A Clinical Evaluation of Plasma Thromboplastin Antecedent (PTA) Deficiency

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Abstract
This study indicates that PTA deficiency is the most common of the hemophilia or hemophilia-like disorders. A first-stage coagulation abnormality, exclusive of platelet disorders and paraproteinemic states, has been identified in 82 persons over the past 3 years. Fourteen patients had an AHF deficiency, 3 had a PTC deficiency, and 65 persons had a PTA deficiency. Experience, accumulated during an evaluation of the 65 PTA deficient patients in 43 families, supports the mode of inheritance as one of autosomal dominance with moderate variability in the expression of the gene. The usual pre-operative screening tests would have been normal in 34% of the PTA deficient patients. The unusual characteristics of apparent reversal to normal of PTA deficient plasma after storage at -20[degree]C is probably a reflection of slow progression of the diminished PTA present in the plasma of these patients to form activation product.
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