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Burkitt lymphoma(BL)

MedGen UID:
2377
Concept ID:
C0006413
Neoplastic Process
Synonyms: BL; Burkitt's lymphoma; Familial Burkitt's lymphoma
SNOMED CT: Burkitt's tumor (118617000); Burkitt's type malignant lymphoma - undifferentiated (118617000); Burkitt's lymphoma - disorder (118617000); Burkitt's type malignant lymphoma - small non-cleaved (118617000); BL - Burkitt's lymphoma (118617000); Burkitt's lymphoma (77381001); Malignant lymphoma, small noncleaved, Burkitt's, diffuse (77381001); Burkitt's tumor (77381001); Burkitt's lymphoma (clinical) (118617000); Burkitt lymphoma/leukemia (397400006); Burkitt lymphoma (77381001); Burkitt's lymphoma (118617000); Burkitt lymphoma (118617000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Gene (location): MYC (8q24.21)
 
HPO: HP:0030080
Monarch Initiative: MONDO:0007243
OMIM®: 113970
Orphanet: ORPHA543

Definition

Burkitt lymphoma is a rare, aggressive B-cell lymphoma that accounts for 30 to 50% of lymphomas in children but only 1 to 2% of lymphomas in adults (Harris and Horning, 2006). It results from chromosomal translocations that involve the MYC gene (190080) and either the lambda or the kappa light chain immunoglobulin genes (147220, 147200). Burkitt lymphoma is causally related to the Epstein-Barr virus (EBV), although the pathogenetic mechanisms are not clear. [from OMIM]

Clinical features

From HPO
Burkitt lymphoma
MedGen UID:
2377
Concept ID:
C0006413
Neoplastic Process
Burkitt lymphoma is a rare, aggressive B-cell lymphoma that accounts for 30 to 50% of lymphomas in children but only 1 to 2% of lymphomas in adults (Harris and Horning, 2006). It results from chromosomal translocations that involve the MYC gene (190080) and either the lambda or the kappa light chain immunoglobulin genes (147220, 147200). Burkitt lymphoma is causally related to the Epstein-Barr virus (EBV), although the pathogenetic mechanisms are not clear.
Immunodeficiency
MedGen UID:
7034
Concept ID:
C0021051
Disease or Syndrome
Failure of the immune system to protect the body adequately from infection, due to the absence or insufficiency of some component process or substance.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBurkitt lymphoma
Follow this link to review classifications for Burkitt lymphoma in Orphanet.

Conditions with this feature

Burkitt lymphoma
MedGen UID:
2377
Concept ID:
C0006413
Neoplastic Process
Burkitt lymphoma is a rare, aggressive B-cell lymphoma that accounts for 30 to 50% of lymphomas in children but only 1 to 2% of lymphomas in adults (Harris and Horning, 2006). It results from chromosomal translocations that involve the MYC gene (190080) and either the lambda or the kappa light chain immunoglobulin genes (147220, 147200). Burkitt lymphoma is causally related to the Epstein-Barr virus (EBV), although the pathogenetic mechanisms are not clear.
Epidermodysplasia verruciformis, susceptibility to, 4
MedGen UID:
1648396
Concept ID:
C4749042
Finding
Immunodeficiency-129 (IMD129) is an autosomal recessive immunologic disorder characterized by recurrent bacterial, viral, and fungal infections beginning in the first or second decades of life. Some affected individuals have increased susceptibility to certain human papillomaviruses (HPV), resulting in warts and skin lesions that do not respond to treatment, as well as opportunistic infections. Immunologic studies usually show decreased CD4+ T cells, increased effector memory T cells, and decreased naive T cells, suggesting a defect in T-cell development. Some individuals may also have decreased NK cells and B cells, as well as hypogammaglobulinemia. The phenotype is variable (Crequer et al., 2012; Zhou et al., 2024).
X-linked lymphoproliferative disease due to SH2D1A deficiency
MedGen UID:
1770239
Concept ID:
C5399825
Disease or Syndrome
X-linked lymphoproliferative disease (XLP) in general is characterized by an inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis, dysgammaglobulinemia, and lymphoproliferative disease (malignant lymphoma). The condition primarily affects males. XLP has two recognizable subtypes, XLP1 (due to pathogenic variants in SH2D1A) and XLP2 (due to pathogenic variants in XIAP). HLH / fulminant infectious mononucleosis is the most common presentation regardless of subtype. HLH is characterized as an acute illness with prolonged and high fever, bi- or trilineage cytopenias, and hepatosplenomegaly, which is often severe or fatal. Death is generally secondary to liver failure or multisystem organ dysfunction. In those with XLP1, dys- or hypogammaglobulinemia can lead to varying degrees of humoral immune dysfunction associated with bronchiectasis and recurrent respiratory infections that, if untreated, may result in death. Lymphoproliferative disease (malignant lymphoma) and other lymphoproliferative diseases are specific to XLP1 and often develop in childhood, usually following EBV exposure. Rarer findings in those with XLP1 can include aplastic anemia, vasculitis, and lymphoid granulomatosis. Males with XLP2 are more likely to have HLH without EBV infection, recurrent episodes of HLH (which is not typically seen in those with XLP1), splenomegaly, and gastrointestinal disease, including enterocolitis and perirectal abscesses or fistulae. Rarely, individuals with XLP2 and inflammatory bowel disease have been reported to develop inflammatory liver disease, which can progress to fatal liver failure. Transient hypogammaglobulinemia has been rarely observed in those with XLP2. To date, neither lymphoproliferative disease nor common variable immunodeficiency has been reported in males with XLP2. Heterozygous females rarely have symptoms. There are, however, increasing numbers of reports of affected females with unfavorable (skewed) X-chromosome inactivation favoring the X chromosome with the pathogenic variant who develop HLH, inflammatory bowel disease, and erythema nodosum.
Joint contractures, osteochondromas, and B-cell lymphoma
MedGen UID:
1824078
Concept ID:
C5774305
Disease or Syndrome
Joint contractures, osteochondromas, and B-cell lymphoma (JCOSL) is an autosomal recessive systemic disorder characterized by the development of painless fixed contractures of the joints in early childhood. There is evidence of abnormal chondrocyte homeostasis, resulting in contractures, osteopenia, and the development of osteochondromas. Laboratory studies show abnormal levels and function of B- and T-cell subsets, and patients can develop B-cell lymphomas or malignancies. Despite the abnormalities in immunologic cells, immunodeficiency is not a feature of the disease, suggesting that it can be classified as a 'primary immune regulatory disorder' (Sharma et al., 2022).

