Surgical Intervention in Extensive Splanchnic Arteriovenous Thrombosis Associated with JAK-2 Mutation

Introduction: Essential thrombocytosis is a rare condition caused by an overproduction of platelets. The overwhelming number of patients are asymptomatic and managed conservatively. We present two cases of complications related to ET that required surgical management. Case Presentation: The first cases was a 75 year-old female with a hypercoagulable states presented with abdominal pain and was noted to have acute splenic and mesenteric infarcts she underwent splenectomy followed by partial enterectomy as definitive management. The second patient presented with distal mesenteric small vessel thrombosis and bowel ischemia underwent an exploratory laparotomy followed by interval small bowel resection and primary anastomosis. Both received therapeutic anticoagulation and oral chemotherapy for their essential thrombocytosis both patients were noted to have JAK-2 mutation. Discussion: Essential thrombocytosis is a rare cause of major thromboembolism. Most cases involve microvascular thrombosis in small or medium sized vessels. The morbidity and mortality exceeds 20% in large vessel disease therefore requiring prompt recognition and appropriate management to improve patient outcomes. JAK-2 gene mutations have recently been found to have an association with many prothrombotic conditions, namely polycythemia vera and other myelodysplastic syndromes. Individuals with this mutation have a 1.8 to 2.3-fold increased risk of thrombosis. our review of literature revealed 58 total articles and case reports. All reported cases were managed with medical therapy with the exception to the cases were have reported. Conclusion: Essential thrombocytosis has a rare association with large vessel thrombosis in both the arterial and venous systems that may require surgical intervention, especially in the elderly population. JAK-2 mutations should be considered as an etiological risk factor.