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Título

Bone marrow fibrosis in myelodysplastic syndromes: a prospective evaluation including mutational analysis

AutorRamos, Fernando; Robledo, Cristina; Izquierdo-García, Francisco Miguel; Suárez-Vilela, Dimas; Benito, Rocío; Fuertes, Marta; Insunza, Andrés; Barragán, Eva; Rey, Mónica del; García-Ruiz de Morales, José María; Tormo, Mar; Salido, Eduardo; Zamora, Lurdes; Pedro, Carmen; Sánchez-del-Real, Javier; Díez-Campelo, María; Cañizo, María Consuelo del; Sanz, Guillermo F.; Hernández, Jesús M. CSIC ORCID
Palabras claveBone marrow fibrosis
Pathogenesis
Myelodysplastic syndromes
Prognosis
Next-generation sequencing
Fecha de publicación2016
EditorImpact Journals
CitaciónOncotarget 7(21): 30492-30503 (2016)
ResumenThe biological and molecular events that underlie bone marrow fibrosis in patients with myelodysplastic syndromes are poorly understood, and its prognostic role in the era of the Revised International Prognostic Scoring System (IPSS-R) is not yet fully determined. We have evaluated the clinical and biological events that underlie bone marrow fibrotic changes, as well as its prognostic role, in a well-characterized prospective patient cohort (n=77) of primary MDS patients. The degree of marrow fibrosis was linked to parameters of erythropoietic failure, marrow cellularity, p53 protein accumulation, WT1 gene expression, and serum levels of CXCL9 and CXCL10, but not to other covariates including the IPSS-R score. The presence of bone marrow fibrosis grade 2 or higher was associated with the presence of mutations in cohesin complex genes (31.5% vs. 5.4%, p=0.006). By contrast, mutations in CALR, JAK2, PDGFRA, PDGFRB, and TP53 were very rare. Survival analysis showed that marrow fibrosis grade 2 or higher was a relevant significant predictor for of overall survival, and independent of age, performance status, and IPSS-R score in multivariate analysis.
DescripciónSpanish Group for Myelodysplastic Syndromes (GESMD).
Versión del editorhttps://doi.org/10.18632/oncotarget.9026
URIhttp://hdl.handle.net/10261/168436
DOI10.18632/oncotarget.9026
Identificadoresdoi: 10.18632/oncotarget.9026
e-issn: 1949-2553
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