O Burkitt's lymphoma (Formerly Non-Hodgkin Lymphoma high grade small non-cleaved cell) Is a neoplasm (cancer) of highly aggressive mature B cells that mainly affects the younger age, being endemic in African regions.
The t (8, 14) (q24; q32) - translocation of genetic material between the 8 and 14 chromosomes - occurs in 80% of cases. The other match at 20% (2, 8) (p12; q24) et (8, 22) (q24; q11).
All these cytogenetic changes involve overexpression of a gene with multiple cellular functions called c-myc. This gene is crucial in the development of this pathology.
The histological tumor sections may present aspect of "starry sky" (starry sky). There is an association between the virus infection Epstein-Barr virus (EBV) and the development of Burkitt's lymphoma.
Often presents with extra nodal disease. Holders may be affected especially in bone structures, with massive oral lesions, and the jaw hit the hardest bone. It can also affect various structures, including kidneys, and ovaries.
The Burkitt's lymphoma cells are morphologically identical to cells of acute lymphoblastic leukemia, FAB subtype L3. Cell immunophenotyping shows the positivity extended CD20 most cases.
The first report of the disease was performed in 1958 by the Irish doctor Denis P. Burkitt, who observed high prevalence of a highly aggressive tumor that reached endemic form children in equatorial Africa.
It was initially described as sarcoma (soft tissue cancer), but later it was observed that it was a primary tumor of the lymphatic system (lymphoma).
It was believed for many years that Burkitt's lymphoma and acute lymphoblastic leukemia L3 (Burkitt leukemia) represented different diseases. However, with the advent of modern techniques of chromosomal and genetic analysis, it was determined that both represented symptoms of the same disease.
In 2001 the World Health Organization (WHO) has classified these conditions as a single entity (cancer of mature B cells, subtype lymphoma / Burkitt leukemia), with three clinical variants.
According to the WHO, three clinical variants are identifiable:
- Endemic variant: occurs in children in equatorial Africa where malaria is endemic and has a strong correlation with EBV infection (98% of cases). It is believed that the status chronic immunosuppression caused by malaria contributes to the development of the disease.
- Sporadic variant: affects children and adults of other areas. It is associated with EBV infection (virus Epstein-Barr virus) In 5-10% of cases.
- Associated with immunodeficiency: in most cases associated with human immunodeficiency virus (HIV) infection. The association with EBV co-infection is between 25-40% of cases.
- Hecht J, Aster J. (2000). "Molecular Biology of Burkitt's lymphoma." J Clin Oncol 18: 3703.
- Burkitt D. (1958). "The sarcoma Involving the jaws in African children." Br J Surg 46: 218.
- Jaffe ES, Harris NL, Stein H, Vardiman JW. Pathology & Genetics of Tumours haematopoietic and lymphoid tissues. [Sl: sn], 2001.
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