JAK2V617F mutant-positive polycythemia vera complicated with acute coronary syndrome: a report of 2 cases and literature review

Abstract

Objective To review the clinical features, diagnosis, prognosis and treatment of polycythemia vera(PV)complicated with acute coronary syndrome(ACS). Methods The clinical data of 2 PV patients complicated with ACS admitted to Peking Union Medical College Hospital (PUMCH) were retrospectively analyzed and the recent literatures were reviewed. Results Case 1 was a 65-year old man who had been diagnosed PV with a positive JAK2V617F mutation 3 years ago. At presentation, the patient was suffering from recurrent angina pectoris, and coronary angiography revealed that there was a severe(80%)stenosis in the middle segment of left circumflex and a Xience V stent was implanted. After the percutaneous transluminal coronary intervention (PCI), secondary prevention for coronary heart disease and hydroxyurea for PV were given and the patient has been followed up regularly for more than three years and he is going well. Case 2 is a 44-year old man who was diagnosed PV with a positive JAK2 mutation 3 years ago and hydroxyurea, interferon, aspirin was prescribed. Then splenic infarction, thrombosis of splenic vein, regional portal hypertension, severe varices of fundus of stomach and upper gastrointestinal bleeding developed with him. Two months ago, an AMI of inferior wall occurred and the angiographic findings demonstrated an thrombotic lesion in the proximal segment of the right coronary artery with a moderate stenosis (60%); 1 month ago an AMI of anterior wall developed and coronary angiography discovered that there were diffuse thrombus in the proximal segment of left anterior descending artery with a severe stenosis (90%) and a complete occlusion in the right coronary artery. After double antiplatelet therapy with anticoagulation therapy of warfarin was given, the patient recovered gradually. Conclusions PV complicated with ACS is relatively rare. According to recent studies, positive JAK2V617F mutation, leukocytosis, age>65 years and positive history of thrombosis are the most important predictors of cardiovascular events. Clinicians should design individualized treatment strategies for patients on the basis of clinical features, coronary angiography findings and complications. For those with thrombotic lesion in the coronary artery due to the hypercoagulative state caused by PV, it should be cautious to carry out a coronary revascularization treatment. Key words: Vera polycythemia; Coronary disease; Review literature