# Burkitt lymphoma

Source: https://onco.cc/cancers/burkitt-lymphoma/  
OnCo record `burkitt-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-10
- Also known as: Burkitt lymphoma/leukaemia; Endemic Burkitt lymphoma; Sporadic Burkitt lymphoma; Immunodeficiency-associated Burkitt lymphoma
- Tags: nci-coverage; paediatric; haematologic; global-health
- Group: haematologic
- Burden: The most common childhood cancer in equatorial Africa and the most common non-Hodgkin lymphoma of children worldwide; rare in adults (NCI PDQ).
- Subtypes: Endemic (EBV-positive, malaria-associated); Sporadic; Immunodeficiency-associated (HIV); Burkitt leukaemia (more than 25% marrow blasts); High-grade B-cell lymphoma with 11q aberration (Burkitt-like, MYC-negative)
- Biomarkers: 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/burkitt-lymphoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/burkitt-lymphoma/#overview [1 subtype, 4 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/burkitt-lymphoma/#what-it-is [6 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/burkitt-lymphoma/#finding-it [6 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/burkitt-lymphoma/#treating-it [4 settings, 2 regimens, 4 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/burkitt-lymphoma/#evidence [4 trials, 2 key papers, 7 milestones]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/burkitt-lymphoma/#science [25 targets, 3 pathways]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/burkitt-lymphoma/where-you-are/ [2 centres]
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/burkitt-lymphoma/#living-with-it [17 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/burkitt-lymphoma/coming/ [6 medicines, 4 trials, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/burkitt-lymphoma/data/ [64 connected records]

## Standard of care

- 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](https://onco.cc/drugs/rituximab/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Vincristine](https://onco.cc/drugs/vincristine/), [Etoposide](https://onco.cc/drugs/etoposide/), [Inter-B-NHL Ritux 2010](https://onco.cc/trials/inter-b-nhl-ritux-2010/))
- 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](https://onco.cc/drugs/rituximab/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Methotrexate](https://onco.cc/drugs/methotrexate/))
- 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](https://onco.cc/drugs/cyclophosphamide/), [Global oncology and access in low- and middle-income countries](https://onco.cc/technologies/global-oncology-access/))
- 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)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/), [CAR-T cell therapy](https://onco.cc/technologies/car-t/))

## State of the art

- Short intensive chemotherapy cures most children; rituximab added a further step in high-risk disease (Inter-B-NHL Ritux 2010).
- MYC translocation plus a small set of cooperating mutations make Burkitt one of the best-understood lymphomas at the genomic level.
- Dose-adjusted EPOCH-R has made adult and HIV-associated Burkitt lymphoma treatable with far less toxicity.
- The largest gap is geographical: most children with Burkitt lymphoma live where intensive protocols and supportive care are hard to deliver, and adapted regimens are closing the gap.

## Open problems

- Relapsed Burkitt lymphoma is rarely curable; CD19-directed CAR-T and bispecifics are being tested.
- Cure rates in sub-Saharan Africa remain far below high-income countries; adapted protocols, rituximab access and supportive-care investment are the response.
- Acute toxicity (tumour lysis, mucositis, infection) of intensive regimens, particularly in adults and the immunocompromised.
- Late effects of anthracyclines and alkylators in survivors treated as children.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Burkitt_lymphoma
- NCI PDQ: childhood non-Hodgkin lymphoma (Burkitt): https://www.cancer.gov/types/lymphoma/hp/child-nhl-treatment-pdq
- Inter-B-NHL Ritux 2010: rituximab in high-risk paediatric B-NHL (NEJM 2020): https://doi.org/10.1056/NEJMoa1915315
- SIOP PODC adapted treatment guidelines for Burkitt lymphoma in low-income settings: https://doi.org/10.1002/pbc.24407

