Congenital factor X deficiency in Japan.

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Abstract
Congenital factor X deficiency is a very rare inherited coagulation abnormality. There were reported 43 cases of this disorder in the world and only 2 cases in Japan. Recently, as many as 3 cases of this rare disease, an 18 yr old male, on 11 yr old male and a 6 yr old female were examined hemostatically and immunologically. Hemostatic examinations revealed prolonged prothrombin time, partial thromboplastin time and decreased serum thromboplastic activity in these 3 cases. Stypven-cephalin clotting time was abnormal. Factor X activities of cases were low when assayed by either tissue thromboplastin and partial thromboplastin or by Stypven-cephalin mixture, which were 2.6, 1.5 and 4.5%, respectively. The half-lives of infused factor X were 24, 38.6 and 56 h, respectively. Immunological assay of factor X (radioimmunoassay) showed 0.47 .mu.g/ml in the 2nd case and 0.15 .mu.g/ml in the 3rd case, from which those cases were considered to be classical factor X deficiency.