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

Written1999-11Antonio Cuneo
Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 2070
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

    Bibliography

    Detailed molecular delineation of 13q14.3 loss in B-cell chronic lymphocytic leukemia.
    Corcoran MM, Rasool O, Liu Y, Iyengar A, Grander D, Ibbotson RE, Merup M, Wu X, Brodyansky V, Gardiner AC, Juliusson G, Chapman RM, Ivanova G, Tiller M, Gahrton G, Yankovsky N, Zabarovsky E, Oscier DG, Einhorn S
    Blood. 1998 ; 91 (4) : 1382-1390.
    PMID 9454769
     
    13q14 deletion in non-Hodgkin's lymphoma: correlation with clinicopathologic features.
    Cuneo A, Bigoni R, Rigolin GM, Roberti MG, Bardi A, Campioni D, Minotto C, Agostini P, Milani R, Bullrich F, Negrini M, Croce C, Castoldi G
    Haematologica. 1999 ; 84 (7) : 589-593.
    PMID 10406898
     
    Cytogenetic evolution patterns in non-Hodgkin's lymphoma.
    Johansson B, Mertens F, Mitelman F
    Blood. 1995 ; 86 (10) : 3905-3914.
    PMID 7579360
     
    Molecular delineation of 13q deletion boundaries in 20 patients with myeloid malignancies.
    La Starza R, Wlodarska I, Aventin A, Falzetti D, Crescenzi B, Martelli MF, Van den Berghe H, Mecucci C
    Blood. 1998 ; 91 (1) : 231-237.
    PMID 9414289
     
    13q deletions in lymphoid malignancies.
    Liu Y, Hermanson M, Grandér D, Merup M, Wu X, Heyman M, Rasool O, Juliusson G, Gahrton G, Detlofsson R, Nikiforova N, Buys C, Söderhäll S, Yankovsky N, Zabarovsky E, Einhorn S
    Blood. 1995 ; 86 (5) : 1911-1915.
    PMID 7655020
     
    Expressed sequences as candidates for a novel tumor suppressor gene at band 13q14 in B-cell chronic lymphocytic leukemia and mantle cell lymphoma.
    Stilgenbauer S, Nickolenko J, Wilhelm J, Wolf S, Weitz S, Döhner K, Boehm T, Döhner H, Lichter P
    Oncogene. 1998 ; 16 (14) : 1891-1897.
    PMID 9583687
     
    Poor prognosis in multiple myeloma is associated only with partial or complete deletions of chromosome 13 or abnormalities involving 11q and not with other karyotype abnormalities.
    Tricot G, Barlogie B, Jagannath S, Bracy D, Mattox S, Vesole DH, Naucke S, Sawyer JR
    Blood. 1995 ; 86 (11) : 4250-4256.
    PMID 7492784
     
    Frequent chromosome arm 13q deletion in aggressive non-Hodgkin's lymphoma.
    Wada M, Okamura T, Okada M, Teramura M, Masuda M, Motoji T, Mizoguchi H
    Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1999 ; 13 (5) : 792-798.
    PMID 10374885
     

    Citation

    This paper should be referenced as such :
    Cuneo, A
    del(13q) in non-Hodgkin's lymphoma
    Atlas Genet Cytogenet Oncol Haematol. 1999;3(4):191-192.
    Free journal version : [ pdf ]   [ DOI ]
    On line version : http://AtlasGeneticsOncology.org/Anomalies/del13qNHLID2070.html


    Translocations implicated (Data extracted from papers in the Atlas)

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

    External links

    arrayMap (UZH-SIB Zurich)
    Mitelman databasedel(13q) [Case List]    del(13q) [Association List] Mitelman database (CGAP - NCBI)
    arrayMap[select an item]
     
     
    Disease databasedel(13q) in non-Hodgkin's lymphoma
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed
    All articlesautomatic search in PubMed


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