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Burkitt lymphoma(BL)

MedGen UID:
2377
Concept ID:
C0006413
Neoplastic Process
Synonyms: BL; Burkitt's lymphoma; Familial Burkitt's lymphoma
Modes of inheritance:
Sporadic
MedGen UID:
342827
Concept ID:
C1853237
Finding
Sources: HPO, OMIM
Cases of the disease in question occur without a previous family history, i.e., as isolated cases without being transmitted from a parent and without other siblings being affected.
not inherited
MedGen UID:
832438
Concept ID:
CN227390
Intellectual Product
Source: Orphanet
Describes a disorder that is not inherited.
Sporadic (HPO, OMIM)
not inherited (Orphanet)
SNOMED CT: Burkitt's tumor (118617000); [M]Burkitt's tumor (77381001); 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 lymphoma (clinical) (118617000); Burkitt lymphoma/leukemia (397400006); FAB L3 [obs] (22197008); Acute lymphoblastic leukemia, mature B-cell type (22197008); Burkitt cell leukemia (22197008); Malignant lymphoma, undifferentiated, Burkitt type [obs] (77381001); Burkitt's tumor [obs] (77381001); Burkitt tumor [obs] (77381001); Burkitt lymphoma (77381001); Burkitt's lymphoma (118617000); Burkitt lymphoma (118617000); Burkitt's cell leukemia (22197008); Acute lymphoblastic leukemia, Burkitt's type (22197008); Burkitt's leukemia (277571004)
 
Gene (location): MYC (8q24.21)
OMIM®: 113970
HPO: HP:0030080
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

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.

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.

Recent clinical studies

Etiology

Chen BJ, Chang ST, Weng SF, Huang WT, Chu PY, Hsieh PP, Jung YC, Kuo CC, Chuang YT, Chuang SS
Leuk Lymphoma 2016;57(3):644-53. Epub 2015 Sep 30 doi: 10.3109/10428194.2015.1076928. PMID: 26422188
De Falco G, Ambrosio MR, Fuligni F, Onnis A, Bellan C, Rocca BJ, Navari M, Etebari M, Mundo L, Gazaneo S, Facchetti F, Pileri SA, Leoncini L, Piccaluga PP
BMC Cancer 2015 Oct 9;15:668. doi: 10.1186/s12885-015-1661-7. PMID: 26453442Free PMC Article
Aukema SM, Theil L, Rohde M, Bauer B, Bradtke J, Burkhardt B, Bonn BR, Claviez A, Gattenlöhner S, Makarova O, Nagel I, Oschlies I, Pott C, Szczepanowski M, Traulsen A, Kluin PM, Klapper W, Siebert R, Murga Penas EM
Br J Haematol 2015 Sep;170(6):814-25. Epub 2015 Jun 24 doi: 10.1111/bjh.13501. PMID: 26104998
Casulo C, Friedberg J
Curr Hematol Malig Rep 2015 Sep;10(3):266-71. doi: 10.1007/s11899-015-0263-4. PMID: 26013028
Kojima Y, Hagiwara S, Uehira T, Ajisawa A, Kitanaka A, Tanuma J, Okada S, Nagai H
Jpn J Clin Oncol 2014 Apr;44(4):318-23. Epub 2014 Feb 20 doi: 10.1093/jjco/hyu012. PMID: 24558129

Diagnosis

Meznarich J, Miles R, Paxton CN, Afify Z
Pediatr Blood Cancer 2016 May;63(5):938-40. Epub 2016 Jan 19 doi: 10.1002/pbc.25907. PMID: 26785246
Aukema SM, Theil L, Rohde M, Bauer B, Bradtke J, Burkhardt B, Bonn BR, Claviez A, Gattenlöhner S, Makarova O, Nagel I, Oschlies I, Pott C, Szczepanowski M, Traulsen A, Kluin PM, Klapper W, Siebert R, Murga Penas EM
Br J Haematol 2015 Sep;170(6):814-25. Epub 2015 Jun 24 doi: 10.1111/bjh.13501. PMID: 26104998
Casulo C, Friedberg J
Curr Hematol Malig Rep 2015 Sep;10(3):266-71. doi: 10.1007/s11899-015-0263-4. PMID: 26013028
Li JN, Gao LM, Wang WY, Chen M, Li GD, Liu WP, Zhang WY
Int J Clin Exp Pathol 2014 Sep 15;7(10):7049-53. PMID: 25400794Free PMC Article
Kojima Y, Hagiwara S, Uehira T, Ajisawa A, Kitanaka A, Tanuma J, Okada S, Nagai H
Jpn J Clin Oncol 2014 Apr;44(4):318-23. Epub 2014 Feb 20 doi: 10.1093/jjco/hyu012. PMID: 24558129

