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Leuk Res. 2017 Dec;63:85-89. doi: 10.1016/j.leukres.2017.10.011. Epub 2017 Oct 28.

Clinical and biological significance of isolated Y chromosome loss in myelodysplastic syndromes and chronic myelomonocytic leukemia. A report from the Spanish MDS Group.

Author information

1
Hospital Plató, Barcelona, Spain; Fundació Clínic per la Recerca Biomèdica, Barcelona, Spain. Electronic address: Meritxell.nomdedeu@gmail.com.
2
Hospital Clinic-IDIBAPS, Barcelona, Spain.
3
Hospital del Mar, Barcelona, Spain.
4
Josep Carreras Leukemia Research Institute, Campus Badalona, Spain.
5
Hospital Central de Asturias, Oviedo. Spain.
6
Hospital Universitari i Politècnic La Fe, CIBERONC, Valencia, Spain.
7
ICO-Hospital Duran i Reynals, Hospitalet de Llobregat, Spain.
8
Hospital Universitario de León, Spain.
9
Hospital Universitario Araba, Vitoria-Gasteiz, Spain.
10
Hospital Universitario Cruces, Bilbao, Spain.
11
Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
12
Hospital Clínico Universitario de Valencia, Spain.
13
Hospital Universitario de Salamanca, CIBERONC, IBSAL, IBMCC, Centro de Investigación del Cáncer Universidad de Salamanca-CSIC, Salamanca, Spain.
14
Hospital Universitari Vall d'Hebron, Barcelona, Spain.
15
Hospital 12 de Octubre, Madrid, Spain.
16
Hospital General Universitario de Valencia, Spain.
17
ICO-Hospital Universitari Germans Trias i Pujol, Josep Carreras Leukaemia Research Institute, Badalona, Spain.

Abstract

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.

KEYWORDS:

IPSS-R; Karyotype; Loss of chromosome Y; MDS; Prognosis

PMID:
29121539
DOI:
10.1016/j.leukres.2017.10.011
[Indexed for MEDLINE]

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