著者
山崎 義亀与 松田 保 黒田 満彦 内田 健三 嶋田 千恵子 大谷 逸子 村上 元孝 北川 正信
出版者
一般社団法人 日本血液学会
雑誌
臨床血液 (ISSN:04851439)
巻号頁・発行日
vol.14, no.1, pp.81-87, 1973

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.<br>Examination revealed scleral icterus, pallor, numerous petechiae and an ecchymosis. The sensorium was clear. The neurologic examination was normal.<br>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.<br>He was treated with prednisolone and heparin without benefits, and expired on the 14 th hospital day.<br>Hyaline thromboses of the vessels of liver and kidney were demonstrated by the examination of the specimens obtained by post mortem needle puncture.<br>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.<br>Immunological analysis of serum proteins showed the increase of acute phase reactants, the decrease of &beta;<sub>1</sub>-AC and haptoglobin, and the appearance of fibrinogen degradation products.<br>These results were discussed in relation to the pathogenesis of the disease.