How I treat Burkitt lymphoma in children, adolescents, and young adults in sub-Saharan Africa

Abstract

Burkitt lymphoma (BL) is the most common pediatric cancer in sub-Saharan Africa (SSA), and also occurs frequently among adolescents and young adults (AYAs), often associated with HIV. Treating BL in SSA poses particular challenges. Although highly effective, high-intensity cytotoxic treatments used in resource-rich settings are usually not feasible, and lower-intensity continuous infusion approaches are impractical. In this article, based on evidence from the region, we review management strategies for SSA focused on diagnosis and use of prephase and definitive treatment. Additionally, potentially better approaches for risk stratification and individualized therapy are elaborated. Compared with historical very low-intensity approaches, the relative safety, feasibility, and outcomes of regimens incorporating anthracyclines and/or high-dose systemic methotrexate for this population are discussed, along with requirements to administer such regimens safely. Finally, research priorities for BL in SSA are outlined including novel therapies, to reduce the unacceptable gap in outcomes for patients in SSA vs high-income countries (HICs). Sustained commitment to incremental advances and innovation, as in cooperative pediatric oncology groups in HICs, is required to transform care and outcomes for BL in SSA through international collaboration.

Figure 1.

Illustration of the BL care cascade in sub-Saharan Africa and opportunities to introduce bias into survival estimates.

Figure 2.

Suggested management for BL in sub-Saharan Africa. *Optimal diagnostic evaluation and definitive treatment will be strongly determined by local expertise, resources, and infrastructure. Treatment on a clinical trial or prospective, longitudinal, cohort study is strongly recommended whenever possible. Concurrent antiretroviral therapy should be administered if HIV-infected. In middle-income countries, high-income country regimens can often be successfully followed. ‡Poor performance status defined as Lansky score ≤50 or Eastern Cooperative Oncology Group (ECOG) score ≥3. Good performance status defined as all others. ‡Low risk defined as all of the following: stage I/II, no abdominal disease, largest tumor bulk <10 cm, and lactate dehydrogenase <2 times upper limit of normal. High risk defined as all others. CSF, cerebrospinal fluid; CT, computed tomography; EBV, Epstein-Barr virus.