A 25-year-old male was admitted because of dark-red urine, jaundice and purpura. 5 days prior to admission, the patient noticed that the urine color became dark red, and purpuric rash and jaundice were noted by his wife.
Examination revealed scleral icterus, pallor, numerous petechiae and an ecchymosis. The sensorium was clear. The neurologic examination was normal.
The patient had hemolytic anemia, thrombocytopenic purpura, fever and proteinuria. Shortly after admission, fluctuating neurologic symptoms developed, and the patient was diagnosed as thrombotic thrombocytopenic purpura.
He was treated with prednisolone and heparin without benefits, and expired on the 14 th hospital day.
Hyaline thromboses of the vessels of liver and kidney were demonstrated by the examination of the specimens obtained by post mortem needle puncture.
Coagulation studies disclosed that partial thromboplastin time and prothrombin time were slightly prolonged, however, factor V activity and fibrinogen titre were not low. Fibrinogen degradation products determined by tanned red cell hemagglutination inhibition immunoassay were markedly increased, although euglobulin lysis time was not shortened.
Immunological analysis of serum proteins showed the increase of acute phase reactants, the decrease of β1-AC and haptoglobin, and the appearance of fibrinogen degradation products.
These results were discussed in relation to the pathogenesis of the disease.
