Splanchnic vein thromboses associated with myeloproliferative neoplasms: An international, retrospective study on 518 cases

Emanuela Sant'Antonio, Paola Guglielmelli, Lisa Pieri, Massimo Primignani, Maria Luigia Randi, Claudia Santarossa, Elisa Rumi, Francisco Cervantes, Federica Delaini, Alessandra Carobbio, Silvia Betti, Elena Rossi, Noa Lavi, Claire N. Harrison, Natalia Curto-Garcia, Heinz Gisslinger, Bettina Gisslinger, Giorgina Specchia, Alessandra Ricco, Nicola VianelliNicola Polverelli, Maya Koren-Michowitz, Marco Ruggeri, Francois Girodon, Martin Ellis, Alessandra Lurlo, Francesco Mannelli, Lara Mannelli, Benedetta Sordi, Giuseppe Gaetano Loscocco, Mario Cazzola, Valerio De Stefano, Tiziano Barbui, Ayalew Tefferi, Alessandro Maria Vannucchi

Research output: Contribution to journalArticle

Abstract

Myeloproliferative Neoplasms (MPN) course can be complicated by thrombosis involving unusual sites as the splanchnic veins (SVT). Their management is challenging, given their composite vascular risk. We performed a retrospective, cohort study in the framework of the International Working Group for MPN Research and Treatment (IWG-MRT), and AIRC-Gruppo Italiano Malattie Mieloproliferative (AGIMM). A total of 518 MPN-SVT cases were collected and compared with 1628 unselected, control MPN population, matched for disease subtype. Those with MPN-SVT were younger (median 44 years) and enriched in females compared to controls; PV (37.1%) and ET (34.4%) were the most frequent diagnoses. JAK2V617F mutation was highly prevalent (90.2%), and 38.6% of cases had an additional hypercoagulable disorder. SVT recurrence rate was 1.6 per 100 patient-years. Vitamin K-antagonists (VKA) halved the incidence of recurrence (OR 0.48), unlike cytoreduction (OR 0.96), and were not associated with overall or gastrointestinal bleeding in multivariable analysis. Esophageal varices were the only independent predictor for major bleeding (OR 17.4). Among MPN-SVT, risk of subsequent vascular events was skewed towards venous thromboses compared to controls. However, MPN-SVT clinical course was overall benign: SVT were enriched in PMF with lower IPSS, resulting in significantly longer survival than controls; survival was not affected in PV and slightly reduced in ET. MPN-U with SVT (n = 55) showed a particularly indolent phenotype, with no signs of disease evolution. In the to-date largest, contemporary cohort of MPN-SVT, VKA were confirmed effective in preventing recurrence, unlike cytoreduction, and safe; the major risk factor for bleeding was esophageal varices that therefore represent a major therapeutic target.

Original languageEnglish
JournalAmerican Journal of Hematology
DOIs
Publication statusAccepted/In press - Jan 1 2019

ASJC Scopus subject areas

  • Hematology

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    Sant'Antonio, E., Guglielmelli, P., Pieri, L., Primignani, M., Randi, M. L., Santarossa, C., Rumi, E., Cervantes, F., Delaini, F., Carobbio, A., Betti, S., Rossi, E., Lavi, N., Harrison, C. N., Curto-Garcia, N., Gisslinger, H., Gisslinger, B., Specchia, G., Ricco, A., ... Vannucchi, A. M. (Accepted/In press). Splanchnic vein thromboses associated with myeloproliferative neoplasms: An international, retrospective study on 518 cases. American Journal of Hematology. https://doi.org/10.1002/ajh.25677