Unraveling Endemic Burkitt's Lymphoma
By Ilene Raymond Editor-in-Chief June 15, 2006
For ten years, Lorenzo Leoncini, MD, professor of pathology and professor of the SHRO labs in University of Siena, Italy, has traveled to sub-Saharan Africa to study endemic Burkitt's lymphoma, the most common childhood cancer in Africa.
Although the cancer affects adults and children around the world, it is most prevalent in equatorial Africa, where it strikes ten out of 100,000 young boys between the ages of 2 and 14. The concentration makes a perfect “genetic footprint” for endemic Burkitt's lymphoma researchers.
“The area is like a natural experimental model,” says Leoncini, whose office is dotted with elephant carvings and photos of his African travels.
Burkitt's lymphoma most often begins with a tumor in the jaw. The rapid rate of growth in the cancer leads to large tumors that, if left untreated, are ultimately fatal. While the disease is curable through chemotherapy, many African patients still die, in part because the inadequate hospital conditions in the area make chemo treatments difficult.
“For that reason, we're very interested in exploring alternative methods of treatment,” says Leoncini.
“Burkitt's lymphoma was a disease of firsts,” says Leoncini. “It was the first time we recognized that an environmental factor combined with a virus and chromosomal damage to cause cancer. It was also the first cancer to be successfully treated with chemotherapy alone.”
Denis Burkitt, an Irish surgeon working in Uganda, described the endemic form of the disease in 1958. Three subtypes of Burkitt's lymphoma exist: endemic, sporadic and a form associated with HIV. Though all of the Burkitt's lymphoma tumor cells show damage in the set of chromosomes that include c-myc, a gene involved in cellular proliferation, two other factors play a role in endemic Burkitt's lymphoma in Africa: malaria and Epstein-Barr virus (EBV) infection.
Yet, according to Leoncini, little recent research has examined the potential mechanisms between these two pathogens.
To help explore how these pathogens interact in Burkitt's lymphoma , Leoncini and his colleagues have been collecting Burkitt's lymphoma tissue samples in equatorial Africa. They plan to use the tissues to try to better understand the mechanism of Burkitt's and then compare that data with cases in Europe and North America.
“We suspect that there will be differences which will help us to better define the role of EBV infection and malaria in the African cases,” he says.
One step towards that goal is a forthcoming meeting planned to mark the 50th anniversary of the discovery of endemic Burkitt's lymphoma to be held in Kampala, Uganda in 2008. Designed to foster new approaches to treatment, the meeting will gather hematologists, pathologists, and biologists to create links between the clinical aspects, biological and pathological features and basic research.
“The meeting aims to bring clinicians and pharmaceutical companies to where Burkitt's lymphoma remains the most common childhood cancer,” says Leoncini. “We want to set up large scale clinical trials and cooperative projects so we can combine resources and expertise from many countries and different medical specialties to improve the treatment of this disease.”
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