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Lymphoplasmacytic lymphoma

Identity

ICD-Topo C420,C421,C424

Clinics and Pathology

Disease lymphoplasmacytic lymphoma (LPL) includes most cases of Waldenstrom's macroglobulinemia and consists of a diffuse proliferation of small lymphocytes, plasmacytoid lymphocytes and plasma cells; the typical histologic features of small lymphocytic lymphoma, mantle cell lymphoma and marginal zone lymphoma must be absent
Phenotype / cell stem origin B-cell antigens are positive; whereas CD5 and, usually, CD23 test negative; the cells express surface and cytoplasmic IgM and are IgD-. CD10 is negative, whereas a proportion of cases have faint CD25 and CD11c expression; the postulated normal counterpart is a B-lymphocyte differentiating into an IgM-secreting plasma cell

Cytogenetics

Note the number of cases studied is small and the interpretation of data must take into account possible variability of histologic classification
Cytogenetics Morphological
  • the t(9;14)(p13;q32), fusing the IgH gene with the BSAP (B-cell specific activator protein), also called PAX5, was found in 50% of the cases in a study; this balanced translocation cannot be viewed as specific for this lymphoma, having been reported in rare marginal zone lymphomas and large cell lymphomas.
  • other recurrent anomalies include 14q32 translocations with 8q24 ( t(8;14)(q24;q32)), 18q21 ( t(14;18)(q32;q21)), trisomies of chromosomes 3 ( +3) and 12 ( +12) and an isochromosome 6p ( i(6p))
  • Other genes implicated (Data extracted from papers in the Atlas)

    Genes IGH

    Translocations implicated (Data extracted from papers in the Atlas)

    Bibliography

    t(9;14)(p13;q32) denotes a subset of low-grade non-Hodgkin's lymphoma with plasmacytoid differentiation.
    Offit K, Parsa NZ, Filippa D, Jhanwar SC, Chaganti RS
    Blood. 1992 ; 80 (10) : 2594-2599.
    PMID 1384792
     
    A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group.
    Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC
    Blood. 1994 ; 84 (5) : 1361-1392.
    PMID 8068936
     
    Genetics of small lymphocyte disorders.
    Panayiotidis P, Kotsi P
    Seminars in hematology. 1999 ; 36 (2) : 171-177.
    PMID 10319386
     
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed

    Contributor(s)

    Written05-2000Antonio Cuneo, Gian Luigi Castoldi

    Citation

    This paper should be referenced as such :
    Cuneo, A ; Castoldi, GL
    Lymphoplasmacytic lymphoma
    Atlas Genet Cytogenet Oncol Haematol. 2000;4(2):85-85.
    Free online version   Free pdf version   [Bibliographic record ]
    URL : http://AtlasGeneticsOncology.org/Anomalies/LPLID2074.html

    © Atlas of Genetics and Cytogenetics in Oncology and Haematology
    indexed on : Thu Sep 25 15:34:48 CEST 2014


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