But the theme here is Burkitt's Lymphoma
Burkitt's Lymphoma Is A Potentially Fatal Opportunistic Disease!
O Burkitt's lymphoma (Formerly High-grade non-Hodgkin's lymphoma of small non-cleaved cells) is a highly aggressive mature B cell neoplasm (cancer) that affects mainly the younger age group, being endemic in African regions.
T (8; 14) (q24; q32) - translocation of genetic material between chromosomes 8 and 14 - occurs in 80% of cases. The other 20% correspond to (2; 8) (p12; q24) and (8; 22) (q24; q11).
All of these cytogenetic changes involve the overexpression of a gene with multiple cellular functions called c-myc. This gene is decisive in the development of this pathology.
Histological sections of the tumor may have the appearance of a “starry sky” (starry sky). There is an association between infection by the virus Epstein-Barr virus (EBV) and the development of Burkitt's lymphoma.
It often presents with extra nodal disease. The carriers can be affected mainly in the bone structures, with massive oral lesions, the mandible being the most affected bone. It can also affect several structures, including kidneys and ovaries.
Burkitt's lymphoma cells are morphologically identical to cells of acute lymphoid leukemia, subtype FAB L3. Cellular immunophenotyping reveals CD20 positivity in the vast majority of cases.
The first description of this disease was made in 1958 by Irish physician Denis P. Burkitt, who observed a high prevalence of a highly aggressive tumor that endemically affected children in equatorial Africa.
It was initially described as sarcoma (cancer of the soft tissues), but later it was observed that it was a primary tumor of the lymphatic system (lymphoma).
Burkitt's lymphoma and acute L3 lymphoid leukemia (Burkitt's leukemia) were believed for many years to represent different diseases. However, with the advent of modern techniques of chromosomal and genetic analysis, it was possible to determine that both represented symptoms of the same disease.
In 2001, the World Health Organization (WHO) classified these conditions as being a single entity (mature B cell neoplasia, subtype Lymphoma / Burkitt's Leukemia), with three clinical variants.
According to the WHO, three clinical variants are identifiable:
- Endemic variant: occurs in children from equatorial Africa where malaria is endemic and has a high correlation with EBV infection (98% of cases). It is believed that the status of chronic immunodepression caused by malaria contributes to the development of the disease.
- Sporadic variant: affects children and adults from other geographical areas. It is associated with EBV infection Epstein-Barr virus) in 5-10% of cases.
- Associated with immunodeficiency: in most cases associated with infection by the human immunodeficiency virus (HIV). 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). "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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