[PDF][PDF] A familial hemorrhagic trait associated with a deficiency of a clot-promoting fraction of plasma

OD Ratnoff, JE Colopy - The Journal of clinical investigation, 1955 - Am Soc Clin Investig
OD Ratnoff, JE Colopy
The Journal of clinical investigation, 1955Am Soc Clin Investig
Recently, three patients have been studied in whom the clotting time ofvenous blood was
greatly prolonged. None of the patients had significant hemorrhagic symptoms. The trait was
apparently familial and appeared in both sexes. The patients' disorder could not be
identified with any known bleeding disease. However, in each case, the plasma was
deficient in a substance found in normal globulin which accelerated the clotting of normal,
platelet-deficient plasma. This clot-promoting fraction, present in heated, barium sulfate …
Recently, three patients have been studied in whom the clotting time ofvenous blood was greatly prolonged. None of the patients had significant hemorrhagic symptoms. The trait was apparently familial and appeared in both sexes. The patients' disorder could not be identified with any known bleeding disease. However, in each case, the plasma was deficient in a substance found in normal globulin which accelerated the clotting of normal, platelet-deficient plasma. This clot-promoting fraction, present in heated, barium sulfate-adsorbed serum, was neither thrombic nor thromboplastic and did not alter the rate of conversion of fibrinogen to fibrin by thrombin, nor of prothrombin to thrombin by tissue throm-boplastin. By inference, its effect was upon an earlier stage of clotting, presumably upon the de-velopment of thromboplastic activity in shed blood. However, the fraction did not appear to contain any of the components of plasma known to be necessary for the optimal evolution of plasma thromboplastic activity. The characteristics of the clot-promotingfraction, deficient in the plasma of these threepatients, suggests that its properties may be identical with those of an unidentified accelerator of clotting describedin an earlier study (1). Tentatively, untilits mode of action becomes clear, this fraction maybe named Hageman factor, after Patient I.
The Journal of Clinical Investigation