Therapy

Chaari Z, Charfi S, Hentati A, Ayadi I, Abid H, Frikha I
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1110-2. Epub 2015 Jun 1 doi: 10.1177/0218492315587817. PMID: 26038605
Casulo C, Friedberg J
Curr Hematol Malig Rep 2015 Sep;10(3):266-71. doi: 10.1007/s11899-015-0263-4. PMID: 26013028
Li ZJ, Yao C, Liu SF, Chen L, Xi YM, Zhang W, Zhang GS
Biomed Res Int 2014;2014:391512. Epub 2014 May 7 doi: 10.1155/2014/391512. PMID: 24895574Free PMC Article
Akar Özkan E, Özdemir BH, Akdur A, Deniz EE, Haberal M
Exp Clin Transplant 2014 Mar;12 Suppl 1:136-8. PMID: 24635811
Kojima Y, Hagiwara S, Uehira T, Ajisawa A, Kitanaka A, Tanuma J, Okada S, Nagai H
Jpn J Clin Oncol 2014 Apr;44(4):318-23. Epub 2014 Feb 20 doi: 10.1093/jjco/hyu012. PMID: 24558129

Prognosis

Chen BJ, Chang ST, Weng SF, Huang WT, Chu PY, Hsieh PP, Jung YC, Kuo CC, Chuang YT, Chuang SS
Leuk Lymphoma 2016;57(3):644-53. Epub 2015 Sep 30 doi: 10.3109/10428194.2015.1076928. PMID: 26422188
Aukema SM, Theil L, Rohde M, Bauer B, Bradtke J, Burkhardt B, Bonn BR, Claviez A, Gattenlöhner S, Makarova O, Nagel I, Oschlies I, Pott C, Szczepanowski M, Traulsen A, Kluin PM, Klapper W, Siebert R, Murga Penas EM
Br J Haematol 2015 Sep;170(6):814-25. Epub 2015 Jun 24 doi: 10.1111/bjh.13501. PMID: 26104998
Chaari Z, Charfi S, Hentati A, Ayadi I, Abid H, Frikha I
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1110-2. Epub 2015 Jun 1 doi: 10.1177/0218492315587817. PMID: 26038605
Casulo C, Friedberg J
Curr Hematol Malig Rep 2015 Sep;10(3):266-71. doi: 10.1007/s11899-015-0263-4. PMID: 26013028
Li JN, Gao LM, Wang WY, Chen M, Li GD, Liu WP, Zhang WY
Int J Clin Exp Pathol 2014 Sep 15;7(10):7049-53. PMID: 25400794Free PMC Article

Clinical prediction guides

Wowro SJ, Schmitt KR, Tong G, Berger F, Schubert S
Int Immunopharmacol 2016 Jan;30:9-17. Epub 2015 Nov 21 doi: 10.1016/j.intimp.2015.11.020. PMID: 26613512
De Falco G, Ambrosio MR, Fuligni F, Onnis A, Bellan C, Rocca BJ, Navari M, Etebari M, Mundo L, Gazaneo S, Facchetti F, Pileri SA, Leoncini L, Piccaluga PP
BMC Cancer 2015 Oct 9;15:668. doi: 10.1186/s12885-015-1661-7. PMID: 26453442Free PMC Article
Chaari Z, Charfi S, Hentati A, Ayadi I, Abid H, Frikha I
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1110-2. Epub 2015 Jun 1 doi: 10.1177/0218492315587817. PMID: 26038605
Li JN, Gao LM, Wang WY, Chen M, Li GD, Liu WP, Zhang WY
Int J Clin Exp Pathol 2014 Sep 15;7(10):7049-53. PMID: 25400794Free PMC Article
Greenough A, Dave SS
Curr Opin Hematol 2014 Jul;21(4):326-32. doi: 10.1097/MOH.0000000000000059. PMID: 24867287

Recent systematic reviews

Masqué-Soler N, Szczepanowski M, Kohler CW, Aukema SM, Nagel I, Richter J, Siebert R, Spang R, Burkhardt B, Klapper W
Br J Haematol 2015 Nov;171(4):501-8. Epub 2015 Jul 27 doi: 10.1111/bjh.13624. PMID: 26218299
Mbulaiteye SM, Morton LM, Sampson JN, Chang ET, Costas L, de Sanjosé S, Lightfoot T, Kelly J, Friedberg JW, Cozen W, Marcos-Gragera R, Slager SL, Birmann BM, Weisenburger DD
J Natl Cancer Inst Monogr 2014 Aug;2014(48):106-14. doi: 10.1093/jncimonographs/lgu003. PMID: 25174031Free PMC Article
Hesseling P, Israels T, Harif M, Chantada G, Molyneux E; Pediatric Oncology in Developing Countries.
Pediatr Blood Cancer 2013 Mar;60(3):357-62. Epub 2012 Nov 28 doi: 10.1002/pbc.24407. PMID: 23192950
Kelly JL, Toothaker SR, Ciminello L, Hoelzer D, Holte H, LaCasce AS, Mead G, Thomas D, Van Imhoff GW, Kahl BS, Cheson BD, Magrath IT, Fisher RI, Friedberg JW
Clin Lymphoma Myeloma 2009 Aug;9(4):307-10. doi: 10.3816/CLM.2009.n.060. PMID: 19717381Free PMC Article
Boerma EG, Siebert R, Kluin PM, Baudis M
Leukemia 2009 Feb;23(2):225-34. Epub 2008 Oct 16 doi: 10.1038/leu.2008.281. PMID: 18923440

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