Giovanni Donadoni,1,2 Marta Bruno-Ventre,1,3 Andrés J. M. Ferreri1,2
1. Unit of Lymphoid Malignancies
2. Division of Onco-Hematological Medicine
3. Unit of Hematology and Bone Marrow Transplantation Department of Onco-Hematology, San Raffaele Scientific Institute, Milan, Italy
Disclosure: No potential conflict of interest.
Citation: EMJ Hema. 2013;1:38-52.
Burkitt lymphoma (BL) is a highly aggressive B-cell malignancy, occurring with increased frequency among patients infected with HIV. For several years, the immunocompromised state of HIV-positive patients was advocated as a sufficient reason to avoid the intensive chemotherapeutic regimens used in HIV-negative BL. However, with the introduction of the highly active antiretroviral therapy (HAART), the subsequent improvement of the immunological state of HIV-positive patients, and the disappointing results of less intensive schedules, investigators began to apply the same chemotherapeutic regimens used as a gold standard in HIV-negative non-Hodgkin lymphoma (NHL), including the use of rituximab. Despite promising results of different schedules in early-phase studies, agreement on the treatment of HIV-positive BL is still lacking, and further trials are needed to define a standard of care. Moreover, new treatment frontiers need to focus on improving the outcome for patients with advanced immunosuppression, unfavourable prognostic features- such as advanced stages and high International Prognostic Index (IPI) scores – and for those with adverse tumour biology.
This paper aims to revise the main epidemiological and physiopathological features of HIV-positive BL, to summarise the most relevant steps in the treatment of affected patients, and to elucidate the role of HAART in allowing HIV-positive patients to be managed with the therapeutic strategies currently used in HIV-negative patients with BL.