Abstract
BackgroundSplanchnic Vein Thrombosis (SVT) is strongly associated with underlying JAK2 V617F positive myeloproliferative neoplasms (MPN).MethodsPatients attending the tertiary haematology service in Northern Ireland with SVT and underlying JAK2 V617F MPN were identified by consultant staff. A retrospective audit was undertaken to examine therapeutic interventions and relevant outcomes. Descriptive statistics were used for qualitative data whilst students t-test allowed comparison of quantitative data.ResultsWe report on the medium to long term follow-up of fourteen patients presenting with SVT on the basis of JAK2 V617F positive MPN. Females comprised 78.5% of the patients and there was an average age of 47.3 years at time of diagnosis. There was significant morbidity evident at diagnosis with liver transplantation attempted in all patients with Budd Chiari (n = 3), oesophageal varices present in 57.1%, ascites present in 42.8% and splenomegaly evident in 71.4%. 42.8% of patients did not exhibit classical phenotypic blood count findings for MPN at time of diagnosis. Over a median follow-up of 88.5 months (range = 8–211 months) recurrence of SVT was only documented in the setting of interventional liver procedure. Major haemorrhagic complications were recorded in 35.7% of patients and there was an association with dual anticoagulation and antiplatelet use. Recurrent thrombosis outside of the splanchnic venous system occurred in 28.5% of patients, predominantly occurring off therapeutic anticoagulation. No deaths were recorded and one transformation to myelofibrosis was seen during follow-up. Cytoreduction therapies were routinely used but had a high discontinuation rate due to cytopenias and intolerance.ConclusionThis analysis highlights the complexities of management of this group of patients over a period of long follow-up with a focus on the evidence behind therapeutic options.
Highlights
Splanchnic Vein Thrombosis (SVT) is strongly associated with underlying JAK2 V617F positive myeloproliferative neoplasms (MPN)
JAK2 V617F positive MPNs have been identified as the most common underlying cause in splanchnic vein thrombosis not associated with local factors, for example cirrhosis or malignancy
In a prospective study of 604 patients with proven SVT, underlying overt MPN was identified in 8% of patients whilst the JAK2 V617F mutation was detected in 20% [6]
Summary
Splanchnic Vein Thrombosis (SVT) is strongly associated with underlying JAK2 V617F positive myeloproliferative neoplasms (MPN). Splanchnic vein thrombosis (SVT) describes a heterogeneous group of disorders involving venous thrombus within the portal vein (PVT), superior mesenteric vein, splenic vein or hepatic veins (Budd-Chiari Syndrome (BCS)). This life changing diagnosis is associated with significant morbidity resulting from liver decompensation. The presence of a JAK2 V617F mutant clone predisposes to the formation of thrombus. JAK2 V617F positive MPNs have been identified as the most common underlying cause in splanchnic vein thrombosis not associated with local factors, for example cirrhosis or malignancy. The number of CALR positive patients detected in the context of SVT is much lower at around 0–2.5% [10]
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