Clinical Pearls & Morning Reports
Published September 21, 2022
The clinical presentation of Burkitt’s lymphoma is often dramatic. Read the NEJM Review Article here.
Q: Is Burkitt’s lymphoma an aggressive lymphoma?
A: Burkitt’s lymphoma is a highly aggressive B-cell lymphoma and the most rapidly proliferating human cancer. Clinical signs suggestive of Burkitt’s lymphoma should be considered a medical emergency, and intensive supportive care designed to prevent sepsis, intestinal perforation, and tumor lysis syndrome should be initiated immediately.
Q: A translocation involving what oncogene is associated with the development of Burkitt’s lymphoma?
A: MYC translocations are nearly universal across Burkitt’s lymphoma variants. Yet deregulation of MYC alone is generally not sufficient for lymphomagenesis, and cooperating genetic alterations are common. It is critical to distinguish Burkitt’s lymphoma from diffuse large-B-cell lymphoma and other high-grade B-cell lymphomas that may carry a MYC translocation. Strong BCL2 expression is uncommon, and the level of expression can be used to distinguish Burkitt’s lymphoma from other high-grade B-cell lymphomas.
A: We currently recognize three Burkitt’s lymphoma variants: endemic, sporadic, and immunodeficiency-associated. Endemic Burkitt’s lymphoma occurs in geographic regions where malaria is holoendemic. Endemic Burkitt’s lymphoma is virtually always associated with Epstein-Barr virus (EBV) infection, and elevated titers of EBV antibodies are associated with an increased risk. The median age at presentation among persons with sporadic Burkitt’s lymphoma is 10 years, with additional incidence peaks at 40 years and 75 years of age. Males are affected 3 to 4 times as often as females. Sporadic Burkitt’s lymphoma can affect virtually any organ but often manifests as a rapidly enlarging abdominal mass, with involvement of the ileocecal region that mimics acute appendicitis or bowel obstruction. Involvement of the central nervous system occurs in up to 20% of cases, and the bone marrow is involved in 30 to 35% of cases. EBV is associated with 20 to 30% of cases of sporadic Burkitt’s lymphoma, most often in patients over the age of 50 years. The final variant most commonly arises in patients infected with human immunodeficiency virus (HIV). Burkitt’s lymphoma accounts for nearly 40% of lymphomas that arise in HIV-infected patients and notably occurs in those with relatively normal CD4 counts.
A: The general treatment approach is the same for all clinical variants and does not depend on EBV status. More than 90% of children and adolescents are cured with highly dose-intensive chemotherapy, whereas adults are more susceptible to the toxic effects of treatment, and prospective trials have shown that only 75 to 85% of patients have a long-term remission. Although front-line therapy is highly effective, nearly all cases of recurrent or refractory disease are fatal. A greater understanding of the biologic basis of resistance to therapy is needed to move beyond chemotherapy with new pathway inhibitors and immunotherapy.