We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.

De Stefano, V., Vannucchi, A. M., Ruggeri, M., Cervantes, F., Alvarez Larrán, A., Iurlo, A., Randi, M. L., Pieri, L., Rossi, E., Guglielmelli, P., Betti, S., Elli, E., Finazzi, M. C., Finazzi, G., Zetterberg, E., Vianelli, N., Gaidano, G., Nichele, I., Cattaneo, D., Palova, M., Ellis, M. H., Cacciola, E., Tieghi, A., Hernandez Boluda, J. C., Pungolino, E., Specchia, G., Rapezzi, D., Forcina, A., Musolino, C., Carobbio, A., Griesshammer, M., Barbui, T., Splanchnic vein thrombosis in myeloproliferative neoplasms: Risk factors for recurrences in a cohort of 181 patients, <<BLOOD CANCER JOURNAL>>, 2016; 6 (11): e493-e493. [doi:10.1038/bcj.2016.103] [http://hdl.handle.net/10807/93251]

Splanchnic vein thrombosis in myeloproliferative neoplasms: Risk factors for recurrences in a cohort of 181 patients

De Stefano, Valerio;Rossi, Elena;Betti, Silvia;
2016

Abstract

We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.
eng
http://www.nature.com/bcj/index.html
De Stefano, V., Vannucchi, A. M., Ruggeri, M., Cervantes, F., Alvarez Larrán, A., Iurlo, A., Randi, M. L., Pieri, L., Rossi, E., Guglielmelli, P., Betti, S., Elli, E., Finazzi, M. C., Finazzi, G., Zetterberg, E., Vianelli, N., Gaidano, G., Nichele, I., Cattaneo, D., Palova, M., Ellis, M. H., Cacciola, E., Tieghi, A., Hernandez Boluda, J. C., Pungolino, E., Specchia, G., Rapezzi, D., Forcina, A., Musolino, C., Carobbio, A., Griesshammer, M., Barbui, T., Splanchnic vein thrombosis in myeloproliferative neoplasms: Risk factors for recurrences in a cohort of 181 patients, <>, 2016; 6 (11): e493-e493. [doi:10.1038/bcj.2016.103] [http://hdl.handle.net/10807/93251]
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