title: Association of Oesophageal Varices and Splanchnic Vein Thromboses in Patients with JAK2-Positive Myeloproliferative Neoplasms: Presentation of Two Cases and Data from a Retrospective Analysis.
abstract: BACKGROUND
  Oesophageal varices and gastrointestinal bleeding are common complications of liver cirrhosis. More rarely, oesophageal varices occur in patients with non-cirrhotic portal hypertension that results from thromboses of portal or splanchnic veins.
  
  
  METHODS
  We describe 2 young men who initially presented with varices as a result of portal vein thromboses. In the clinical follow-up, both were tested positive for a JAK2 mutation and consequently diagnosed with myeloproliferative neoplasms (MPNs). In an attempt to characterise the frequency of gastrointestinal complications in patients with JAK2-positive MPNs, we retrospectively analysed all known affected patients from our clinic for the diagnosis of portal vein thromboses and oesophageal varices. Strikingly, 48% of those who had received an oesophagogastroduodenoscopy had detectable oesophageal or gastric varices, and 82% of those suffered from portal or splanchnic vein thromboses.
  
  
  CONCLUSIONS
  While the association between JAK2, myeloproliferative disease and thrombotic events is well established, patients with idiopathic oesophageal varices are not regularly tested for JAK2 mutations. However, the occurrence of oesophageal varices may be the first presenting symptom of a MPN with a JAK2 mutation, and affected patients may profit from a close haematological monitoring to assure the early detection of developing MPN.
authors.affiliation: Departments of Medicine I, Dresden Technical University, Dresden, Germany.
authors.forename: Cornelia S
authors.initials: CS
authors.lastname: Link
authors.forename: Uwe
authors.initials: U
authors.lastname: Platzbecker
authors.forename: Frank
authors.initials: F
authors.lastname: Kroschinsky
authors.forename: Sven
authors.initials: S
authors.lastname: Pannach
authors.forename: Christian
authors.initials: C
authors.lastname: Thiede
authors.forename: Ivan
authors.initials: I
authors.lastname: Platzek
authors.forename: Gerhard
authors.initials: G
authors.lastname: Ehninger
authors.forename: Markus K
authors.initials: MK
authors.lastname: Schuler
keywords: JAK2; Myeloproliferative neoplasms; Oesophageal varices; Portal vein thrombosis; Splanchnic vein thrombosis; Variceal bleeding
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