Ruxolitinib reverses systemic vasculitis driven by JAK2 V617F–mutated essential thrombocythemia: a case report

Background Vasculitis associated with myeloproliferative neoplasms is rare, and reports on its treatment are limited. Herein, we report a case of Janus Kinase2 (JAK2) V617F mutation–positive essential thrombocythemia (ET) associated with vasculitis. Case presentation A 60-year-old man with JAK2 V617F mutation–positive ET underwent coronary artery bypass grafting after recurrent myocardial infarctions and coronary stent occlusions. Intraoperative and pathological findings led to a diagnosis of vasculitis. Treatment with hydroxycarbamide and ruxolitinib resulted in decreased platelet counts and improved vasculitis, with no subsequent recurrence of cardiovascular events. Conclusion This rare case shows that ruxolitinib can be effective in treating vasculitis complications in patients with JAK2 mutation–positive ET.