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dc.contributor.authorSolenthaler, Men_US
dc.contributor.authorMatutes, Een_US
dc.contributor.authorBrito-Babapulle, Ven_US
dc.contributor.authorMorilla, Ren_US
dc.contributor.authorCatovsky, Den_US
dc.identifier.citationHAEMATOLOGICA, 2002, 87 pp. 1141 - 1150en_US
dc.description.abstractBackground and Objectives. In mantle cell lymphoma (MCL), abnormalities additional to t(11;14) including those affecting genes involved in the p53 pathway, are important for disease-development and progression. This study aimed to assess the frequency, relationship and impact of p53 abnormalities and those of its inhibitor mdm2 in blastoid and non-blastoid MCL in leukemic phase. Design and Methods. Isolated blood lymphocytes from 21 patients with MCL in leukemic phase, characterized by the presence of t(11;14), were analyzed by flow cytometry and by fluorescent in situ hybridization in order to investigate whether there is a correlation between overexpression and deletion of p53, overexpression of mdm2 and gain of chromosome 12. Results were also correlated with morphologic subtypes, proliferative activity assessed by expression of Ki67 and,clinical outcome. Results. Cells from 2/21 (10%) and 7/21 (33%) patients overexpressed p53 and mdm2, respectively. No single case expressed both proteins. Ten out of 19 (53%) patients had a hemizygous loss of 17p (p53) including the 2 patients (11%) overexpressing p53.,Gains of chromosome 12 (mdm2) were found in only 2 cases with expression of mdm2 in one of them. Overall, p53 deletion and/or overexpression of mdm2 was found in 71% of cases. Ten of 19 patients had-a blastoid MCL, including all 5 patients who were W positive, 6 of the 7 patients expressing mdm2 and one of the 2 patients expressing p53. There was no correlation between p53 deletion and morphologic subtypes. All patients with blastoid MCL have died after 6 median time of 25 months. Interpretation and Conclusions. In MCL in leukemic phase there is a high frequency of p53 deletion and/or overexpression of mdm2. In contrast, overexpression of p53 is relatively rare. Overexpression of mdm2 is seen predominantly in blastoid MCL, the latter being characterized by a short median survival, and seems unrelated to a numerical gain of chromosome 12. It does not reflect a high proliferative rate but might indicate an alternative mechanism of inactivating p53 in prognostically adverse types of MCL (C) 2002, Ferrata Storti Foundation.en_US
dc.format.extent1141 - 1150en_US
dc.titlep53 and mdm2 in mantle cell lymphoma in leukemic phaseen_US
dc.typeJournal Article
rioxxterms.typeJournal Article/Reviewen_US
pubs.notesaffiliation: Solenthaler, M (Reprint Author), Univ Hosp Bern, Inselspital, Cent Hematol Lab, CH-3010 Bern, Switzerland. Univ Hosp Bern, Inselspital, Cent Hematol Lab, CH-3010 Bern, Switzerland. Inst Canc Res, Acad Dept Hematol Cytogenet, London SW3 6JB, England. Royal Marsden NHS Trust, London, England. keywords: p53; mdm2; mantle cell lymphoma; flow cytometry; 17p deletion keywords-plus: NON-HODGKINS-LYMPHOMAS; TUMOR-SUPPRESSOR GENE; POOR-PROGNOSIS; HEMATOLOGICAL MALIGNANCIES; AGGRESSIVE VARIANTS; BONE-MARROW; MUTATIONS; EXPRESSION; OVEREXPRESSION; ABNORMALITIES research-areas: Hematology web-of-science-categories: Hematology number-of-cited-references: 41 times-cited: 20 usage-count-last-180-days: 0 usage-count-since-2013: 0 journal-iso: Haematologica doc-delivery-number: 616KD unique-id: ISI:000179302200008 oa: gold da: 2018-09-17en_US
pubs.notesNot knownen_US
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Molecular Haematology (including Cytogenetics Group and Cell Markers)
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Molecular Pathology
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Molecular Pathology/Molecular Haematology (including Cytogenetics Group and Cell Markers)
pubs.embargo.termsNot knownen_US
icr.researchteamMolecular Haematology (including Cytogenetics Group and Cell Markers)en_US
dc.contributor.icrauthorMatutes, Estellaen_US

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