Guglielmelli, Paola ORCID: 0000-0003-1809-284X, Carobbio, Alessandra ORCID: 0000-0001-9664-7084, Rumi, Elisa, De Stefano, Valerio ORCID: 0000-0002-5178-5827, Mannelli, Lara, Mannelli, Francesco ORCID: 0000-0003-4810-6501, Rotunno, Giada, Coltro, Giacomo, Betti, Silvia, Cavalloni, Chiara, Finazzi, Maria Chiara, Thiele, Juergen, Cazzola, Mario, Vannucchi, Alessandro Maria and Barbui, Tiziano (2020). Validation of the IPSET score for thrombosis in patients with prefibrotic myelofibrosis. Blood Cancer J., 10 (2). LONDON: NATURE PUBLISHING GROUP. ISSN 2044-5385

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Abstract

Pre-fibrotic myelofibrosis (pre-PMF) and essential thrombocythemia (ET) are characterized by similarly increased rate of thrombotic events, but no study specifically analyzed risk factors for thrombosis in pre-PMF. In a multicenter cohort of 382 pre-PMF patients collected in this study, the rate of arterial and venous thrombosis after diagnosis was 1.0 and 0.95% patients/year. Factors significantly associated with arterial thrombosis were age, leukocytosis, generic cardiovascular risk factors, JAK2V617F and high molecular risk mutations, while only history of previous thrombosis, particularly prior venous thrombosis, was predictive of venous events. The risk of total thromboses was accurately predicted by the the international prognostic score for thrombosis in essential thrombocythemia (IPSET) score, originally developed for ET, and corresponded to 0.67, 2.05, and 2.95% patients/year in the low-, intermediate-, and high-risk categories. IPSET was superior to both the conventional 2-tiered score and the revised IPSET in this cohort of pre-PMF patients. We conclude that IPSET score can be conveniently used for thrombosis risk stratification in patients with pre-PMF and might represent the basis for individualized management aimed at reducing the increased risk of major cardiovascular events. Further refinement of the IPSET score in pre-PMF might be pursued by additional, prospective studies evaluating the inclusion of leukocytosis and/or adverse mutational profile as novel variables.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Guglielmelli, PaolaUNSPECIFIEDorcid.org/0000-0003-1809-284XUNSPECIFIED
Carobbio, AlessandraUNSPECIFIEDorcid.org/0000-0001-9664-7084UNSPECIFIED
Rumi, ElisaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
De Stefano, ValerioUNSPECIFIEDorcid.org/0000-0002-5178-5827UNSPECIFIED
Mannelli, LaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mannelli, FrancescoUNSPECIFIEDorcid.org/0000-0003-4810-6501UNSPECIFIED
Rotunno, GiadaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Coltro, GiacomoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Betti, SilviaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cavalloni, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Finazzi, Maria ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thiele, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cazzola, MarioUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vannucchi, Alessandro MariaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Barbui, TizianoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-344060
DOI: 10.1038/s41408-020-0289-2
Journal or Publication Title: Blood Cancer J.
Volume: 10
Number: 2
Date: 2020
Publisher: NATURE PUBLISHING GROUP
Place of Publication: LONDON
ISSN: 2044-5385
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
INTERNATIONAL PROGNOSTIC SCORE; ESSENTIAL THROMBOCYTHEMIA; CALRETICULIN MUTATIONS; POLYCYTHEMIA-VERA; CLINICAL-COURSE; RISK; DIAGNOSIS; PHENOTYPE; NEOPLASMS; SURVIVALMultiple languages
Oncology; HematologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/34406

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