Plasma Thromboplastin Component (PTC) A Hitherto Unrecognized Blood Coagulation Factor Case Report of PTC Deficiency

Abstract

1. A new hemorrhagic disease characterized by a prolonged whole blood coagulation time caused by the delayed formation of thrombin is described. 2. The patient suffering from this disease was found to have normal concentrations of all previously described coagulation factors. 3. Because the coagulation defect could be completely corrected by the addition of tissue thromboplastin or platelet-poor hemophilic plasma and because tests for prothrombin and prothrombin conversion accelerators gave normal results and prothrombin utilization was impaired, it was concluded that the missing factor was concerned with the production of thromboplastin in the plasma. Accordingly it was named the plasma thromboplastin component (PTC). 4. The clinical and laboratory resemblance of PTC deficiency and hemophilia is striking. However, the two diseases can be distinguished by the fact that normal serum, the 45 to 50 per cent saturated ammonium sulfate fraction of normal plasma, the citrate eluate from barium sulfate, which has been mixed with normal plasma or serum, and hemophilic plasma correct PTC deficiency but are ineffective in hemophilia. On the other hand, while the 33 per cent saturated ammonium sulfate fraction of normal plasma, barium sulfate adsorbed normal plasma, Cohn's Fraction I and PTC-deficient plasma correct the hemophilic defect, they are ineffective in PTC deficiency. 5. A method for the partial purification and concentration of the PTC factor is described.

Physiological Basis for Transfusion Therapy in Hemorrhagic Disorders: A Critical Review

“…There are no other reliable studies in the literature regarding the disappearance rate of factor IX, although, since the original description of the severe congenital deficiency of this factor, the prolonged effect of transfusion on clotting time and other coagulation parameters has been noted.s. 98 Factor X: Graham41 studied the disappearance rate of factor X in a patient with severe congenital deficiency of this factor. Although there was considerable scatter of the points, the disappearance rate of factor X, studied over a period of five days, appeared to be a single exponential function with a half-life of approximately 47 hours.+…”

Intermediate Stages in the Formation of Blood Thromboplastin

“…This reagent was prepared from normal serum by the method of White, Aggeler and Glendening (1953). The Factor-VII activity of the serum is destroyed by exposing the serum to a pH of 2.9 for 2 hours.…”

The Preparation for Therapeutic Use of a Concentrate of Factor IX Containing also Factors II, VII and X

“…The practical difficulties are the loss of the red cells to the transfusion service, loss of Factor VIII, and technical difficulty in obtaining a satisfactory yield of serum. White, Aggeler and Glendening (1953) reported that a potent preparation of Factor IX could be made from serum by adsorption with BaSO, from which the factor was eluted with citrate but this preparation was 'unsuitable for intravenous administration in the human subject'. For a time the Paris group made a therapeutic fraction from serum which they called CSB (Soulier, Blatrix, Prou-Wartelle and Vignal, 1962) by a method similar to that which they use for plasma, but this separate preparation has now been discontinued.…”