先天性第V因子欠乏症(Parahemophilia)の1例:—自験例と本邦報告例の文献的考察—

概要

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Congenital factor V deficiency is a relatively rare disease and up to now, only 28 cases (20 families) have been reported in Japan.A report was made of 6-year-old Japanese boy with a bleeding tendency charactarized by excessive bleeding from the operation wound, after tooth extraction, and following injury. Familial history revealed that father, paternal uncle, and grandfather suffered from hemorrhagic symptoms. The hemostatic findings showed a prolongation of whole blood clotting time, one stage prothrombin time, and partial thromboplastin time. On thromboplastin generation test using human brain extract, the patient's absorbed plasma showed a delay with a normal terminal yield. Circulating anticoagulant was not present. Fibrinolysis was normal. Factor V activity was remarkably reduced. (3% of the normal value) But Factor I, II, VII-complex, VIII, IX, X, and XI-XII were within normal range. A diagnosis of congenital facton V deficiency was made.The properties of the deficient factor in the patient were pursued. A review of the previously reported 28 cases of congenital factor V deficiency was made.
一般社団法人日本血液学会の論文