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Burkitt's lymphoma (BL)

Written2001-03Antonio Cuneo, Gianluigi Castoldi
Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9687/3 Burkitt lymphoma
Atlas_Id 2077

Clinics and Pathology

Phenotype / cell stem origin Pan-B antigens positive; TdT-, CD10+; CD5-; sIgM+. The cell of origin is a peripheral IgM+ memory B-cell (presence of somatic hypermutation of the Ig gene)
Epidemiology Most common in children (1/3 of lymphomas). In adult it accounts for 3-4% of all lymphomas in western countries and it is frequently associated with immunodeficiency
Clinics
  • There is an endemic variant, affecting africans, which primarily involves the jaws and other facial bones.
  • The non-endemic variant may be associated with immunodeficiency states and usually presents with abdominal involvement (distal ileum, ciecum, mesentery). The disease is very aggressive and requires prompt treatment with appropriate regimens.
  • Cytology The blast cells in the peripheral blood and bone marrow display a basophilic cytoplasm with characteristic vacuolization, a picture indisinguishable from acute lymphoblastic leukemia (ALL) L3 of the FAB classification, which represents the leukemic counterpart of BL.
    Pathology
  • The lymphoma consists of a monomorphic infiltrate of the lymph node by medium-sized cells showing round nuclei with several nucleoli and basophilic cytoplasm. Numerous benign macrophages confer a histologic pattern referred to as "starry sky". Involvement of the peripheral blood and bone marrow may occur.
  • The related form 'Burkitt-lik' lymphoma shows intermediate features between diffuse large cell lymphoma and BL and probably includes different disease entities. It was suggested by the WHO panel that only those cases with c-MYC rearrangement and/or a >99% proliferation fraction as demonstrated by Ki-67 positivity should be classified as Burkitt-like lymphoma
  • Treatment Aggressive regimens specifically designed for this lymphoma must be used
    Evolution Very rapid if untreated. Patients with limited disease and favourable prognostic features at presentation may rapidly show disease dissemination
    Prognosis If treated promptly with appropriate regimens the majority of patients can be cured

    Cytogenetics

    Cytogenetics Morphological
  • The primary chromosome anomaly is the translocation t(8;14)(q24;q32), found in 60-70% of the cases. Variant translocations having in common an 8q24 break, i.e the t(8;22)(q24;q11) and t(2;8)(p12;q24) occur in approximately 10-15% and 2-5% of the cases, respectively. A minority of cases may carry a duplication of chromosome 1, involving the 1q21-25 segment as the only detectable chromosome lesion
  • In the Burkitt-like form there are at least 3 cytogenetic categories: one with an 8q24/c-MYC translocation, one with an 8q24 and 18q21/ BCL2 translocation and another with "miscellaneous" rearrangements, frequently including an 18q21 break
  • Additional anomalies Recurrent chromosome aberrations associated with the 8q24 translocations include 1q21-25 duplications, deletions of 6q11-14, 17p deletions and trisomy 12, +7, +8 and +18.

    Genes involved and Proteins

    Note
  • The t(8,14) and the variant t(8;22) and t(2;8) juxtapose IgH sequences and the c-MYC oncogene, bringing about its constitutional expression.
  • The 17p deletion may have a correlation with p53 loss of function, determined by deletion of one allele and inactivating mutation of the remaining allele
  • Result of the chromosomal anomaly

    Fusion Protein
    Oncogenesis Constitutive expression of c-MYC is crucial for the pathogenesis of BL, this protein being a key transcriptional regulator, controlling cell proliferation, differentiation and death. The deregulated expression of c-MYC, caused by the 8q24 translocations, is achieved through multiple mechanisms: a) juxtaposition to regulatory elements of the Ig loci, b) mutations in the c-MYC 5' regulatory regions and, c) aminoacid substitutions occurring in exon 2, making the c-MYC transactivation domain less susceptible to modulation
      

    Bibliography

    Molecular genetics of malignant lymphoma. In: Fo R (ed): Reviews in clinical and experimental hematology.
    Gaidano G
    Forum Service Editore, Genova and Martin Dunitz, London,. 1997.
     
