Successful treatment of refractory hemolytic uremic syndrome with intravenous gamma-immunoglobulin therapy / 대한내과학회지

ABSTRACT

The hemolytic uremic syndrome (HUS) is a clinical syndrome defined by the presence of thrombocytopenia, microangiopathic hemolytic anemia and acute renal failure with or without a clinically apparent etiology. The conventional treatment of choice is plasmapheresis as a first-line therapy. Most patients respond to plasmapheresis whereas some patients are refractory to the therapy. The second-line therapy is not well established although various therapies such as steroid, vincristine, intravenous immunoglobulin have been suggested. The intravenous immunoglobulin therapy in refractory hemolytic-uremic syndrome have rarely been successful in complete remission. We report a case of refractory HUS in a 48 year-old man who developed hemolytic anemia, thrombocytopenia, acute renal failure and ischemic retinopathy. The patient was refractory to plasmapheresis as a first-line therapy. The patient received intravenous immunoglobulin therapy as a second-line therapy after 8 days of plasmapheresis, which subsequently resulted in a complete remission of refractory HUS. The complete remission using immunoglobulin in HUS has not been previously reported in Korea.