Vollständiger Abstract
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Abstract MDS/MPN syndromes comprise a heterogeneous group of myeloid neoplasms. While CMML has been extensively characterized, data on other MDS/MPN entities remain limited, particularly in real-world settings. We conducted a nationwide, multicenter retrospective study including adult patients with MDS/MPN syndromes other than CMML from 17 Italian centers. Patients were classified as MDS/MPN with SF3B1 mutation and thrombocytosis ( SF3B1 -T), atypical chronic myeloid leukemia/chronic neutrophilic leukemia (aCML/CNL), or MDS/MPN not otherwise specified (NOS). A total of 101 patients were included (median age 71 years). MDS/MPN SF3B1 -T showed a significant survival advantage compared with MDS/MPN NOS and aCML/CNL (OS p = 0.0058, LFS p = 0.012). aCML/CNL displayed the worst outcomes and most genetic complexity. Across the overall cohort, IPSS-M demonstrated the highest discriminative ability and calibration accuracy for both OS and LFS, outperforming CMML-directed models, including BLAST-clinical and BLAST-molecular whereas disease-specific scores provided optimal stratification within individual entities but failed to adequately predict outcomes in MDS/MPN NOS. In this real-world analysis, IPSS-M emerged as the most robust prognostic framework across MDS/MPN entities other than CMML. The inability of current tools to reliably stratify patients with MDS/MPN NOS underscores the biological heterogeneity of this category and highlights the urgent need for integrated clinical and genomic prognostic models.
Bibliografischer Nachweis
Publikationsdaten
- Autor:innen
- Luca Guarnera, Giuseppe Gaetano Loscocco, Massimo Breccia, Mara Memoli, Bruno Fattizzo, Marta Riva, Ambra Di Veroli, Michelina Santopietro, Giovanni Iaquinta, Annalisa Biagi, Sabrina Leonetti Crescenzi, Caterina Tatarelli, Roberto Latagliata, Paola Grammatico, Sabrina Mariani, Anna Lina Piccioni, Emilia Scalzulli, Costanza Andriola, Lorenzo Rizzo, Prassede Salutari, Gianluca Albanese, Francesca Romana Iovene, Alfredo Molteni, Novella Pugliese, Francesco Muriano, Federica Catania, Alessandro Andriani, Katia Paciaroni, Antonio Spadea, Paola Guglielmelli, Alessandro Maria Vannucchi, Emiliano Fabiani, Maria Teresa Voso, Adriano Venditti, Luca Maurillo
- Quelle
- Clinical and Experimental Medicine
- Publikation
- 2026-01-01
- Band / Ausgabe
- Nicht angegeben
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- ISSN / ISBN
- 1591-9528
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Zitierfähiger Nachweis
Luca Guarnera, Giuseppe Gaetano Loscocco, Massimo Breccia, Mara Memoli, Bruno Fattizzo, Marta Riva, Ambra Di Veroli, Michelina Santopietro, Giovanni Iaquinta, Annalisa Biagi, Sabrina Leonetti Crescenzi, Caterina Tatarelli, Roberto Latagliata, Paola Grammatico, Sabrina Mariani, Anna Lina Piccioni, Emilia Scalzulli, Costanza Andriola, Lorenzo Rizzo, Prassede Salutari, Gianluca Albanese, Francesca Romana Iovene, Alfredo Molteni, Novella Pugliese, Francesco Muriano, Federica Catania, Alessandro Andriani, Katia Paciaroni, Antonio Spadea, Paola Guglielmelli, Alessandro Maria Vannucchi, Emiliano Fabiani, Maria Teresa Voso, Adriano Venditti, Luca Maurillo (2026). Prognostic stratification in MDS/MPN syndromes other than CMML: an Italian multicenter real-world study. Clinical and Experimental Medicine. https://doi.org/10.1007/s10238-026-02287-0
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