## Connected records

- cancers: [Acute lymphoblastic leukaemia](https://onco.cc/cancers/all-leukemia/), [Burkitt leukaemia](https://onco.cc/cancers/burkitt-leukaemia/), [Childhood cancers (all types)](https://onco.cc/cancers/childhood-cancers/), [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [HIV-associated (AIDS-related) lymphomas](https://onco.cc/cancers/hiv-associated-lymphoma/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/), [Post-transplant lymphoproliferative disorder (PTLD)](https://onco.cc/cancers/post-transplant-lymphoproliferative-disorder/), [Primary CNS lymphoma](https://onco.cc/cancers/primary-cns-lymphoma/), [Primary mediastinal (thymic) large B-cell lymphoma](https://onco.cc/cancers/primary-mediastinal-b-cell-lymphoma/)
- technologies: [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [CAR-T cell therapy](https://onco.cc/technologies/car-t/), [Cytogenetics and FISH](https://onco.cc/technologies/cytogenetics-fish/), [Cytotoxic chemotherapy](https://onco.cc/technologies/cytotoxic-chemotherapy/), [Global oncology and access in low- and middle-income countries](https://onco.cc/technologies/global-oncology-access/), [Monoclonal antibodies](https://onco.cc/technologies/monoclonal-antibody/)
- targets: [BCL7A](https://onco.cc/targets/bcl7a/), [BMP7](https://onco.cc/targets/bmp7/), [CCND3](https://onco.cc/targets/ccnd3/), [CD19](https://onco.cc/targets/cd19/), [CD20](https://onco.cc/targets/cd20/), [CDKN2C](https://onco.cc/targets/cdkn2c/), [CHD8](https://onco.cc/targets/chd8/), [EIF4A1](https://onco.cc/targets/eif4a1/), [FOXO1](https://onco.cc/targets/foxo1/), [GNA13](https://onco.cc/targets/gna13/), [GNAI2](https://onco.cc/targets/gnai2/), [HNRNPU](https://onco.cc/targets/hnrnpu/), [ID3](https://onco.cc/targets/id3/), [P2RY8](https://onco.cc/targets/p2ry8/), [PCBP1](https://onco.cc/targets/pcbp1/), [PHF6](https://onco.cc/targets/phf6/), [RFX7](https://onco.cc/targets/rfx7/), [RHOA](https://onco.cc/targets/rhoa/), [SIN3A](https://onco.cc/targets/sin3a/), [TCF3](https://onco.cc/targets/tcf3/), [TFAP4](https://onco.cc/targets/tfap4/), [USP7](https://onco.cc/targets/usp7/), [WNK1](https://onco.cc/targets/wnk1/), [Xanthine oxidase (XDH)](https://onco.cc/targets/xdh/)
- drugs: [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [Rituximab](https://onco.cc/drugs/rituximab/), [Vincristine](https://onco.cc/drugs/vincristine/)
- companies: [Children's Oncology Group (COG)](https://onco.cc/companies/childrens-oncology-group/)
- institutions: [SIOP Europe (European Society for Paediatric Oncology)](https://onco.cc/institutions/siop-europe/), [Uganda Cancer Institute](https://onco.cc/institutions/uganda-cancer-institute/)
- pathways: [B-cell receptor / BTK signalling (to NF-κB)](https://onco.cc/pathways/bcr-signalling/), [MYC](https://onco.cc/pathways/myc/), [Oncogenic viruses](https://onco.cc/pathways/oncogenic-viruses/)
- terms: [Epstein-Barr virus (EBV) in cancer](https://onco.cc/terms/ebv-term/), [Lymphoma (tissue type)](https://onco.cc/terms/lymphoma-type/), [Tumour lysis syndrome (TLS)](https://onco.cc/terms/tumor-lysis-syndrome/)
- trials: [Inter-B-NHL Ritux 2010](https://onco.cc/trials/inter-b-nhl-ritux-2010/), [Rituximab, Rasburicase, and Combination Chemotherapy in Treating Young Patients With Newly Diagnosed Advanced B-Cell Leukemia or Lymphoma](https://onco.cc/trials/nct00057811/), [Sepantronium Bromide for the Treatment of High-grade B-cell Lymphoma](https://onco.cc/trials/nct05263583/), [Substudy 01A: Zilovertamab Vedotin in Pediatric and Young Adult Participants With Hematologic Malignancies or Solid Tumors (MK-9999-01A/LIGHTBEAM-U01)](https://onco.cc/trials/nct06395103/)
- bottlenecks: [Most of the world has almost no cancer care](https://onco.cc/bottlenecks/b-global-access/)
- key papers: [Practical recommendations for the management of children with endemic Burkitt lymphoma (BL) in a resource limited setting](https://onco.cc/key-papers/paper-hesseling-pediatr-blood-cancer/), [Rituximab for High-Risk, Mature B-Cell Non-Hodgkin's Lymphoma in Children](https://onco.cc/key-papers/paper-minard-colin-n-engl-j-med/)
- journals: [Tumour virus research](https://onco.cc/journals/tumour-virus-research/)

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JSON: https://onco.cc/api/v1/entities/burkitt-lymphoma.json