Congenital SPCA deficiency: a hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fractions.
نویسندگان
چکیده
The importance of non-prothrombin plasma and serum constituents in the physiological conversion of prothrombin to thrombin has been recognized only recently. A hemorrhagic disorder, called "parahemophilia" (Owren's disease), is associated with an elevated prothrombin time attributable to deficiency of a plasma component termed "Factor V" (1, 2). Inadequacy of plasma Ac-globulin, required for rapid evolution of thrombin, has been described by Sykes and associates (3) in experimentally induced liver poisoning, and Alexander and Goldstein have reported on Labile Factor (Quick) deficiency in severe hepatic disease in man (4). Although conclusive evidence is lacking, most investigators agree that Factor V, Acglobulin, and Labile Factor are identical. Subnormal concentrations result in retarded prothrombin conversion (elevated one-stage prothrombin time), in poor yield of thrombin by the two-stage prothrombin method, and in hemorrhagic phenomena despite the fact that the prothrombin level may actually be normal. The clinical and laboratory investigation of a unique case herein reported provides substantial evidence that an additional constituent besides that (or those) described above is involved in prothrombin conversion, and that its lack may underlie a hemorrhagic diathesis, hitherto unrecognized, which simulates hypoprothrombinemia. Deficiency of Ac-globulin, Labile Factor, or Factor V were excluded as the basis for the clotting abnormality. The evidence warrants the conclusion that the defect consisted in derangement of the early activation of prothrombin due to congenital inadequacy of the precursor of the serum prothrombin conversion
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ورودعنوان ژورنال:
- The Journal of clinical investigation
دوره 30 6 شماره
صفحات -
تاریخ انتشار 1951