October 2002, Volume 16, Number 10, Pages 2055-2061 Table of contents Previous Abstract Next Full text PDF Original Manuscript Partial deletions of long arm of chromosome 6: biologic and clinical implications in adult acute lymphoblastic leukemia M Mancini1, M L Vegna1, G L Castoldi2, C Mecucci3, F Spirito1, L Elia1, A Tafuri1, L Annino1, F Pane4, G Rege-Cambrin5, M Gottardi6, P Leoni7, E Gallo8, A Camera4, L Luciano4, G Specchia9, G Torelli10, M Sborgia11, A Gabbas12, A Tedeschi13, I Della Starza1, N Cascavilla14, F Di Raimondo15, F Mandelli1 and R Foà1 1Department of Cellular Biotechnologies and Hematology, University 'La Sapienza', Rome, Italy 2Department of Biomedical Sciences, Hematology Unit, University of Ferrara, Italy 3Hematology, University of Perugia, Italy 4Hematology, Department of Biochemistry and Medical Biotechnologies, University 'Federico II', Naples, Italy 5Department of Clinical and Biological Sciences, University of Turin, 'SL Gonzaga' Hospital, Orbassano-Turin, Turin, Italy 6Hematology Section, Department of Clinical and Experimental Medicine, University of Verona, Italy 7Department of Hematology, Ancona University School of Medicine, Ancona, Italy 8Division of Hematology, 'S Giovanni Battista' Hospital, Turin, Italy 9Department of Hematology, University of Bari, Bari, Italy 10Department of Medical Sciences, Section of Hematology, University of Modena and Reggio Emilia, Modena, Italy 11Division of Hematology, Civil Hospital, Pescara, Italy 12Division of Hematology, 'S Francesco' Hospital, Nuoro, Italy 13Division of Hematology, 'Niguarda Cà Granda' Hospital, Milan, Italy 14Department of Hematology, IRCCS  'Casa Sollievo Della Sofferenza' Hospital, San Giovanni Rotondo, Italy 15Chair of Hematology, University of Catania, Catania, Italy Correspondence to: M Mancini, Department of Cellular Biotechnologies and Hematology, University 'La Sapienza', Via Benevento 6, 00161 Rome Italy; Fax: 39 06 85795537 Abstract Within 285 adult acute lymphoblastic leukemias (ALL) included in the multicenter GIMEMA 0496 trial and prospectively studied by conventional cytogenetics, 18 cases (6%) with long arm deletion of chromosome 6 (6q) were identified. These cases were divided into: (i) del(6q) only (n = 6); (ii) del(6q) plus other numerical andor structural abnormalities (n = 8); (iii) del(6q) and other 'specific' translocations (n = 4). The biologic and clinical features of the patients carrying this anomaly, as well as their outcome, were compared with those of 267 patients without del(6q). A T cell phenotype was more frequently associated with del(6q) cases in general (P = 0.001) and particularly with cases presenting del(6q) as the isolated abnormality (P = 0.0027). No significant difference with respect to multidrug resistance (MDR)P glycoprotein expression was observed between the two groups of patients (21% vs 28% of MDR-positive cases, respectively). A BCR-ABL fusion transcript was less frequently detected in cases with del(6q) (11%) compared with those without the anomaly (29%). p15 and p16 deletions were identified by Southern blot analysis in 21% of cases with del(6q) and in 26% of cases without del(6q). In this latter group, a T cell phenotype was less frequently associated with p15 andor p16 deletion than in the group carrying del(6q) (36% vs 100% of cases, P = 0.011). Overall, patients with ALL and del(6q) had a high complete remission (CR) rate (83%); however, they had a lower 18 month event-free survival (31% vs 41%) and a higher relapse rate (70% vs 37%, P = 0.02) compared with patients without del(6q). To date, this is the largest series of adult ALL cases reported with del(6q) homogeneously treated, which have also been prospectively studied for MDR expression and for the detection of known fusion genes. This anomaly, as an isolated change, identifies a subset of cases with hyperleukocytosis (median WBC count 52  109l) and a strict correlation with a T cell phenotype. Overall, del(6q) seems to be associated with an unfavorable clinical outcome, although this finding will need to be confirmed by extended FISH analysis. Leukemia (2002) 16, 20552061. doi:10.1038/sj.leu.2402640

Partial deletion of long arm of chromosome 6: biologic and clinical implications in adult acute lymphoblastic leukemi / M., Mancini; Castoldi, M. L. V. e. g. n. a. G. L.; C., Mecucci; F., Spirito; F., Elia; A., Tafuri; L., Annino; F., Pane; G., Rege Cambrin; M., Gottardi; Leoni, Pietro; E., Gallo; A., Camera; L., Luciano; G., Specchia; G., Torelli; M., Sborgia; A., Gabbas; A., Tedeschi; I., Della Starza; N., Cascavilla; F., Di Raimondo; F., Mandelli; R., Foa. - In: LEUKEMIA. - ISSN 0887-6924. - STAMPA. - 2002:(2002), pp. 2055-2061.

