logo logo
Search: Organ site Search: Tumor / Syndrome
or
WHO Classification of Tumours
Burkitt lymphoma
Small intestine


Definition

Burkitt lymphoma (BL) is a fast growing, highly aggressive lymphoma composed of monomorphic medium-sized B-cells with basophilic cytoplasm and numerous mitotic figures. It often presents in extranodal sites or as an acute leukaemia. It typically harbours the Epstein-Barr virus (EBV); MYC [t(8;14)] translocation is highly characteristic but not specific.

> Endemic Burkitt lymphoma
This variant is the most common childhood malignancy in equatorial Africa, with an incidence peak at 4 to 7 years and a male:female ratio of 2:1. BL is also endemic in Papua, New Guinea. In endemic BL, the EBV genome is present in the majority of the neoplastic cells in all patients and there is strong epidemiological link with holoendemic malaria but climatic factors may alo play a role. The jaw and other facial bones are most frequently affected.

> Sporadic Burkitt lymphoma
This variant is seen through out the world, mainly in children and young adults. The incidence is low, representing only 1−2% of all lymphomas in Western Europe and in the USA, but accounts for 30−50% of all childhood lymphomas. The median age of adult patients is 30 years and the M:F tatio is 2-3:1. The majority of cases presents with abdominal masses, particulary in the ileo-caecal region.

> Immunodeficiency-associated Burkitt lymphoma
This variant is primarily seen in association with the human immunodeficiency virus (HIV) infection, often occurring as the initial manifestation of the acquired immunodeficiency syndrome (AIDS).

In all variants, the CNS, ovaries, kidneys and breasts are also frequently involved.

> Burkitt leukaemia
A leukaemic phase can be observed in patients with bulky disease. Only rare, mostly male cases present as pure acute leukaemia with peripheral blood (P)B and bone marrow (BM) involvement. Burkitt leukaemia, also termed acute lymphocytic leukaemia-L3, tends to involve the CNS at diagnosis or in early disease stages. Its rapid chemosensitivity easily leads to an acute tumour lysis syndrome

Click for details
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.