Abstract
We report a case of Prinzmetal angina as inaugural manifestation of coronary disease, in a young adult male patient, recently started on anagrelide for essential thrombocythemia. Moderate proximal left anterior descendent coronary artery stenosis was documented by angiography, and interventional or surgical revascularization has been discussed. Patient’s option was for medical therapy alone. Anagrelide was temporarily withdrawn and rechallenged uneventfully after a couple of months and clinical evolution is good at four years follow-up. The mechanism by which anagrelide could induce coronary spasm and ischemia remains to be clarified.