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.

Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients / De Stefano, V; Vannucchi, Am; Ruggeri, M; Cervantes, F; Alvarez-Larran, A; Iurlo, A; Randi, Ml; Pieri, L; Rossi, E; Guglielmelli, P; Betti, S; Elli, E; Finazzi, Mc; Finazzi, G; Zetterberg, E; Vianelli, N; Gaidano, G; Nichele, I; Cattaneo, D; Palova, M; Ellis, Mh; Cacciola, E; Tieghi, A; Hernandez-Boluda, Jc; Pungolino, E; Specchia, G; Rapezzi, D; Forcina, A; Musolino, C; Carobbio, A; Griesshammer, M; Barbui, T. - In: BLOOD CANCER JOURNAL. - ISSN 2044-5385. - 6:11(2016), pp. e493-e493. [10.1038/bcj.2016.103]

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

Carobbio A;
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.
2016
6
11
e493
e493
Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients / De Stefano, V; Vannucchi, Am; Ruggeri, M; Cervantes, F; Alvarez-Larran, A; Iurlo, A; Randi, Ml; Pieri, L; Rossi, E; Guglielmelli, P; Betti, S; Elli, E; Finazzi, Mc; Finazzi, G; Zetterberg, E; Vianelli, N; Gaidano, G; Nichele, I; Cattaneo, D; Palova, M; Ellis, Mh; Cacciola, E; Tieghi, A; Hernandez-Boluda, Jc; Pungolino, E; Specchia, G; Rapezzi, D; Forcina, A; Musolino, C; Carobbio, A; Griesshammer, M; Barbui, T. - In: BLOOD CANCER JOURNAL. - ISSN 2044-5385. - 6:11(2016), pp. e493-e493. [10.1038/bcj.2016.103]
De Stefano, V; Vannucchi, Am; Ruggeri, M; Cervantes, F; Alvarez-Larran, A; Iurlo, A; Randi, Ml; Pieri, L; Rossi, E; Guglielmelli, P; Betti, S; Elli, E; Finazzi, Mc; Finazzi, G; Zetterberg, E; Vianelli, N; Gaidano, G; Nichele, I; Cattaneo, D; Palova, M; Ellis, Mh; Cacciola, E; Tieghi, A; Hernandez-Boluda, Jc; Pungolino, E; Specchia, G; Rapezzi, D; Forcina, A; Musolino, C; Carobbio, A; Griesshammer, M; Barbui, T
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