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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Burkitt lymphoma is the fastest-growing human tumour, driven by a single rearrangement that switches on the MYC gene. That speed makes it exquisitely sensitive to chemotherapy: short, intense courses, now with the antibody rituximab, cure the great majority of children in well-resourced settings. The remaining task is to bring the same cure to the African children who make up most cases.
Burkitt lymphoma is a mature B-cell neoplasm defined by translocation of MYC to an immunoglobulin locus, most often t(8;14), with cooperating mutations in ID3, TCF3 and CCND3. It exists in three epidemiological forms: endemic (equatorial Africa and Papua New Guinea, almost always Epstein-Barr virus positive, linked to Plasmodium falciparum malaria, classically presenting in the jaw or abdomen), sporadic (worldwide, usually abdominal, EBV in a minority) and immunodeficiency-associated (HIV). The 2022 WHO classification separates EBV-positive and EBV-negative Burkitt lymphoma. Bone-marrow and CNS involvement define the highest-risk group and are common at presentation.
Treatment is short, dose-intense, CNS-directed multi-agent chemotherapy: the French LMB and German BFM regimens in children (cyclophosphamide, vincristine, prednisone, high-dose methotrexate, cytarabine, etoposide, doxorubicin with intrathecal therapy), CODOX-M/IVAC or dose-adjusted EPOCH-R in adults. The Inter-B-NHL Ritux 2010 trial (NEJM 2020) showed that adding rituximab to LMB chemotherapy in high-risk children and adolescents improved event-free survival, making rituximab part of paediatric standard care. Tumour lysis syndrome at treatment start is a major hazard and rasburicase, hydration and a low-intensity pre-phase are integral to the protocols. Relapse is uncommon but very hard to treat; CD19 CAR-T and bispecific antibodies are being explored.
| Setting | Approach | Guideline |
|---|---|---|
| Children and adolescents, all stages | Risk-stratified LMB or BFM regimen with intrathecal therapy; rituximab added for high-risk (stage III with high LDH, stage IV, leukaemic) disease per Inter-B-NHL Ritux 2010; low-intensity pre-phase and tumour lysis prophylaxis. | not mapped |
| Adults | Dose-adjusted EPOCH-R (lower toxicity, effective in older and HIV-positive patients) or CODOX-M/IVAC-R or hyper-CVAD-R; CNS prophylaxis in all. | NCCN Category 2A |
| Resource-limited settings | Cyclophosphamide-based or modified LMB regimens with intrathecal therapy, adding rituximab where available; investment in supportive care (transfusion, antimicrobials, tumour lysis management) is the main lever. | not mapped |
| Relapsed or refractory | No standard; salvage chemotherapy with autologous or allogeneic transplant in responders, CD19 CAR-T and bispecific antibodies in trials. | not mapped |
| Burkitt lymphoma in adults: which intensive regimen, and the pre-phase that prevents tumour lysis | The fastest-growing human tumour, with a doubling time measured in hours, and curable in the large majority when treated immediately with an intensive multi-agent regimen that includes central nervous system-directed treatment. R-CHOP is not adequate and should not be used. Three accepted regimens. Risk-adapted dose-adjusted EPOCH-R, tested in 113 adults across 22 centres: low-risk patients received three cycles with no central nervous system prophylaxis and high-risk patients six cycles with intrathecal prophylaxis; event-free survival was 84.5 per cent and overall survival 87.0 per cent at a median 58.7 months, with event-free survival of 100 per cent in the low-risk group and 82.1 per cent in the high-risk group. It worked equally well regardless of age, HIV status and IPI group, and five patients (4 per cent) died of treatment. CODOX-M/IVAC and hyper-CVAD with high-dose methotrexate and cytarabine are the older, more intensive inpatient alternatives, used particularly where there is central nervous system involvement, for which dose-adjusted EPOCH-R performed least well. Before the first full dose: a pre-phase of low-dose cyclophosphamide and prednisolone for about a week to shrink the tumour gradually, intravenous fluids, allopurinol or rasburicase, and electrolyte monitoring every six to eight hours. Tumour lysis syndrome, not the lymphoma, is what kills people in the first week. | not mapped |
| Burkitt lymphoma with HIV, and in countries where the endemic form is common | HIV does not change the regimen. In the 113-adult dose-adjusted EPOCH-R study a quarter of patients were HIV positive and did as well as the rest; antiretroviral therapy is continued through chemotherapy, with attention to interactions, and co-trimoxazole prophylaxis is given. Rituximab is used in HIV-associated Burkitt lymphoma provided the CD4 count is not very low. The endemic form, driven by Epstein-Barr virus and malaria and presenting as a jaw or abdominal mass in children in equatorial Africa, is treated with regimens designed for what a unit can actually deliver: reduced-intensity cyclophosphamide-based protocols with intrathecal therapy, given where blood products, dialysis and intensive care may not be available. Cure rates in those settings are lower than in high-income countries, and the limiting factors are late presentation, abandonment of treatment and supportive care rather than the drugs. Where rituximab can be obtained, adding it to chemotherapy improves outcomes in high-risk paediatric mature B-cell lymphoma. | not mapped |
| Burkitt lymphoma that relapses | Relapse is uncommon, occurs early and is difficult to treat; it is the reason the first regimen must be the right one. Options are a non-cross-resistant salvage regimen such as R-ICE or R-GDP followed by autologous or allogeneic transplant in those who respond, CD19 CAR-T, which has activity but is less well established here than in diffuse large B-cell lymphoma, and a clinical trial. Central nervous system involvement at relapse requires high-dose methotrexate or cytarabine with intrathecal therapy. Early and explicit discussion of what is realistic belongs in this conversation, alongside the offer of a trial. | not mapped |