MASCOT Registry of Patients With Myeloproliferative Neoplasms Associated Splanchnic Vein Thrombosis

Study Overview
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Brief Summary
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A registry of UK patients diagnosed to have splanchnic vein thrombosis and myeloproliferative neoplasm, including isolated mutation of JAK2V617f. The purpose of the registry is to understand outcomes, treatment variations and data to inform and enable future clinical trial design and facilitate regulatory approval decision-making.

Detailed Description
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Splanchnic or abdominal vein thrombosis (SVT) comprises thrombosis of hepatic, portal, superior mesenteric, splenic vein in isolation or combination. It has a prevalence of 1-2% in the general population based on autopsy reports. However, 40-50% of patients with hepatic vein thrombosis and about 25-30% of patients with PVT have an underlying myeloproliferative disease that is the cause of the thrombosis. Although some data on MPN-SVT have been published from European registries, the lack of sufficient clinical and scientific data in these patients has led to variation in treatment patterns with regard to timing, choice and duration of anticoagulation, the use of cytotoxic or biologic agents and interventions with thrombolysis and stenting with TIPSS in the acute stage. Data on the patterns of treatment of these patients in U.K and their outcomes with regard to morbidity and mortality are lacking. Availability of such data in U.K. will lead to the design of good quality prospective interventional studies. PROJECT DESIGN This registry is designed for longitudinal real-world data collection on UK patients with MPN-SVT who are managed at tertiary liver centres in U.K. A pre-defined data set, built by a committee of specialist clinicians from the Mascot registry investigators will be collected and entered into the electronic web-based database built by Dendrite Clinical Systems Ltd. The data captured includes demographic information linked to the NHS number, which will enable linked-anonymous data accrual and analysis as well as correlation with the Systemic Anti-Cancer Therapy Data Set (SACT) via the National Cancer Registration and Analysis Service (NCRAS) and Cancer Drug Fund (CDF) database. It will also allow interrogation of the national registries for stroke (SSNAP) and cardiac surgery (NACSA) as co-morbidities involving arterial circulation are prevalent in 30% of patients.

Official Title
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Myeloproliferative Neoplasms Associated SplanChnic Vein Thrombosis: Mascot Registry

Conditions
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JAK2 V617, Myeloproliferative Neoplasm, Splanchnic Vein Thrombosis

Intervention / Treatment
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* Combination Product: Drugs, procedures


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age>18 years Myeloproliferative neoplasm (MPN, WHO 2008, WHO 2016, BCSH) and splanchnic vein thrombosis Isolated Jak2v617f allele and splanchnic vein thrombosis MPN and portal cavernoma Exclusion Criteria: -

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Combination Product: Drugs, procedures|standard of care only. non interventional registry.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| transformation of MPN | transformation to Myelofibrosis or leukaemia | 5 years | 
| progression of liver disease | progression of liver disease | 5 years | 
| thrombosis events | new or recurrent or improvement of thrombosis | 5 years | 
| haemorrhage events | haemorrhage | 5 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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registry