Lymphoplasmacytic lymphoma

2000-05-01   Gian Luigi Castoldi , Antonio Cuneo 

1.Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

Clinics and Pathology

Disease

lymphoplasmacytic lymphoma (LPL) includes most cases of Waldenstroms 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 stem cell 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))
  • Bibliography

    Pubmed IDLast YearTitleAuthors
    80689361994A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group.Harris NL et al
    13847921992t(9;14)(p13;q32) denotes a subset of low-grade non-Hodgkin's lymphoma with plasmacytoid differentiation.Offit K et al
    103193861999Genetics of small lymphocyte disorders.Panayiotidis P et al

    Citation

    Gian Luigi Castoldi ; Antonio Cuneo

    Lymphoplasmacytic lymphoma

    Atlas Genet Cytogenet Oncol Haematol. 2000-05-01

    Online version: http://atlasgeneticsoncology.org/haematological/2074/lymphoplasmacytic-lymphoma

    External Links