Unilateral emboli in a patient with thrombotic thrombocytopenic purpura.

Abstract

Thrombotic thrombocytopenic purpura is a rare disease most commonly associated with microangiopathic hemolytic anemia, thrombocytopenia, fever, neurologic disorders, and renal dysfunction. We describe a patient with a history of thrombotic thrombocytopenic purpura that had been quiescent for 4 months; he had a 3-week history of painful purpuric lesions on the left hand only. He also had mottling and a livedoid purpura of the distal fingertips, splinter hemorrhages of the left fingernails, and a decreased radial pulse. Findings of a biopsy specimen revealed multiple capillary and small vessel thromboses. Contrast aortography demonstrated a pseudoaneurysm of the proximal descending thoracic aorta with stenosis of the left subclavian artery at its origin and an associated thrombus.