Atlas of Genetics and Cytogenetics in Oncology and Haematology

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t(14;19)(q32;q13)

Identity

Note The t(14;19)(q32.3;q13.2) is a rare but recurrent translocation found in patients with B-cell malignancies, mainly in chronic B-cell lymphoproliferative disorders. When occurring in chronic lymphocytic leukaemia (CLL), atypical lymphocyte morphology and immunophenotype have been reported.
 
  t(14;19)(q32;q13) G- banding - Courtesy Jean-Luc Lai (top) and Diane H. Norback, Eric B. Johnson, and Sara Morrison-Delap, UW Cytogenetic Services (middle and bottom)

Clinics and Pathology

Disease chronic lymphocytic leukemia (most are the atypical form). Other diseases (maybe less well defined): low grade B-NHL, mantle cell lymphoma, small non-cleaved cell lymphoma, one case of biphenotypic (B/M) acute leukemia.
Phenotype / cell stem origin chronic B-cell lymphoproliferation
Epidemiology annual incidence 30/106; median age: 60-80 yrs; A high proportion of patients with CLL and t(14;19) are aged lessthan 40 years.
Clinics often a slow evolutive disease
Prognosis highly variable according to the staging: from staging A: where the survival is not reduced compared to age matched population, to staging C: with a median survival of 2 yrs. t(14;19) is often associated with rapidly progressive disease, and overall prognosis is poor compared to the expected survival in chronic lymphocytic leukemia and low-grade B-cell lymphoma.

Cytogenetics

Cytogenetics Morphological The t(14;19)(q32.3;q13.2) is reciprocal and results in 14q+ and a 19q- derivative chromosomes.
Cytogenetics Molecular FISH is useful for identifying variant translocations.
Additional anomalies t(14;19) is rarely the sole cytogenetic aberration. Trisomy 12 is the most frequent associated abnormality, and is observed in 50% of cases; this may even be underestimated as with FISh more cases with +12 are detected. Other chromosomes involved in structural aberrations are 6, 2 and 10.
Variants Three way variants are relatively frequent, compared to variants in other recurrent translocations. t(14;17;19) and t(7;19;14) were described.

Genes involved and Proteins

Gene Name IgH
Location 14q32
Gene Name BCL3
Location 19q13
Dna / Rna 9 exons, spanning 11.5 kb. BCL3 mRNA is expressed in a variety of tissues, particularly in spleen, liver and lung
Protein
  • encodes the a protein which contains seven ankyrin repeats. Similar repeats are described in the structural protein ankyrin, as well as in proteins involved in cell cycle control and lineage determination (SW14, SW16, lin2).
  • BCL3 is a member of the IkappaB family, whose proteins regulate the NFkappaB family of transcription factors. NFkappaB plays a major role in B-cell development.

    • Result of the chromosomal anomaly

    Hybrid gene
    Description The breakpoint is located in the 5' untranslated region of the BCL3 gene. BCL3 is juxtaposed to the immunoglobulin heavy chain gene locus on chromosome 14 (often in the switch alpha region) in a "head-to-head" configuration.
      
    Fusion Protein
    Oncogenesis
  • no fusion protein. The translocation does not interrupt the transcriptional integrity of BCL3, but is associated with increased production of a BCL3 RNA of normal size. The immunoglobulin enhancer is not present on the same derivative chromosome as BCL3, suggesting other mechanisms for overexpression.
  • The genes affected by overexpression of BCL3 remain to be identified.
    •   

    External links

    Other databaset(14;19)(q32;q13) Mitelman database (CGAP - NCBI)
    Other databaset(14;19)(q32;q13) CancerChromosomes (NCBI)

    Bibliography

    BCL3 rearrangement and t(14;19)(q32;q13) in lymphoproliferative disorders.
    Michaux L, Mecucci C, Stul M, Wlodarska I, Hernandez JM, Meeus P, Michaux JL, Scheiff JM, Noˆ´l H, Louwagie A, Criel A, Boogaerts M, Van Orshoven A, Cassiman JJ, Van Den Berghe H
    Genes, chromosomes & cancer. 1996 ; 15 (1) : 38-47.
    PMID 8824724
     
    t(14;19)/BCL3 rearrangements in lymphoproliferative disorders: a review of 23 cases.
    Michaux L, Dierlamm J, Wlodarska I, Bours V, Van den Berghe H, Hagemeijer A
    Cancer genetics and cytogenetics. 1997 ; 94 (1) : 36-43.
    PMID 9078289
     

    Contributor(s)

    Written08-2001Peter Meeus and Lucienne Michaux
    Centrum voor Menselijke Erfelijkheid, UZ Gasthuisberg, Herestraat 49, 3000 Leuven, Belgie

    Citation

    This paper should be referenced as such :
    Meeus P, Michaux L . t(14;19)(q32;q13). Atlas Genet Cytogenet Oncol Haematol. August 2001 .
    URL : http://AtlasGeneticsOncology.org/Anomalies/t1419ID2050.html

    © Atlas of Genetics and Cytogenetics in Oncology and Haematology
    indexed on : Mon May 12 18:13:12 2008

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