Abstract
Summary: An Italian family has been investigated in whom there was an inherited abnormality in the activity of coagulation factor X. This abnormality was confined to the intrinsic activation pathway, giving abnormal results in tests of this system, while the extrinsic pathway tested with tissue thromboplastin and Russell's viper venom, was normal. This is a previously undescribed abnormality, as other cases reported have had defective activity in the extrinsic pathways of coagulation. Immunological studies, using antibody neutralization techniques, revealed a normal concentration of factor X protein. The abnormal coagulation factor was not associated with a bleeding tendency. The inheritance pattern appeared to be of the autosomal recessive type.
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