Non-endemic Burkitt’s Lymphoma
Until characterization of HIV/AIDS malignancies non-endemic was one of the two categories of Burkitt’s lymphoma. The types vary mainly in the proportions of several characterizing features. In 1904 Albert Cook made observations in a Mohammandan child who had a malignant tumor that appears to be what Denis Burkitt in 1958 and subsequent years by others provided for founding characteristics. The major defining features included a peculiar age distribution, characteristic anatomical distribution and geographical distribution unrelated to genetic factors. Additionally the disease has typical cytogenesis and cytologic, histologic, immunologic, biologic, and clinical features. Non-endemic Burkitt’s is rare and predominantly found in the temperate regions. In Europe, North and South America and Oceanic Age Standardized Rates in most populations are less than 1 per million in many countries. In Asia, the levels are low and vary from country to country while Australian pediatric cancer registry documented Crude Incidence of 1.8.
Mean age of onset is about 12 years with a minor percentage of adult lymphoma, peak incidents in second and third decades of life. Male to female ratio is 3.5:1 and varies from one region to another. Primary chromosome anomaly is t (8; 14) (q24; q32), found in 60–70 % of the cases.
Cytological features on hematological stains show medium size lymphoid cells exhibiting little morphologic heterogeneity, small to moderately plentiful bluish cytoplasm and some nuclear vacuoles. The characteristic histologic pattern is referred to as starry sky appearance. Cell markers are of B cell while the EBV genome is expressed in 15–20 % of cases.
The anatomic sites involved generate the presenting clinical features; pain, feeling sick, and sometimes obstruction in the chest, throat, abdomen, or central nervous system. Ascites is present on about 24 %. Hepatic involvement is usually before spleen. Other sites include kidneys, ovaries, skeletal system, bone, thyroid gland, and oral pharyngeal, and these could present with protean features.
Age and anatomic primary sites are apparent with abdomen, breast, and lymph nodes, Stages C and D often with B symptoms frequent in adults.
Generally this is a severe disease; however, a lot is still to be learnt about sporadic Burkitt’s lymphoma to find signatures that will obviate the descriptions in terms of largely geographical and clinical basis.
KeywordsCrude Incidence Danish Cancer Registry Nuclear Vacuole Primary Chromosome Elevated Serum Uric Acid Level
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