ACUTE HEMOLYTIC-ANEMIA AS INITIAL MANIFES TATION IN A PATIENT WITH WILSONS-DISEASE

A 25-year-old woman who had delivered a normal child after a normal pr egnancy four months ago, was suffering from a common cold. The latter was treated by tetracyclines. A few days later she developed an acute severe hemolysis. Moreover she had signs of hepatic failure which was characterized by a considerably reduced synthetic capacity of the Live r but low serum aminotransferase levels and a proximal renal tubular d isorder. With a negative Coombs test an autoimmune hemolytic anemia wa s unlikely. Therefore diagnostic procedures were directed at the cause of the liver injury. An increased urinary excretion of copper, strong ly elevated levels of liver tissue copper and the detection of a Kayse r-Fleischer ring by slit-lamp examination proved the diagnosis of Wils on's disease - presenting clinically only as severe hemolysis. Cerulop lasmin concentration in serum was in low normal range and not diagnost ic.