Burkitt lymphoma in adolescents and young adults: management challenges

Abstract

About one-half of all Burkitt lymphoma (BL) patients are younger than 40 years, and one-third belong to the adolescent and young adult (AYA) subset, defined by an age between 15 and 25–40 years, based on selection criteria used in different reports. BL is an aggressive B-cell neoplasm displaying highly characteristic clinico-diagnostic features, the biologic hallmark of which is a translocation involving immunoglobulin and c-MYC genes. It presents as sporadic, endemic, or epidemic disease. Endemicity is pathogenetically linked to an imbalance of the immune system which occurs in African children infected by malaria parasites and Epstein–Barr virus, while the epidemic form strictly follows the pattern of infection by HIV. BL shows propensity to extranodal involvement of abdominal organs, bone marrow, and central nervous system, and can cause severe metabolic and renal impairment. Nevertheless, BL is highly responsive to specifically designed short-intensive, rotational multiagent chemotherapy programs, empowered by the anti-CD20 monoclonal antibody rituximab. When carefully applied with appropriate supportive measures, these modern programs achieve a cure rate of approximately 90% in the average AYA patient, irrespective of clinical stage, which is the best result achievable in any aggressive lymphoid malignancy to date. The challenges ahead concern the following: optimization of management in underdeveloped countries, with reduction of diagnostic and referral-for-care intervals, and the applicability of currently curative regimens; the development of lower intensity but equally effective treatments for frail or immunocompromised patients at risk of death by complications; the identification of very high-risk patients through positron-emission tomography and minimal residual disease assays; and the assessment in these and the few refractory/relapsed ones of new monoclonals (ofatumumab, blinatumomab, inotuzumab ozogamicin) and new molecules targeting c-MYC and key proliferative steps of B-cell malignancies.

HIV-related Malignancies and Mimics: Imaging Findings and Management

“…Burkitt lymphoma occurs in HIV-infected patients with a high CD4 lymphocyte count (> 200/ µl), suggesting that immunosuppression alone is not the cause of malignancy (32). Immunodeficiency-related Burkitt lymphoma more commonly involves the lymph nodes, bone marrow, and central nervous system (Fig 8) (33). Primary hepatic Burkitt lymphoma, a highly aggressive form of extranodal lymphoma, has been reported as a rare initial manifestation in HIV-infected patients with hepatitis B seropositivity (34).…”

Burkitt's Lymphoma Presenting as Endobronchial Lesion

“…The optimal initial therapy of Burkitt's lymphoma has not been clearly defined given the paucity of randomized studies in this uncommon disease and the fact that different, modern Burkitt's lymphoma regimens can all achieve excellent results in younger age groups. Standard chemotherapy such as CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) is inadequate for treating Burkitt's lymphoma, and there is no role for radiation therapy, even for localized disease or paraspinal presentations, which respond very quickly to chemotherapy [13].…”

Adult Burkitt lymphoma- an Island between lymphomas and leukemias

“…BL is characterized by translocation between c-MYC gene and one of the immunoglobulin (Ig) chains on chromosome 8 [5,6]. The dysregulation causes activation of the c-MYC proto-oncogene located at 8q24.…”