Moonlit Night - "Starry sky pattern" of Burkitt lymphoma.
Burkitt lymphoma - Syn: Burkitt's
1. Burkitt lymphoma was described in 1958 by Dr Denis Burkitt in Uganda.
2. It was initially thought to be a sarcoma of the jaw. Originally it was called "round cell sarcoma.
3. In 1961 Burkitt and O'Connor further described the features of the tumours which is now known as Burkitt lymphoma.
4. Shortly it became well known that this was a distinct form of Non Hodgkin's lymphoma. Based on histopathologic features, Wright distinguished this lymphoma from other forms of lymphomas.
5. Burkitt lymphoma is most common form of lymphoma in the children. Boys are more susceptible than girls. It is an aggressive tumour but curable in children.
6. Study led to the discovery of Epstein Barr virus (EBV) and its importance in the cause of several viral illnesses and malignancies. EBV may play a role in the pathogenesis of Burkitt lymphoma by deregulation of the oncogene c-MYC by chromosomal translocation.
7. There are three general subtypes of Burkitt lymphoma:
- A) Endemic Burkitt lymphoma: This subtype was originally described by Burkitt.It is usually found in Africa and involves the facial skeleton in children between ages 2 to 9.
- B) Sporadic Burkitt lymphoma: It is the form which is usually described outside the Africa. It is morphologically similar to Burkitt lymphoma and affecting mainly abdominal viscera.This type of lesion can occur at any age. Sporadic cases also arise in the absence of immune impairment, and EBV is detectable in less than a quarter of these cases.
- C) Burkitt lymphoma associated with HIV infection. HIV associated Burkitt lymphoma can be identified in any geographical area and at all ages. It has been reported in developed countries and in HIV positive adults in Africa.
8. Articles have been published showing role of EBV and Plasmodium falciparum in the development of Burkitt lymphoma. Plasmodium falciparum is not an oncogenic agent. It has been suggested that malaria could disrupt immune response against EBV, leading to development of Burkitt lymphoma.
9. It has been reported that exposure to milk bush Euphorbia tirucalli and other Euphorbiaceae species are possible environmental risk factors for Burkitt lymphoma. According to the authors it is probably due to their ability to activate the viral replication cycle in the latent phase of EBV-infected cells.
10. This tumour is composed of medium sized B-cells with a high proliferation fraction, interpersed with macrophages containing cellular debris, giving a "starry sky" appearance. Mitotic rate is high.
11. It may be difficult to distinguish with certainty from diffuse large cell lymphoma.
- Surface IgM : Positive
- CD5, CD23 : Negative
- CD10 : Positive
- CD19, 20, 22, 79a : Positive
- Ki67 of cells : More than 85%
13. Genetics t(2;8), t(8;14) or t(8;22). Almost all cases, from whatever country, show a chromosomal translocation involving the MYC gene on chromosome 8 and the gene for Ig heavy chain or less commonly, one of the two Ig light chain genes.
14. The disease may respond to aggressive therapy.
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