Loading
Below, week by week, is what OnCo's record of Burkitt lymphoma says about the first two months: the order is typical, the timing is yours to ask about. 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. Sections appear only where the record has something to say. Orientation, not medical advice.
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
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.
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.
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.
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.
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.
No standard; salvage chemotherapy with autologous or allogeneic transplant in responders, CD19 CAR-T and bispecific antibodies in trials.
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.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
Every term links to the glossary.