    World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997.
    Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 1999 ; 17 (12) : 3835-3849.
    PMID 10577857
     
    Chromosomal abnormalities in adult non-endemic Burkitt's lymphoma and leukemia: 22 new reports and a review of 148 cases from the literature.
    Kornblau SM, Goodacre A, Cabanillas F
    Hematological oncology. 1991 ; 9 (2) : 63-78.
    PMID 1869243
     
    Small noncleaved, non-Burkitt's (Burkit-Like) lymphoma: cytogenetics predict outcome and reflect clinical presentation.
    Macpherson N, Lesack D, Klasa R, Horsman D, Connors JM, Barnett M, Gascoyne RD
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 1999 ; 17 (5) : 1558-1567.
    PMID 10334544
     
    Small noncleaved cell lymphomas (Burkitt's and Burkitt-like lymphomas).
    Magrath I
    In. : page M.
     
    Catalogue of chromosome aberrations in cancer (5th edition).
    Mitelmam F ed
    Wiley Liss, New York. 1994.
     
    High frequency of EBV association with non-random abnormalities of the chromosome region 1q21-25 in AIDS-related Burkitt's lymphoma-derived cell lines.
    Polito P, Cilia AM, Gloghini A, Cozzi M, Perin T, De Paoli P, Gaidano G, Carbone A
    International journal of cancer. Journal international du cancer. 1995 ; 61 (3) : 370-374.
    PMID 7729949
     
    Clinicopathogenetic significance of chromosomal abnormalities in patients with blastic peripheral B-cell lymphoma. Kiel-Wien-Lymphoma Study Group.
    Schlegelberger B, Zwingers T, Harder L, Nowotny H, Siebert R, Vesely M, Bartels H, Sonnen R, Hopfinger G, Nader A, Ott G, Müller-Hermelink K, Feller A, Heinz R
    Blood. 1999 ; 94 (9) : 3114-3120.
    PMID 10556197
     
    Application of interphase fluorescence in situ Hybridization for the detection of the Burkitt translocation t(8;14)(q24;q32) in B-cell lymphomas.
    Siebert R, Matthiesen P, Harder S, Zhang Y, Borowski A, Zühlke-Jenisch R, Metzke S, Joos S, Weber-Matthiesen K, Grote W, Schlegelberger B
    Blood. 1998 ; 91 (3) : 984-990.
    PMID 9446660
     

    Citation

    This paper should be referenced as such :
    Cuneo, A ; Castoldi, GL
    Burkitt's lymphoma (BL)
    Atlas Genet Cytogenet Oncol Haematol. 2001;5(2):121-122.
    Free journal version : [ pdf ]   [ DOI ]
    On line version : http://AtlasGeneticsOncology.org/Anomalies/BurkittID2077.html


    Other genes implicated (Data extracted from papers in the Atlas) [ 25 ]

    Genes BACH2 BCL7B BCLAF1 BIRC2 CALR CDKN1A CXCR3 HTRA2 IGL ING1
    MAPK6 MIR150 MME MYBL1 NCR2 TP53 PTPN6 RBL2 LMO2 RHOA
    SOX11 TCL1B TP53BP2 TPD52 TRAP1

    External links

    COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
    arrayMapTopo ( C42) Morph ( 9687/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
     
     
    Other databaseBurkitt lymphomas&subsettype=CLINICALGROUP Lymphomas: Burkitt lymphomas (Clinical group) - ArrayMap (UHZ-SIB)
    Disease databaseBurkitt's lymphoma (BL)
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed
    All articlesautomatic search in PubMed


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    indexed on : Fri Jun 30 11:22:05 CEST 2017


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