Burkitt lymphoma
Prepared with OnCo (onco.cc/prep/burkitt-lymphoma/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
23 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example MYC rearrangement, t, t) by FISH, EBV status, Ki-67 near 100%, ID3, TCF3, CCND3 mutations, Lactate dehydrogenase and uric acid), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (children and adolescents, all stages), which of the standard options do you recommend and why?
- 6.Am I a candidate for Rituximab, Cyclophosphamide, Methotrexate or related drugs, and what side effects should I expect?
- 7.How do the results of Inter-B-NHL Ritux 2010 apply to someone like me?
- 8.For my situation (adults), which of the standard options do you recommend and why?
- 9.Am I a candidate for Rituximab, Cyclophosphamide, Doxorubicin or related drugs, and what side effects should I expect?
- 10.For my situation (resource-limited settings), which of the standard options do you recommend and why?
- 11.Am I a candidate for Cyclophosphamide, and what side effects should I expect?
- 12.For my situation (relapsed or refractory), which of the standard options do you recommend and why?
- 13.For my situation (burkitt lymphoma in adults: which intensive regimen, and the pre-phase that prevents tumour lysis), which of the standard options do you recommend and why?
- 14.Am I a candidate for Rasburicase, Rituximab, Etoposide or related drugs, and what side effects should I expect?
- 15.For my situation (burkitt lymphoma with hiv, and in countries where the endemic form is common), which of the standard options do you recommend and why?
- 16.Am I a candidate for Rituximab, Cyclophosphamide, and what side effects should I expect?
- 17.For my situation (burkitt lymphoma that relapses), which of the standard options do you recommend and why?
- 18.Am I a candidate for Axicabtagene ciloleucel, Methotrexate, Cytarabine, and what side effects should I expect?
- 19.Are there clinical trials I could join, for example of Inter-B-NHL Ritux 2010, CAR-T cell therapy, Global oncology and access in low- and middle-income countries?
- 20.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 21.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 22.I read that “Relapsed Burkitt lymphoma is rarely curable; CD19-directed CAR-T and bispecifics are being tested”. How does that affect my plan?
- 23.I read that “Cure rates in sub-Saharan Africa remain far below high-income countries; adapted protocols, rituximab access and supportive-care investment are the response”. How does that affect my plan?
The words I may hear
- Tumour lysis syndrome in lymphoma: who is at risk, and rasburicase: When a large, fast-growing lymphoma breaks up quickly, the contents of the cells flood the blood and can stop the kidneys or the heart.
- Double-hit / high-grade B-cell lymphoma: Double-hit lymphoma is a large B-cell lymphoma with rearrangements of two oncogenes (MYC plus BCL2 and/or BCL6), which behaves aggressively and often escapes R-CHOP.
- Epstein-Barr virus latency programmes, and why they decide which lymphoma: Almost everyone carries Epstein-Barr virus for life without harm.
- The germinal centre: why lymphoma starts where antibodies are made: To make a better antibody, a B cell has to deliberately damage its own DNA while dividing fast, with its safety checks switched off.
- CNS prophylaxis in aggressive B-cell lymphoma, and the evidence against it: Some people with aggressive lymphoma are given extra methotrexate, into the spine or into a vein, to stop the lymphoma reaching the brain.
- Lymphoma (tissue type): Cancer of lymphocytes, the white blood cells of the immune system, usually growing as masses in lymph nodes, spleen or other organs.
- Intrathecal therapy (lumbar puncture, Ommaya reservoir): Giving drugs directly into the fluid around the brain and spinal cord, by needle in the lower back or through a small reservoir under the scalp, because most drugs cannot cross from the blood into that space.
- Fertility before lymphoma treatment: what to ask for, and when: Several lymphoma treatments can end fertility, and the chance to preserve it exists only before the first dose.
- Fertility preservation before lymphoma treatment: a decision with a deadline in days: Most of the ways of preserving fertility have to happen before the first dose of chemotherapy, and two of them take about a fortnight.
- Infection prophylaxis in lymphoma: PJP, herpes, fungal risk and vaccination: Several lymphoma treatments knock out the part of the immune system that keeps a particular lung infection, Pneumocystis, at bay.
Tests and results to bring
Biomarker results to ask for: MYC rearrangement (t(8;14), t(2;8), t(8;22)) by FISH, EBV status (EBER), Ki-67 near 100%, ID3, TCF3, CCND3 mutations, Lactate dehydrogenase and uric acid (tumour lysis risk), Bone marrow and cerebrospinal-fluid involvement (stage IV / leukaemic).
Scans and tests linked to this cancer: Comprehensive genomic profiling, Cytogenetics and FISH, Histopathology & immunohistochemistry, Multidisciplinary tumour boards.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- 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. (Tumour lysis syndrome in lymphoma: who is at risk, and rasburicase, Rasburicase, The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest, Rituximab, Etoposide, Doxorubicin, Cyclophosphamide, Vincristine, Prednisone, Methotrexate, Cytarabine, Ifosfamide, Intrathecal therapy (lumbar puncture, Ommaya reservoir), Fertility before lymphoma treatment: what to ask for, and when)
- 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. (Rituximab, Cyclophosphamide, Methotrexate, Doxorubicin, Vincristine, Etoposide, Inter-B-NHL Ritux 2010)
- 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. (Cyclophosphamide, Global oncology and access in low- and middle-income countries)
- 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. (Rituximab, Cyclophosphamide, Doxorubicin, Etoposide, Methotrexate)
- 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. (Practical recommendations for the management of children with endemic Burkitt lymphoma (BL) in a resource limited setting, Rituximab for High-Risk, Mature B-Cell Non-Hodgkin's Lymphoma in Children, Rituximab plus concurrent infusional EPOCH chemotherapy is highly effective in HIV-associated B-cell non-Hodgkin lymphoma, Rituximab, Cyclophosphamide, Intrathecal therapy (lumbar puncture, Ommaya reservoir), Epstein-Barr virus (EBV) in cancer, Tumour lysis syndrome in lymphoma: who is at risk, and rasburicase, Infection prophylaxis in lymphoma: PJP, herpes, fungal risk and vaccination)
- Relapsed or refractory: No standard; salvage chemotherapy with autologous or allogeneic transplant in responders, CD19 CAR-T and bispecific antibodies in trials. (Autologous stem cell transplant (high-dose therapy), Allogeneic stem cell transplantation, CAR-T cell therapy)
- 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. (The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest, Stem cell transplant in lymphoma: what it is still for, Autologous stem cell transplant (high-dose therapy), Allogeneic stem cell transplantation, CAR-T cell therapy, Axicabtagene ciloleucel, Methotrexate, Cytarabine, Early integrated palliative care)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.