Professional guidelines

PubMed

Crombie J, LaCasce A
Blood 2021 Feb 11;137(6):743-750. doi: 10.1182/blood.2019004099. PMID: 33171490
Moleti ML, Testi AM, Foà R
Br J Haematol 2020 Jun;189(5):826-843. Epub 2020 Mar 6 doi: 10.1111/bjh.16461. PMID: 32141616
Ferry JA
Oncologist 2006 Apr;11(4):375-83. doi: 10.1634/theoncologist.11-4-375. PMID: 16614233

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Aggressive Mature B-Cell Lymphomas, 2024

Recent clinical studies

Etiology

Atallah-Yunes SA, Murphy DJ, Noy A
Lancet Haematol 2020 Aug;7(8):e594-e600. doi: 10.1016/S2352-3026(20)30126-5. PMID: 32735838
Rodrigues-Fernandes CI, Pérez-de-Oliveira ME, Aristizabal Arboleda LP, Fonseca FP, Lopes MA, Vargas PA, Santos-Silva AR
Int J Pediatr Otorhinolaryngol 2020 Jul;134:110033. Epub 2020 Apr 5 doi: 10.1016/j.ijporl.2020.110033. PMID: 32302884
Dunleavy K, Little RF, Wilson WH
Hematol Oncol Clin North Am 2016 Dec;30(6):1333-1343. doi: 10.1016/j.hoc.2016.07.009. PMID: 27888884
Matar CG, Jacobs NT, Speck SH, Lamb TJ, Moormann AM
Parasite Immunol 2015 Sep;37(9):433-45. doi: 10.1111/pim.12212. PMID: 26121587Free PMC Article
Molyneux EM, Rochford R, Griffin B, Newton R, Jackson G, Menon G, Harrison CJ, Israels T, Bailey S
Lancet 2012 Mar 31;379(9822):1234-44. Epub 2012 Feb 13 doi: 10.1016/S0140-6736(11)61177-X. PMID: 22333947

Diagnosis

Eche-Ugwu IJ
Clin J Oncol Nurs 2023 Sep 15;27(5):571-574. doi: 10.1188/23.CJON.571-574. PMID: 37729460
Roschewski M, Staudt LM, Wilson WH
N Engl J Med 2022 Sep 22;387(12):1111-1122. doi: 10.1056/NEJMra2025746. PMID: 36129999
Ferry JA
Oncologist 2006 Apr;11(4):375-83. doi: 10.1634/theoncologist.11-4-375. PMID: 16614233
Shapira J, Peylan-Ramu N
Oral Oncol 1998 Jan;34(1):15-23. doi: 10.1016/s1368-8375(97)00041-9. PMID: 9659515
Ziegler JL
N Engl J Med 1981 Sep 24;305(13):735-45. doi: 10.1056/NEJM198109243051305. PMID: 7022215

