Cardiology and Angiology: An International Journal

Acute limb ischemia (ALI) is mainly caused by thrombosis or embolism which commonly occurs in the elderly population with various comorbidities. But young people may also present with ALI especially who suffer from various types of hypercoagulable diseases. Among the inherited hypercoagulable diseases Polycythemia Rubra Vera (PRV) is an extremely rare entity. A 35 year old lady came to the Vascular Surgery Department of Dhaka medical College Hospital with the complaints of gangrene of right middle finger 7 days. She also gave auto amputation of part of small finger two weeks back due to gangrene. On examination her ulnar pulse was absent. Her blood picture showed increased RBC, WBC and platelet count with raised fibrinogen degradation product (FDP) and D-dimer level. Her echocardiography was unremarkable. There was no evidence of small vessel vasculitis. Duplex ultrasound and computed tomography (CT) angiogram both showed thrombotic occlusion of ulnar and digital arteries. Besides diagnosis of PRV was confirmed by presence JAK 2 mutation. She underwent open catheter guided thrombectomy through proximal ulnar artery as endovascular facilities were unavailable. Post operatively she was on anticoagulant. She was also administered hydroxyurea and aspirin for management of PRV. Follow up after one month showed there was no sign of ischemia.