Increased risk of thrombosis in JAK2 V617F-positive patients with primary myelofibrosis and interaction of the mutation with the IPSS score
Tiziano Barbui, Arianna Ghirardi, Alessandra Carobbio, Arianna Masciulli, Greta Carioli, Alessandro Rambaldi, Maria Chiara Finazzi, Marta Bellini, Elisa Rumi, Daniele Vanni, Oscar Borsani, Francesco Passamonti, Barbara Mora, Marco Brociner, Paola Guglielmelli, Chiara Paoli, Alberto Álvarez‐Larrán, Ana Triguero, Marta Garrote, Helna Pettersson, Björn Andréasson, Giovanni Barosi, Alessandro M. Vannucchi
Abstract
Thrombosis remains a major unmet need in polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), accounting for three to four-fold higher probability of risk compared to controls [1]. In PMF, this risk is poorly characterized given that, unlike PV and ET, death due to disease progression and/or evolution to leukemia represents a competing risk that significantly overwhelms that attributable to vascular complications [2–5]. We analyzed a large cohort of PMF patients enrolled in the European ERNEST registry with the aim to identify patients at higher risk in which anti-thrombotic prophylaxis could be suggested. Primary prophylaxis with aspirin is debated in PMF and, since thrombosis is not considered a major issue, some experts prescribe this agent only as a secondary prophylaxis after an arterial event [6].