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Splanchnic vein thromboses associated with myeloproliferative neoplasms: An international,retrospective study on 518 cases |
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| Authors: | 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 Vianelli Nicola Polverelli Maya Koren-Michowitz Marco Ruggeri Francois Girodon Martin Ellis Alessandra Iurlo Francesco Mannelli Lara Mannelli Benedetta Sordi Giuseppe Gaetano Loscocco Mario Cazzola Valerio De Stefano Tiziano Barbui Ayalew Tefferi Alessandro Maria Vannucchi |
| Affiliation: | 1. CRIMM, Centro di Ricerca e Innovazione per le Malattie Mieloproliferative, Azienda Ospedaliera Universitaria Careggi, Florence, Italy Dipartimento di Medicina Sperimentale e Clinica, Università degli Studi, Firenze, DENOTHE Excellence Center, Florence, Italy Medical Genetics, University of Siena, Siena, Italy;2. CRIMM, Centro di Ricerca e Innovazione per le Malattie Mieloproliferative, Azienda Ospedaliera Universitaria Careggi, Florence, Italy;3. CRIMM, Centro di Ricerca e Innovazione per le Malattie Mieloproliferative, Azienda Ospedaliera Universitaria Careggi, Florence, Italy Dipartimento di Medicina Sperimentale e Clinica, Università degli Studi, Firenze, DENOTHE Excellence Center, Florence, Italy;4. CRC "A. M. e A. Migliavacca" Center for Liver Disease, Division of Gastroenterology and Hepatology, Foundation IRCCS Cà Granda Maggiore Policlinico Hospital, University of Milan, Milan, Italy;5. Department of Medicine – DIMED, University of Padova Medical School, Padova, Italy;6. Department of Hematology Oncology, IRCCS Policlinico S. Matteo Foundation and University of Pavia, Pavia, Italy;7. Hematology Department, Hospital Clinic, IDIBAPS, Barcelona, Spain;8. Hematology and Bone Marrow Transplant Unit, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy;9. Institute of Hematology, Catholic University, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy;10. Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel;11. Department of Haematology, Guy's and St. Thomas NHS Foundation Trust, London, UK;12. Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria;13. Department of Emergency and Organ Transplantation, Section of Hematology with Transplantation, Medical School, University of Bari, Bari, Italy;14. Department of Hematology and Clinical Oncology “L. and A. Seràgnoli” S. Orsola-Malpighi Hospital, Bologna, Italy;15. Department of Hematology, Shamir Medical Center (Assaf Harofeh), Zerifin, Israel and Sackler school of Medicine, Tel Aviv University, Tel Aviv, Israel;16. Hematology, San Bortolo Hospital, Vicenza, Italy;17. CHU Dijon, Laboratoire d'Hematologie, Dijon, France;18. The Hematology Institute and Blood Bank and Translational Hemato-Oncology, Meir Hospital, Kfar-Saba, Israel;19. Hematology Division, Foundation IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy;20. Research Foundation, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy;21. Division of Hematology, Mayo Clinic, Rochester, Minnesota |
| 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. |
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