Categories of Burkitt lymphoma
Burkitt lymphoma is a cancer of the lymphatic system, particularly B lymphocytes found in the germinal center. The overall cure rate for Burkitt lymphoma in developed countries is about 90%, but worse in low-income countries. Burkitt lymphoma is uncommon in adults, in whom it has a worse prognosis.Burkitt lymphoma can be divided into three main clinical variants:
The endemic, the sporadic, and the immunodeficiency-associated variants.
- The Endemic variant: It is most commonly occurs in children living in malaria endemic regions of the world. Epstein–Barr virus (EBV) infection is found in nearly all patients. Chronic malaria is believed to reduce resistance to EBV, allowing it to take hold. The disease characteristically involves the jaw or other facial bone, distal ileum, cecum, ovaries, kidney, or breast.
- The sporadic: It is the type of Burkitt lymphoma is the most common variant found in places where malaria is not holoendemic. The tumour cells have a similar appearance to the cancer cells of classical endemic Burkitt lymphoma. Sporadic lymphomas are rarely associated with the Epstein–Barr virus. Non-Hodgkin lymphoma, which includes Burkitt, accounts for 30–50% of childhood lymphoma. The jaw is less commonly involved, compared to the endemic variant. The ileocecal region is the common site of involvement.
- Immunodeficiency-associated: Burkitt lymphoma is usually associated with HIV infection] or occurs in the setting of post-transplant patients who are taking immunosuppressive drugs. Burkitt lymphoma can be one of the diseases associated with the initial manifestation of AIDS.
By morphology (i.e., microscopic appearance) or immunophenotype, it is almost impossible to differentiate these three clinical variants. Immunodeficiency-associated Burkitt lymphoma may demonstrate more plasmacytic appearance or more pleomorphism, but these features are not specific.
Burkitt lymphoma is commonly associated with the infection of B cell lymphocytes with the Epstein–Barr virus (EBV) and in these cases in considered to be one form of the Epstein–Barr virus-associated lymphoproliferative diseases. The epidemic variant of Burkitt lymphoma (eBL) is in almost all cases associated with EBV infection. However, the sporadic variant, which afflicts ~1,200 individuals.The immunodeficiency-associated variant of Burkitt lymphoma strikes 30-40% of individuals with HIV-induced AIDS and rare cases of patients who received a bone marrow or other organ transplant; in the latter cases, individuals have almost always received intensive chemotherapy and therefore are immunodeficient. About 30% of individuals with the immunodeficiency variant are infected with EBV. The fact that some Burkitt lymphoma cases do not involve EBV allows that many cases of the disease are not caused and/or promoted by EBV, i.e. the virus may be an innocent passenger virus in these cases. However, the almost ubiquitous presence of the virus in the epidemic variant of Burkitt's lymphoma suggests that it contributes to the development and/or progression of this variant. The mutational landscape in BL has recently been found to differ between tumors with and without EBV infection, further strengthening the role of the virus in lymphomagenesis.
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