Authors
Maria Julia Montoro, Victor Navarro, Pamela Acha, Claudia Haferlach, Onyee Chan, Yasuo Kubota, Felicitas Isabel Schulz, Robert Briski, Najla Al-Ali, Blanca Xicoy, Felix Lopez-Cadenas, Francesc Bosch, Anna Aguilera, Manja Meggendorfer, Lea Naomi Eder, Andrés Jerez, Yu Hung Wang, Alessia Campagna, Luca Lanino, Beate Betz, Valeria Santini, Teresa Bernal Del Castillo, Esperanza Such, Anne Sophie Platzbecker, Tariq Kewan, Carmelo Gurnari, Carla Zindel, Austin G Kulasekararaj, Maria Teresa Voso, Mikkael A Sekeres, Sangeetha Venugopal, Nicolas Diaz Varela, Aref Al-Kali, Uwe Platzbecker, Detlef Thomas Haase, Amer M Zeidan, Pierre Fenaux, María Díez-Campelo, Guillermo Garcia-Manero, Daniel Howard Wiseman, Matteo Giovanni Della Porta, Ulrich Germing, Jaroslaw P Maciejewski, Rami S Komrokji, Francesc Sole, Torsten Haferlach, Laura Palomo, David Valcarcel
Published in
Blood advances. Jul 16, 2026. Epub Jul 16, 2026.
Abstract
Myelodysplastic syndromes with isolated deletion of chromosome 5q [MDS-del(5q)] constitute a distinct biological entity traditionally associated with favorable outcomes, although up to one quarter of patients progress to acute myeloid leukemia (AML). Existing prognostic models, developed in heterogeneous MDS populations, may not adequately capture risk within this subgroup. We assembled an international cohort of 682 patients with MDS-del(5q) to evaluate the performance of the IPSS-R and IPSS-M, identify prognostic variables, and develop a disease-specific prognostic tool, the IPSS-del(5q). Most patients were classified as lower-risk by IPSS-R (94.4%) and IPSS-M (85.5%), yet both systems showed limited discriminatory ability (C-indices ≈0.5). Independent adverse prognostic factors included age ≥70 years, male sex, anemia (hemoglobin ≤10 g/dL), thrombocytopenia (platelets ≤100×10⁹/L), the presence of one additional chromosomal abnormality, ≥2 gene mutations, SF3B1 mutations, and high-risk TP53 status. Six variables were included in the IPSS-del(5q), stratifying patients into standard-risk (74.3%) and high-risk (25.7%) groups with significantly different LFS (69.2 vs. 32.0 months; p<0.01). Moreover, this model reclassified 19.1% of lower-risk IPSS-R and 14.6% of lower-risk IPSS-M patients into the high-risk IPSS-del(5q) group. However, its discriminative power remained modest, with a C-index of 0.60. Overall, this study provides the most comprehensive prognostic evaluation of MDS-del(5q) to date, demonstrates the limited discriminatory capacity of existing MDS scores in this entity, and underscores the need to develop refined disease-specific prognostic approaches for this MDS subtype.
PMID:
42466989
Bibliographic data and abstract were imported from PubMed on 17 Jul 2026.
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