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Mantle cell lymphoma (incomplete)

Written1998-05Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9673/3 Mantle cell lymphoma
Atlas_Id 2062

Clinics and Pathology

Disease NHL of the low to intermediate grade
Phenotype / cell stem origin B-cell lineage
Epidemiology 5% of NHL; sex ratio: 3M/1F; median age: 65 yrs
Clinics advanced disease (Ann Harbor stage III-IV) with generalized lymphadenopathies in 90%, bone marrow involvement (70%), frequent splenomegaly (50%), hepatomegaly (30%), and gastro-intestinal involvement (20%), and lymphocytosis (30%); elevated LDH in 50%
 
Leukemic phase of mantle cell lymphoma. Peripheral blood. Small to medium sized lymphoid cells with slightly to markedly irregular nuclear contour. The nuclei have moderately dispersed chromatin bur inconspicuous nucleoli. From a patient with 46,XX,t(11;14)(q13;q32) - Text and iconography Courtesy Georges Flandrin 2005.
Pathology small cleaved cell B-lymphocytes with inconspicuous nucleoli and pale cytoplasm; with a pan B-cell, CD5+, CD10-, CD23- (in contrast with CLL) phenotype; diffuse, nodular and blastoid types, the latter having large cells and higher mitotic count
Treatment according to the age and the disease stage
Prognosis median survival is 3 to 4 yrs; less than 2 yrs in the case of a leukaemic form (blastoid); in contrast with the good prognosis of the MALT lymphoma, a closely related disease.

Cytogenetics

Cytogenetics Morphological
  • knowledge is still scarce, and data complex
  • t(11;14)(q13;q32) is found in 50-70% of cases, but this translocation may also, at a much lesser frequency, be found in other diseases; the genes involved in this translocation are described below; t(11;14) is found in complex karyotypes
  • other frequent findings: deletion of parts of :1p, 6q 9p, 11q (in particular 11q22-23), 13q; gains of parts of: 3q, 8q, 15q; frequent markers; tetraploidy may be found, especially in the blastoid subtype
  • Cytogenetics Molecular CGH (compatrative genomic hybridization) may disclose important events in this disease, taken into account that the t(11;14) may be insufficient for oncogenesis; furthermore, this and other molecular cytogenetic techniques may come in addition to the usual techniques to "dissect" complex karyotypes

    Genes involved and Proteins

    Gene Name CCND1
    Location 11q13
    Dna / Rna 5 exons
    Protein encodes the cyclin D1; role in the cell cycle control: G1 progression and G1/S transition
    Gene Name IGH
    Location 14q32

    Result of the chromosomal anomaly

    Hybrid gene
    Description 5' BCL1 translocated on chromosome 14 near JH (junctions genes of IgH) and C in 3'; the breakpoint in BCL1 is in MTC (major translocation cluster), centromeric to the gene (in 5'), in 80% of cases, or dispersed in mTC1, 2, or 3 in 5'of the gene or in the 3' untranslated region of exon 5.
      
    Fusion Protein
    Description no fusion protein, but promoter exchange; the immunoglobulin gene enhancer stimulates the expression of BCL1.
    Oncogenesis overexpression of BCL1 accelerates passage through the G1 phase
      

    Bibliography

    Mantle cell lymphoma: a clinicopathologic study of 80 cases.
    Argatoff LH, Connors JM, Klasa RJ, Horsman DE, Gascoyne RD
    Blood. 1997 ; 89 (6) : 2067-2078.
    PMID 9058729
     
    Mantle cell lymphoma: presenting features, response to therapy, and prognostic factors.
    Bosch F, López-Guillermo A, Campo E, Ribera JM, Conde E, Piris MA, Vallespí T, Woessner S, Montserrat E
    Cancer. 1998 ; 82 (3) : 567-575.
    PMID 9452276
     
    Cytogenetics of lymphomas: a brief review of its theoretical and practical significance.
    Donner LR
    Cancer genetics and cytogenetics. 1997 ; 94 (1) : 20-26.
    PMID 9078287
     
    Non-Hodgkin's lymphoma.
    Maloney DG
    Current opinion in hematology. 1995 ; 2 (4) : 255-261.
    PMID 9372005
     
    Mantle cell lymphoma: diagnostic criteria, clinical aspects and therapeutic problems.
    Meusers P, Hense J, Brittinger G
    Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1997 ; 11 Suppl 2 : S60-S64.
    PMID 9178843
     
    Gain of 3q and deletion of 11q22 are frequent aberrations in mantle cell lymphoma.
    Monni O, Oinonen R, Elonen E, Franssila K, Teerenhovi L, Joensuu H, Knuutila S
    Genes, chromosomes & cancer. 1998 ; 21 (4) : 298-307.
    PMID 9559341
     
    Blastoid variants of mantle cell lymphoma: frequent bcl-1 rearrangements at the major translocation cluster region and tetraploid chromosome clones.
    Ott G, Kalla J, Ott MM, Schryen B, Katzenberger T, Müller JG, Müller-Hermelink HK
    Blood. 1997 ; 89 (4) : 1421-1429.
    PMID 9028966
     
    Rearrangement and overexpression of the BCL-1/PRAD-1 gene in intermediate lymphocytic lymphomas and in t(11q13)-bearing leukemias.
    Rimokh R, Berger F, Delsol G, Charrin C, Berthéas MF, Ffrench M, Garoscio M, Felman P, Coiffier B, Bryon PA
    Blood. 1993 ; 81 (11) : 3063-3067.
    PMID 8499640
     

    Citation

    This paper should be referenced as such :
    Huret, JL
    Mantle cell lymphoma
    Atlas Genet Cytogenet Oncol Haematol. 1998;2(4):127-128.
    Free journal version : [ pdf ]   [ DOI ]
    On line version : http://AtlasGeneticsOncology.org/Anomalies/mantle2062ID2062.html


    Other genes implicated (Data extracted from papers in the Atlas) [ 32 ]

    Genes ADAM10 AKT1 ATM BAK1 CCND1 CCND1 CCND1 BCL2L11 BCL2L14 BCLAF1
    BIRC2 BMI1 CCR1 CD74 CDC6 CPM CXCR3 DBN1 EIF4E EPHA3
    EZH2 FCER2 MTOR GLI2 IGH ING1 MERTK PASD1 RAN SOX11
    SYK TPD52

    External links

    COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
    arrayMapTopo ( C42) Morph ( 9673/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
     
     
    Disease databaseMantle cell lymphoma (incomplete)
    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 11:22:31 CEST 2017


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