del(13q) in non-Hodgkin's lymphoma

Identity

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-Hodgkin's 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.
	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

Clinics and Pathology

Disease	B-NHL
Phenotype / cell stem 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

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

External links

Other database	del(13q) in non-Hodgkin's lymphoma	Mitelman database (CGAP - NCBI)

Bibliography

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Contributor(s)

Written	11-1999	Antonio Cuneo

Citation

This paper should be referenced as such :

Cuneo A . del(13q) in non-Hodgkin's lymphoma. Atlas Genet Cytogenet Oncol Haematol. November 1999 . URL : http://AtlasGeneticsOncology.org/Genes/del13qNHLID2070.html

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