부산대학교 도서관

A 20-year-old man came to a local hospital with complaining of headache and general malaise and he was referred to our hospital for the treatment of renal dysfunction, anemia, thrombocytopenia and severe hypertension. The diagnosis of thrombotic microangiopathy (TMA) was made because of thrombocytopenia, hemolytic anemia in accompanied with fragmentation of erythrocytes and progressive renal failure. After hospitalization, antihypertensive therapy by calcium channel blockers, plasmapheresis and administration of glucocorticoid hormone were performed. Because the activity of plasma von Willebrand factor cleaving protease (vWF-CP)/ADAMTS13 was normal, he was diagnosed as having TMA associated with malignant hypertension. Thrombocytopenia and hemolytic anemia were immediately improved by the beginning of these treatments, however renal insufficiency and proteinuria were deteriorated and congestive heart failure developed. Following continuous hemodiafiltration (CHDF) and administration of angiotensin converting enzyme inhibitor and angiotensin receptor II antagonist, overall general condition and renal function improved. Blood purification therapy such as CHDF was no longer required, and the patient was discharged from our hospital. Renal biopsy performed during convalescence showed endothelial edematous thickening of arteriole, indicating malignant nephrosclerosis. As causes of TMA comprise a divergent range of factors, it is often difficult to establish the differential diagnosis based on clinical manifestations only. Here, we report it because the measurement of the activity of plasma vWF-CP/ADAMTS13 was useful for diagnosing TMA with malignant hypertension.