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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.
The global picture is stark: in sub-Saharan Africa, where most cases occur, cure rates are limited by late presentation, supportive-care capacity and the toxicity of intensive regimens. Cyclophosphamide-based and modified LMB regimens with rituximab (where affordable) have improved outcomes in Malawi, Uganda and elsewhere, and the AfriBL and other consortia are testing risk-adapted, resource-appropriate protocols. Burkitt lymphoma is therefore both a model of curable cancer and a test of whether cures can travel.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
The most common childhood cancer in equatorial Africa and the most common non-Hodgkin lymphoma of children worldwide; rare in adults (NCI PDQ).
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
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.
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.
No standard; salvage chemotherapy with autologous or allogeneic transplant in responders, CD19 CAR-T and bispecific antibodies in trials.
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.
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.
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.
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One cancer page and one trial page on OnCo cite this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing pages listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
The start of viral oncology. Everything in cancer prevention that works by preventing or treating an infection, from hepatitis B vaccination to Helicobacter eradication for gastric MALT lymphoma, descends from the idea this paper established.
The clinical description that set off the search for a cancer virus. It is also an argument for geographic epidemiology: Burkitt found the cause by asking where the disease was, not by looking down a microscope.
Query for this cancer: (TITLE:"Burkitt lymphoma" OR ABSTRACT:"Burkitt lymphoma" OR TITLE:"Burkitt lymphoma/leukaemia" OR ABSTRACT:"Burkitt lymphoma/leukaemia" OR TITLE:"Endemic Burkitt lymphoma" OR ABSTRACT:"Endemic Burkitt lymphoma" OR TITLE:"Sporadic Burkitt lymphoma" OR ABSTRACT:"Sporadic Burkitt lymphoma" OR TITLE:"Immunodeficiency-associated Burkitt lymphoma" OR ABSTRACT:"Immunodeficiency-associated Burkitt lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Burkitt lymphoma, not a curated reading list.
Later maps its distribution to the malaria belt.
Epstein, Achong and Barr identify the first human tumour virus.
Dalla-Favera and Taub show MYC is juxtaposed to the immunoglobulin heavy-chain locus.
French SFOP protocol cures most children including stage IV.
Schmitz and Richter (Nature, Nature Genetics) define the cooperating mutations.
Minard-Colin and colleagues (NEJM 2020).
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Breathing very fast, confusion or slurred speech, blue, pale or blotchy skin, a very high or very low temperature, shivering, or a rash that does not fade when pressed: the NHS says call 999 or go to A and E, and do not drive yourself.
A rising potassium level can disturb the heart rhythm, which is the reason blood is checked frequently during the first cycle. The triage standard sends chest pain or tightness straight to 999 whatever the cause.
Fever of 38 C or higher is grade 1 CRS; fever with low blood pressure, fast heartbeat, breathlessness or low oxygen is grade 2 or higher. The labels carry a boxed warning and say to report fever immediately.
Macmillan lists difficulty passing urine, loss of bladder or bowel control and constipation among the signs of spinal cord compression and says to contact the hospital straight away. If you cannot reach anyone, go to A and E and say you have lymphoma and symptoms of spinal cord compression.
Yellowing during or after antibody treatment for a B-cell lymphoma is a reason to be seen the same day and to have liver blood tests, not to wait for the next cycle.
See all on the product pages:Axicabtagene ciloleucelCentral venous access (port, PICC line)CyclophosphamideCytarabineDoxorubicinEtoposideFebrile neutropeniaHepatitis B reactivation before rituximab and other anti-CD20 antibodiesHypogammaglobulinaemia and infection risk after B-cell therapiesIfosfamideMetastatic spinal cord compression (MSCC)MethotrexateNeutropeniaTumour lysis syndrome (TLS)Tumour lysis syndrome in lymphoma: who is at risk, and rasburicaseVincristine·Printable cards in the navigator
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Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
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