del(13q) in non-Hodgkins lymphoma

1999-11-01   Antonio Cuneo  

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

Clinics and Pathology

Disease

B-NHL

Phenotype stem cell origin

peripheral B-cells at different stages of differentiation
  • pre germinal centre: small lymphocytic lymphoma (SLL), mantle cell lymphoma (MCL)
  • post-germinal centre: marginal zone B-cell lymphoma (MZBCL) follicle centre cell lymphoma (FCCL), diffuse large cell lymphoma (DLCL)

    • Epidemiology

    Incidence
  • SLL: 5-10% of all NHL diagnosed by surgical biospy
  • MCL: 5-10% of all NHL in western countries
  • MZBCL: 0-15% of NHL, including the extra-nodal form the nodal and the splenic form
  • FCCL: 30-40% of NHL
  • DLCL: 30-40% of NHL

    • Clinics

  • SLL: low-grade histology, usually running an indolent course; survival largely dependent on clinical stage at presentation
  • MCL: intermediate-grade histology, poor response to therapy, median survival 3-4 years
  • MZBCL: low-grade histology, indolent disease, median survival >5 years
  • FCCL: low-grade histology, indolent disease, median survival > 5 years
  • DLCL: high grade histology, aggressive disease, survival influenced by age, stage at presentation, performance status

    • Prognosis

    the significance of 13q- is uncertain because of heterogeneity of patients population and histology; a low CR rate was described but it is not clear whether this depends on its close association with MCL

    Cytogenetics

    Atlas Image

    Additional anomalies

    with the notable exception of SLL/CLL the 13q deletion is not found as an isolated change in NHL; it was reported as a stemline-associated anomaly in most cases having complex karyotypes, suggesting that it may represent a relatively early event in the cytogenetic history of NHL; the association with other anomalies reflects the incidence of the 13q- chromosome in distinct histologic subsets: thus it was frequently found in karyotypes presenting the t(11;14)(q13;q32); many patients with the inv(14)(q11q32), associated with T-cell lymphoid neoplasias, were found to carry a 13q- chromosome

    Genes Involved and Proteins

    Note
    involved loci: the few characterized cases showed a deletion of the D13S319 marker, located between the Rb locus and the D13S25 marker; FISH studies were performed using probes targeting the Rb locus or the loci comprised between Rb and the D13S25 marker

    Article Bibliography

    Pubmed IDLast YearTitleAuthors

    Summary

    Note

    the chromosome 13q deletion is a relatively common finding in chronic myeloproliferative disorders and lymphoid neoplasias, including B-cell chronic lymphocytic leukemia (CLL), non-Hodgkins lymphoma (NHL) and multiple myeloma (MM). Whereas the commonly deleted region comprise a 100-kb gene-rich segment at the 13q14 chromosome band in CLL, the commonly deleted segment in NHL was not characterized in detail.
    Atlas Image
    del(13)(q14q21) in NHL (G-banding) - Antonio Cuneo; the vertical bar indicates the missing chromosome segment (left); del(13)(q14q33) R- banding (right) - Editor

    Citation

    Antonio Cuneo

    del(13q) in non-Hodgkins lymphoma

    Atlas Genet Cytogenet Oncol Haematol. 1999-11-01

    Online version: http://atlasgeneticsoncology.org/haematological/2070/css/case-report-explorer/welcome