Partial deletion of long arm of chromosome 6: biologic and clinical implications in adult acute lymphoblastic leukemi

LEONI, Pietro;
2002-01-01

Abstract

October 2002, Volume 16, Number 10, Pages 2055-2061 Table of contents Previous Abstract Next Full text PDF Original Manuscript Partial deletions of long arm of chromosome 6: biologic and clinical implications in adult acute lymphoblastic leukemia M Mancini1, M L Vegna1, G L Castoldi2, C Mecucci3, F Spirito1, L Elia1, A Tafuri1, L Annino1, F Pane4, G Rege-Cambrin5, M Gottardi6, P Leoni7, E Gallo8, A Camera4, L Luciano4, G Specchia9, G Torelli10, M Sborgia11, A Gabbas12, A Tedeschi13, I Della Starza1, N Cascavilla14, F Di Raimondo15, F Mandelli1 and R Foà1 1Department of Cellular Biotechnologies and Hematology, University 'La Sapienza', Rome, Italy 2Department of Biomedical Sciences, Hematology Unit, University of Ferrara, Italy 3Hematology, University of Perugia, Italy 4Hematology, Department of Biochemistry and Medical Biotechnologies, University 'Federico II', Naples, Italy 5Department of Clinical and Biological Sciences, University of Turin, 'SL Gonzaga' Hospital, Orbassano-Turin, Turin, Italy 6Hematology Section, Department of Clinical and Experimental Medicine, University of Verona, Italy 7Department of Hematology, Ancona University School of Medicine, Ancona, Italy 8Division of Hematology, 'S Giovanni Battista' Hospital, Turin, Italy 9Department of Hematology, University of Bari, Bari, Italy 10Department of Medical Sciences, Section of Hematology, University of Modena and Reggio Emilia, Modena, Italy 11Division of Hematology, Civil Hospital, Pescara, Italy 12Division of Hematology, 'S Francesco' Hospital, Nuoro, Italy 13Division of Hematology, 'Niguarda Cà Granda' Hospital, Milan, Italy 14Department of Hematology, IRCCS  'Casa Sollievo Della Sofferenza' Hospital, San Giovanni Rotondo, Italy 15Chair of Hematology, University of Catania, Catania, Italy Correspondence to: M Mancini, Department of Cellular Biotechnologies and Hematology, University 'La Sapienza', Via Benevento 6, 00161 Rome Italy; Fax: 39 06 85795537 Abstract Within 285 adult acute lymphoblastic leukemias (ALL) included in the multicenter GIMEMA 0496 trial and prospectively studied by conventional cytogenetics, 18 cases (6%) with long arm deletion of chromosome 6 (6q) were identified. These cases were divided into: (i) del(6q) only (n = 6); (ii) del(6q) plus other numerical andor structural abnormalities (n = 8); (iii) del(6q) and other 'specific' translocations (n = 4). The biologic and clinical features of the patients carrying this anomaly, as well as their outcome, were compared with those of 267 patients without del(6q). A T cell phenotype was more frequently associated with del(6q) cases in general (P = 0.001) and particularly with cases presenting del(6q) as the isolated abnormality (P = 0.0027). No significant difference with respect to multidrug resistance (MDR)P glycoprotein expression was observed between the two groups of patients (21% vs 28% of MDR-positive cases, respectively). A BCR-ABL fusion transcript was less frequently detected in cases with del(6q) (11%) compared with those without the anomaly (29%). p15 and p16 deletions were identified by Southern blot analysis in 21% of cases with del(6q) and in 26% of cases without del(6q). In this latter group, a T cell phenotype was less frequently associated with p15 andor p16 deletion than in the group carrying del(6q) (36% vs 100% of cases, P = 0.011). Overall, patients with ALL and del(6q) had a high complete remission (CR) rate (83%); however, they had a lower 18 month event-free survival (31% vs 41%) and a higher relapse rate (70% vs 37%, P = 0.02) compared with patients without del(6q). To date, this is the largest series of adult ALL cases reported with del(6q) homogeneously treated, which have also been prospectively studied for MDR expression and for the detection of known fusion genes. This anomaly, as an isolated change, identifies a subset of cases with hyperleukocytosis (median WBC count 52  109l) and a strict correlation with a T cell phenotype. Overall, del(6q) seems to be associated with an unfavorable clinical outcome, although this finding will need to be confirmed by extended FISH analysis. Leukemia (2002) 16, 20552061. doi:10.1038/sj.leu.2402640
2002
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11566/73663
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