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.
Highlights
Splanchnic vein thrombosis (SVT) encompasses hepatic veins (Budd–Chiari syndrome, BCS) and portal, mesenteric and splenic veins, and is a rare event in the general population: the annual incidence accounts for 0.8 per million for BCS, 0.7 per 100 000 for portal vein thrombosis and 2.7 per 100 000 for mesenteric vein thrombosis.[1,2,3,4] In these cases, the JAK2V617F mutation, the hallmark of myeloproliferative neoplasms (MPNs), can be detected in 13.6–26.6% of non-cirrhotic and non-malignant SVT patients, even in the absence of a clear diagnostic picture of MPN.[5]
A recent survey involving expert haematologists revealed a marked heterogeneity of choices on the duration of vitamin K-antagonist (VKA) and on the use of cytoreduction; this survey showed great uncertainty regarding the use of aspirin either alone or in association with VKA.[10]
Backward logistic regression analysis showed an independent association with the new thrombotic event for BCS (HR: 3.03, 95% confidence interval (CI): 1.37–6.69), history of previous thrombosis (HR: 3.62, 95% CI: 1.22–10.78), splenomegaly (HR: 2.66, 95% CI: 1.06–6.64) and leukocytosis higher than the highest quartile, 14 × 109/l (HR: 2.8, 95% CI: 1.32–6.28)
Summary
Splanchnic vein thrombosis (SVT) encompasses hepatic veins (Budd–Chiari syndrome, BCS) and portal, mesenteric and splenic veins, and is a rare event in the general population: the annual incidence accounts for 0.8 per million for BCS, 0.7 per 100 000 for portal vein thrombosis and 2.7 per 100 000 for mesenteric vein thrombosis.[1,2,3,4] In these cases, the JAK2V617F mutation, the hallmark of myeloproliferative neoplasms (MPNs), can be detected in 13.6–26.6% of non-cirrhotic and non-malignant SVT patients, even in the absence of a clear diagnostic picture of MPN.[5] In fully expressed Philadelphia-negative MPN, including polycythaemia vera (PV), essential thrombocythaemia and primary myelofibrosis, the more frequent sites of thrombosis are arterial vessels or leg veins and pulmonary embolisms (PEs) and include unusual sites such as cerebral veins[6] and SVT. A recent survey involving expert haematologists revealed a marked heterogeneity of choices on the duration of VKA and on the use of cytoreduction; this survey showed great uncertainty regarding the use of aspirin either alone or in association with VKA.[10]
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