The Key B.L. Specific Features (1):

• B.L. is the most aggressive lymphoma;
• B.L. was the first human tumor associated with a virus;
• The C-MYC was the first oncogene to be described in a lymphoma;
• B.L. has the shortest cell doubling time among all human cancer;
• B.L. is divided into three clinical variants (W.H.O. classification): sporadic, endemic and immunodeficiency associated. 

Epidemiology:

The epidemiology must be considered separately in each clinical variant, and is different for adults and children:

Sporadic B.L.:

• Rare and predominantly affecting adult men in western countries:
  • 6.0 per 1 million men (3)
  • 2.4 per million women (3)
• 1 to 2 % of the adults lymphomas (1)
• 40 % of the children lymphomas (1)
• The link with Epstein Barr Virus (EBV) is low: 15 to 30% (1).

Endemic B.L. (2) (4):

• Mainly children, usually 4-7 years old, more male than female
• 3 to 6 per 100,000 children (around 50 times more than in western countries)
• 30 to 50% of childhood cancer in equatorial Africa
• EBV found in nearly all cases
• Correlation with geographical area of endemic Malaria

Immunodeficiency Associated B.L. (5) (6):

• Mainly patients with HIV
• B.L. is much more frequent in immunodeficient patients: 0.2% of HIV infected patients(5); 19% of B.L. occurred among HIV infected individuals (6)

Prognosis:

The prognosis is very different between sporadic and endemic, adults and children, and also following the stage of the disease and the age (2) (7) (10).

Sporadic B.L.:

Among the patients treated by the current intensive regimen, the 5 years survival rate is in average:

• Around 80 - 90% for children (3)
• Between 50 - 60% for adults. (3) (7) (11)

But this average for adults is the result of very different outcome following the stage of the disease which defines the type of risk: Low risk around 70%, Low-Intermediate around 55%, High-Intermediate around 40%, High risk around 30%

There is also a big variance following the ages: 20 - 39 around 60%, 40 - 59 around 45%, and more than 60 around 30%.

More recent treatment with Rituximab (8) (10), as well as low intensity therapy (9), has demonstrated some progress.

Endemic B.L.:

Overal survival is low, but almost impossible to approach with the same criteria than sporadic (12) (13).

Immunodeficiency Associated B.L.:

Immunodeficiency patients with H.I.V. have an estimated 5 years survival rate around 50% (5).

The BLGSP is detailed on ocg.cancer.gov

Bibliography

1. Ferry J A.  Burkitt’s lymphoma: Clinicopathologic features and differential diagnosis. The Oncologist. Apr 2006;11(4): 375-383
2. Blum K A, Lozanski G, Byrd J C.   Adult Burkitt leukemia and lymphoma. Blood. Nov 15 2004;104(10): 3009-3020
3. Costa L J, Xavier A C, Wahlquist A E et al.  Trends in survival of patients with Burkitt lymphoma/leukemia in the USA: an analysis of 3,691 cases. Blood. June 13 2013;121(24): 4861-4872
4. Magrath J .  Epidemiology: clues to the pathogenesis of Burkitt lymphoma. British Journal of Haematology. 2012; 156, 744-756
5. Gopal S, Patel M R, Yanick E L et al. Temporal trends in presentation and survival for HIV-associated lymphoma in the antiretroviral therapy era. J Natl Cancer Inst. 2013; 105 (16): 1221-1229
6. Shiels M S, Engels E A, Linet M S et al.  The epidemic of non-Hodgkin lymphoma in the United States: Disentangling the effect of HIV, 1992-2009. Cancer Epidemiology Biomarkers & Prevention. June 2013; 22(6): 1069-78
7. Wasterlid T, Brown P N, Hagberg O et al.  Impact of chemotherapy regimen and rituximab in adult Burkitt lymphoma: a retrospective population-based study from the Nordic Lymphoma Group. Annals of Oncology.2014; 24 (7): 1879-1886.
8. Prica A, Pratzer A, Cheung M C et al.  Rituximab improves overall survival in patients treated with CODOX-M/IVAC for Burkitt lymphoma (BL) and B-cell lymphoma, unclassifiable, with features intermediate between Diiffuse large B-cell lymphoma and BL: a single center experience and review of the literature. Blood. Nov 15 2013; 122(21): 5093-      .
9. Dunleavy K, Pittaluga S, Shovlin M et al.  Low-intensity therapy in adults with Burkitt’s lymphoma. N Engl J Med. 2013; 369: 1915-1925.
10. Hoelzer D, Walewski J, Dohner H et al.  Improved outcome of adult Burkitt lymphoma/leukemia patients with rituximab and intensive chemotherapy: report of a large prospective multicenter trial. Blood. 2014;  124:3870-3879
11. Jacobson C, LaCasce A.  How I treat Burkitt lymphoma in adults. Blood. Nov 6 2014; 124(19): 2913-2920
12. Ngoma T, Adde M, Durosinmi M et al.   Treatment of Burkitt  lymphoma  in equatorial Africa using a simple three-drug  combination followed by a salvage regimen for patients with persistent or recurrent disease.  British Journal of Haematology. 2012; 158: 749–762
13. Mutyaba I, Orem J, Wabinga H et al.  Access to cancer chemotherapy and predictors of early mortality for childhood cancers in Uganda.  Journal of Clinical Oncology. 2013; Vol 31,n°15 supplement: 10070

General Definition

Scientific Definition and Genetic Findings

History of Burkitt Lymphoma

Treatment

There are several major unmet needs in the clinical management of BL that require urgent attention. First, current chemotherapy is ineffective in roughly 30% of cases, and is associated with therapy induced toxicity and death.  Because of the toxicity, patients with BL over the age of 70 are ineligible for this potentially curative therapy.  Second, young individuals exposed to these intensive regimens face the real possibility of secondary malignancies years later.  Finally, children with endemic BL in Africa, in general do not receive these curative chemotherapy regimens because of the lack of hospital support for the management of post-treatment infections, and consequently many will die of their disease.  What is needed, therefore, are new approaches to the therapy of BL that are based on an understanding of its molecular pathogenesis and the regulatory pathways that it utilizes for proliferation and survival.

 

Treatment Paradigms and Principles:

Treatment of Burkitt Lymphoma in Developed Countries:

Challenges and Opportunities in Treating Burkitt Lymphoma in Developing Countries