Clinical and biological significance of isolated Y chromosome loss in myelodysplastic syndromes and chronic myelomonocytic leukemia. A report from the Spanish MDS Group
Por:
Nomdedeu, M, Pereira, A, Calvo, X, Colomer, J, Sole, F, Arias, A, Gomez, C, Luno, E, Cervera, J, Arnan, M, Pomares, H, Ramos, F, Oiartzabal, I, Espinet, B, Pedro, C, Arrizabalaga, B, Blanco, ML, Tormo, M, Hernandez-Rivas, JM, Diez-Campelo, M, Ortega, M, Valcarcel, D, Cedena, MT, Collado, R, Grau, J, Granada, I, Sanz, G, Campo, E, Esteve, J, Costa, D
Publicada:
1 dic 2017
Resumen:
Isolate loss of chromosome Y (-Y) in myelodysplastic syndromes (MDS) is associated to a better outcome but it is also well described as an age-related phenomenon. In this study we aimed to analyze the prognostic impact of -Y in the context of the IPSS-R cytogenetic classification, evaluate the clinical significance of the percentage of metaphases with isolated -Y, and test whether finding -Y may predispose to over-diagnose MDS in patients with borderline morphological features. We evaluated 3581 male patients from the Spanish MDS Registry with a diagnosis of MDS or chronic myelomonocytic leukemia (CMML). -Y was identified in 177 patients (4.9%). Compared with the 2246 male patients with normal karyotype, -Y group showed a reduced risk of leukemic transformation that did not translate into a survival advantage. The overall survival and the risk of leukemic transformation were not influenced by the percentage of metaphases with -Y. The -Y group was not enriched in patients with minor morphologic traits of dysplasia, suggesting that the better outcome in the -Y group cannot be explained by enrichment in cases misdiagnosed as MDS. In conclusion, our results support the current recommendation of classifying patients with -Y within the very good risk category of the IPSS-R for MDS and rule out a selection bias as a possible explanation of this better outcome. An analysis of the molecular basis of MDS with isolated -Y would be of interest as it may provide a biological basis of protection against progression to acute leukemia.
Filiaciones:
Nomdedeu, M:
Hosp Plato, Plato 21, Barcelona 08006, Spain
Fundacio Clin Recerca Biomed, Barcelona, Spain
Pereira, A:
Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Calvo, X:
Hosp del Mar, Barcelona, Spain
Colomer, J:
Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Sole, F:
Josep Carreras Leukemia Res Inst, Campus Badalona, Badalona, Spain
Arias, A:
Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Gomez, C:
Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Luno, E:
Hosp Cent Asturias, Oviedo, Spain
Cervera, J:
Hosp Univ & Politecn La Fe, CIBERONC, Valencia, Spain
Arnan, M:
ICO Hosp Duran & Reynals, Lhospitalet De Llobregat, Spain
Pomares, H:
ICO Hosp Duran & Reynals, Lhospitalet De Llobregat, Spain
Ramos, F:
Hosp Univ Leon, Leon, Spain
Oiartzabal, I:
Hosp Univ Araba, Vitoria, Spain
Espinet, B:
Hosp del Mar, Barcelona, Spain
Pedro, C:
Hosp del Mar, Barcelona, Spain
Arrizabalaga, B:
Hosp Univ Cruces, Bilbao, Spain
Blanco, ML:
Hosp Santa Creu & Sant Pau, Barcelona, Spain
Tormo, M:
Univ Valencia, Hosp Clin, Valencia, Spain
Hernandez-Rivas, JM:
Univ Salamanca, Hosp Univ Salamanca, CSIC, Ctr Invest Canc,CIBERONC,IBSAL,IBMCC, Salamanca, Spain
Diez-Campelo, M:
Univ Salamanca, Hosp Univ Salamanca, CSIC, Ctr Invest Canc,CIBERONC,IBSAL,IBMCC, Salamanca, Spain
Ortega, M:
Hosp Univ Vall Hebron, Barcelona, Spain
Valcarcel, D:
Hosp Univ Vall Hebron, Barcelona, Spain
Cedena, MT:
Hosp 12 Octubre, Madrid, Spain
Collado, R:
Hosp Gen Univ Valencia, Valencia, Spain
Grau, J:
ICO Hosp Univ Germans Trias & Pujol, Josep Carreras Leukaemia Res Inst, Badalona, Spain
Granada, I:
ICO Hosp Univ Germans Trias & Pujol, Josep Carreras Leukaemia Res Inst, Badalona, Spain
Sanz, G:
Hosp Univ & Politecn La Fe, CIBERONC, Valencia, Spain
Campo, E:
Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Esteve, J:
Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Costa, D:
Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
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