Validation of the IPSET score for thrombosis in patients with prefibrotic myelofibrosis

Paola Guglielmelli, Alessandra Carobbio, Elisa Rumi, Valerio De Stefano, Lara Mannelli, Francesco Mannelli, Giada Rotunno, Giacomo Coltro, Silvia Betti, Chiara Cavalloni, Maria Chiara Finazzi, Juergen Thiele, Mario Cazzola, Alessandro Maria Vannucchi, Tiziano Barbui

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Article number21
JournalBlood Cancer Journal
Issue number2
Publication statusPublished - Feb 1 2020

ASJC Scopus subject areas

  • Hematology
  • Oncology


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