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 (PB) 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
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
International Agency for Research on Cancer: Lyon 2008
In the stomach, classical BLs may be encountered
Andoh A, Takaya H, Bamba M, Sakumoto H, Inoue T, Tujikawa T, Koyama S, Fujiyama Y, Bamba T (1998)
Primary gastric Burkitt's lymphoma presenting with c-myc gene rearrangement.
J Gastroenterol 33: 710-5
Park YH, Kim WS, Kang HJ, Na II, Ryoo BY, Yang SH, Lee SS, Uhm JE, Kim K, Jung CW, Park K, Ko YH (2006)
. The morphology is identical to that of Burkitt lymphoma encountered elsewhere, with diffuse sheets of medium-sized cells with scanty cytoplasm and round/oval nuclei containing small nucleoli. Within the sheets there are numerous macrophages, giving a “starry sky” appearance. Mitoses are frequent and apoptotic debris is abundant. The cells express CD10, CD20 and BCL6, but not BCL2. Nearly 100% of nuclei are immunoreactive for Ki67. The cases carry t(8;14). The proportion that are EBV positive varies, being relatively low (15–20%) in sporadic Burkitt lymphoma, and higher in cases associated with immunodeficiency (25–40%)
Gastric Burkitt lymphoma is a distinct subtype that has superior outcomes to other types of Burkitt lymphoma/leukemia.
Ann Hematol 85: 285-90
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
International Agency for Research on Cancer: Lyon 2010