One Case of Henoch-Sch nelin Purpura Associated with Thrombotic Thrombocytopenic Purpura / 대한신장학회잡지

ABSTRACT

Henoch-Sch nlein purpura(HSP) is primarily a disorder of childhood and chracterized by skin rash, joint pain, abdominal pain and renal involvement. Thrombotic thrombocytopenic purpura(TTP) is a rare clinical syndrome of unknown etiology and classically presents with microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological signs, renal dysfuncion and fever. Recently, we experienced one patient with histologically proven HSP, and manifested as a TTP like clinical feature. Patient is a 8-year-old boy who had been diagnosed one month earlier as HSP. He showed microangiopathic hemolytic anemia, renal failure and seizure during the course of his illness. Histological evaluation of renal biopsy confirmed the presence of intravascular thrombosis. Treatment with fresh frozen plasma, plasmapheresis, steroid therapy and peritoneal dialysis resulted in clinical improvement. But he expired on 130th day after admission due to aspiration pneumonia and pneumothorax.