Therapy

Crombie J, LaCasce A
Blood 2021 Feb 11;137(6):743-750. doi: 10.1182/blood.2019004099. PMID: 33171490
Grommes C, Rubenstein JL, DeAngelis LM, Ferreri AJM, Batchelor TT
Neuro Oncol 2019 Feb 19;21(3):296-305. doi: 10.1093/neuonc/noy192. PMID: 30418592Free PMC Article
Lacasce A, Howard O, Lib S, Fisher D, Weng A, Neuberg D, Shipp M
Leuk Lymphoma 2004 Apr;45(4):761-7. doi: 10.1080/1042819031000141301. PMID: 15160953
Ziegler JL
N Engl J Med 1981 Sep 24;305(13):735-45. doi: 10.1056/NEJM198109243051305. PMID: 7022215
Ziegler JL
Med Clin North Am 1977 Sep;61(5):1073-82. doi: 10.1016/s0025-7125(16)31282-2. PMID: 197352

Prognosis

Johnston WT, Erdmann F, Newton R, Steliarova-Foucher E, Schüz J, Roman E
Cancer Epidemiol 2021 Apr;71(Pt B):101662. Epub 2020 Jan 8 doi: 10.1016/j.canep.2019.101662. PMID: 31924557
Atallah-Yunes SA, Murphy DJ, Noy A
Lancet Haematol 2020 Aug;7(8):e594-e600. doi: 10.1016/S2352-3026(20)30126-5. PMID: 32735838
Roschewski M, Dunleavy K, Abramson JS, Powell BL, Link BK, Patel P, Bierman PJ, Jagadeesh D, Mitsuyasu RT, Peace D, Watson PR, Hanna WT, Melani C, Lucas AN, Steinberg SM, Pittaluga S, Jaffe ES, Friedberg JW, Kahl BS, Little RF, Bartlett NL, Fanale MA, Noy A, Wilson WH
J Clin Oncol 2020 Aug 1;38(22):2519-2529. Epub 2020 May 26 doi: 10.1200/JCO.20.00303. PMID: 32453640Free PMC Article
Hoffmann J, Ohgami RS
Semin Diagn Pathol 2017 Sep;34(5):462-469. Epub 2017 May 28 doi: 10.1053/j.semdp.2017.05.007. PMID: 28662998
Molyneux EM, Rochford R, Griffin B, Newton R, Jackson G, Menon G, Harrison CJ, Israels T, Bailey S
Lancet 2012 Mar 31;379(9822):1234-44. Epub 2012 Feb 13 doi: 10.1016/S0140-6736(11)61177-X. PMID: 22333947

Clinical prediction guides

Rodrigues-Fernandes CI, Pérez-de-Oliveira ME, Aristizabal Arboleda LP, Fonseca FP, Lopes MA, Vargas PA, Santos-Silva AR
Int J Pediatr Otorhinolaryngol 2020 Jul;134:110033. Epub 2020 Apr 5 doi: 10.1016/j.ijporl.2020.110033. PMID: 32302884
Hoffmann J, Ohgami RS
Semin Diagn Pathol 2017 Sep;34(5):462-469. Epub 2017 May 28 doi: 10.1053/j.semdp.2017.05.007. PMID: 28662998
Vockerodt M, Yap LF, Shannon-Lowe C, Curley H, Wei W, Vrzalikova K, Murray PG
J Pathol 2015 Jan;235(2):312-22. doi: 10.1002/path.4459. PMID: 25294567
Lossos IS, Gascoyne RD
Best Pract Res Clin Haematol 2011 Jun;24(2):147-63. Epub 2011 May 6 doi: 10.1016/j.beha.2011.02.006. PMID: 21658615Free PMC Article
Evens AM, Gordon LI
Curr Treat Options Oncol 2002 Aug;3(4):291-305. doi: 10.1007/s11864-002-0029-9. PMID: 12074766

Recent systematic reviews

Kotepui KU, Kotepui M
Int J Environ Res Public Health 2021 May 30;18(11) doi: 10.3390/ijerph18115886. PMID: 34070881Free PMC Article
Della Rocca AM, Leonart LP, Ferreira VL, Tonin FS, Steffenello-Durigon G, Del Moral JAG, Fernandez-Llimos F, Pontarolo R
Clin Lymphoma Myeloma Leuk 2021 Aug;21(8):514-525. Epub 2021 Apr 3 doi: 10.1016/j.clml.2021.03.012. PMID: 33947633
Saleh K, Michot JM, Camara-Clayette V, Vassetsky Y, Ribrag V
Curr Oncol Rep 2020 Mar 6;22(4):33. doi: 10.1007/s11912-020-0898-8. PMID: 32144513
Hong X, Khalife S, Bouhabel S, Bernard C, Daniel SJ, Manoukian JJ, Nguyen LHP
Int J Pediatr Otorhinolaryngol 2019 Jun;121:127-136. Epub 2019 Mar 13 doi: 10.1016/j.ijporl.2019.03.013. PMID: 30897372
Nie M, Wang Y, Bi XW, Xia Y, Sun P, Liu PP, Li ZM, Jiang WQ
Ann Hematol 2016 Jan;95(1):19-26. Epub 2015 Oct 1 doi: 10.1007/s00277-015-2501-1. PMID: 26423805

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    Clinical resources

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    • PubMed
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024
    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Aggressive Mature B-Cell Lymphomas, 2024

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