Management of adults with Burkitt lymphoma

Abstract

Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma characterized by marked tumor proliferation resulting from translocation of the MYC oncogene. Distinct clinical variants include endemic, sporadic, and immunodeficiency-associated cases. All variants are characterized by rapidly dividing tumor masses that quickly disseminate to extranodal sites, including the bone marrow and central nervous system (CNS). Although common in children, BL is rare in adults, mandating a high index of clinical suspicion for timely diagnosis. Prompt recognition and initiation of comprehensive supportive care are essential for prevention of early complications, such as tumor lysis syndrome and multisystem organ dysfunction. BL is highly sensitive to chemotherapy, and patients who tolerate highly intensive combination chemotherapy regimens are frequently cured. Most regimens were developed in children and young adults, however, and the treatment-related toxicities remain a major barrier for those with advanced age and/or comorbid conditions. Younger patients are less susceptible to acute toxicities but are more likely to experience long-term sequelae of treatment, including infertility and secondary malignancies. The infusional regimen of dose-adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, and regular- or double-dose rituximab (DA-EPOCH-R or -RR) is less toxic than standard BL regimens, yet maintains high rates of cure across a diverse range of patients, including those with disseminated disease, advanced age, and HIV infection. Patients with low-risk BL can be cured with just 3 cycles of DA-EPOCH-RR. Still, patients with CNS involvement remain at high risk for early death, and prevention of late CNS relapses remains a priority. Future studies combining rational targeted agents with DA-EPOCH-R or -RR may further improve the cure rate.