Burkitt lymphoma is a form of non-Hodgkin's lymphoma in which cancer starts in immune cells called B-cells. Recognized as the fastest growing human tumor, Burkitt lymphoma is associated with impaired immunity and is rapidly fatal if left untreated. However, intensive chemotherapy can achieve long term survival in more than half the people with Burkitt lymphoma.
Burkitt lymphoma is named after British surgeon Denis Burkitt, who first identified this unusual disease in 1956 among children in Africa. In Africa, Burkitt lymphoma is common in young children who also have malaria and Epstein-Barr, the virus that causes infectious mononucleosis. One mechanism may be that malaria weakens the immune system's response to Epstein-Barr, allowing it to change infected B-cells into cancerous cells. About 98% of African cases are associated with Epstein-Barr infection.
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Outside of Africa, Burkitt lymphoma is rare. In the U.S., about 1,200 people are diagnosed each year, and about 59% of patients are over age 40. Burkitt lymphoma is especially likely to develop in people infected with HIV, the virus that causes AIDS. Before highly active antiretroviral therapy (HAART) became a widespread treatment for HIV/AIDS, the incidence of Burkitt lymphoma was estimated to be 1,000 times higher in HIV-positive people than in the general population.
Types of Burkitt Lymphoma
In the World Health Organization classification, there are three types of Burkitt lymphoma:
Endemic (African). Endemic Burkitt lymphoma primarily affects African children ages 4 to 7 and is twice as common in boys as in girls.
Sporadic (non-African). Sporadic Burkitt lymphoma occurs worldwide. Globally, it accounts for 1% to 2% of adult lymphoma cases. In the U.S. and Western Europe, it accounts for up to 40% of pediatric lymphoma cases.
Immunodeficiency-associated. This variant of Burkitt lymphoma is most common in people with HIV/AIDS. It accounts for 30% to 40% of non-Hodgkin lymphoma in HIV patients and may be an AIDS-defining disease. It also can occur in people with congenital conditions that cause immune deficiency and in organ-transplant patients who take immunosuppressive drugs.
Compared to the endemic type, the incidence of Epstein-Barr infection is considerably lower in the other two types of Burkitt lymphoma. In the sporadic disease, Epstein-Barr occurs in about 20% of the patients. With the immunodeficiency-associated type it occurs in about 30% to 40%. So the association of Epstein-Barr with these two types of Burkitt lymphoma is unclear.