JAK2V617F mutant-positive polycythemia vera complicated with acute coronary syndrome:a report of 2 cases and literature review / 中华全科医师杂志

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 JAK 2 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 .