burkitt's lymphoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Burkitt's lymphoma may be classified according to the World Health Organization (WHO) classification into 3 clinical variants. The clinical variants are endemic Burkitt's lymphoma, sporadic Burkitt's lymphoma, and immunodeficiency-associated Burkitt's lymphoma.

Classification

Burkitt's lymphoma may be classified according to WHO classification into 3 clinical variants:

• Endemic Burkitt's lymphoma
• Sporadic Burkitt's lymphoma
• Immunodeficiency-associated Burkitt's lymphoma

It is almost impossible to differentiate these three clinical variants based on histology (i.e. microscopic appearance) or immunophenotype.

Classification Based on The Geographic Distribution and Clinical Presentation

Name	Description
Endemic Burkitt's lymphoma (African Burkitt's lymphoma)	The endemic form always presents as a jaw or facial bone tumor Primary involvement of the abdomen is not very common The primary tumor can disseminate to the extranodal sites such as the mesentery, ovary, testis, kidney, breast, and meninges Peripheral lymph nodes, mediastinum, and spleen involvement are uncommon Bone marrow involvement is noted in less than 10 percent of patients at the time of initial diagnosis but is a common complication of treatment-resistant disease.
Sporadic Burkitt's lymphoma (Non-African or Non-endemic Burkitt's lymphoma)	The most common type of Burkitt's lymphoma in North America and Europe Sometimes associated with the Epstein-Barr virus, but in many cases this virus is not present Non-Hodgkin's lymphoma, which includes Burkitt's, accounts for 30-50% of childhood lymphoma This form usually has an abdominal presentation, most often with massive dissemination and ascites; the distal ileum, stomach , cecum and/or mesentery, kidney, testis, ovary, breast, bone marrow, or central nervous system (CNS) are involved Presenting symptoms can be those that are like bowel obstruction or gastrointestinal bleeding, often disguised as acute appendicitis or intussusception Almost 25 percent of cases will have presentation of the disease in the jaw or facial bones Lymphadenopathy, is generally localized Bone marrow and CNS involvement occurs in about 30 and 15 percent of cases, respectively, but are likely complications of recurrent or treatment resistant disease
Immunodeficiency-associated Burkitt's lymphoma	The presentation of patients with immunodeficiency-related Burkitt's lymphoma is often accompanied by signs or symptoms related to the underlying immunodeficiency which can be the following: AIDS Congenital immunodeficiency Acquired immunodeficiency due to bone marrow or solid organ transplantation Immunodeficiency-related cases frequently often involve lymph nodes, bone marrow, and CNS

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