TY - JOUR
T1 - Splanchnic vein thrombosis in myeloproliferative neoplasms
T2 - Risk factors for recurrences in a cohort of 181 patients
AU - De Stefano, Valerio
AU - Vannucchi, Alessandro M.
AU - Ruggeri, Marco
AU - Cervantes, Francisco
AU - Alvarez-Larrán, Alberto
AU - Iurlo, Alessandra
AU - Randi, M. L.
AU - Pieri, Lisa
AU - Rossi, E.
AU - Guglielmelli, Paola
AU - Betti, Silvia
AU - Elli, Elena
AU - Finazzi, Mariachiara
AU - Finazzi, Guido
AU - Zetterberg, E.
AU - Vianelli, Nicola
AU - Gaidano, Gianluca
AU - Nichele, Ilaria
AU - Cattaneo, Daniele
AU - Palova, M.
AU - Ellis, M. H.
AU - Cacciola, Emma
AU - Tieghi, Alessia
AU - Hernández-Boluda, Juan Carlos
AU - Pungolino, Esther
AU - Specchia, Giorgina
AU - Rapezzi, Davide
AU - Forcina, A.
AU - Musolino, Caterina
AU - Carobbio, Alessandra
AU - Griesshammer, M.
AU - Barbui, Tiziano
PY - 2016/11/4
Y1 - 2016/11/4
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84994624429&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84994624429&partnerID=8YFLogxK
U2 - 10.1038/bcj.2016.103
DO - 10.1038/bcj.2016.103
M3 - Article
VL - 6
JO - Blood Cancer Journal
JF - Blood Cancer Journal
SN - 2044-5385
IS - 11
M1 - e493
ER -