A CRM‐Positive Variant of Factor‐VII Deficiency and the Detection of Heterozygotes with the Assay of Factor‐like Antigen

Publisher Website
Google Scholar
Abstract
Nine patients with severe factor-VII deficiency, belonging to 7 pedigrees were studied for the presence of factor-VII-CRM [cross reacting material] with an inhibitor neutralization assay. The antibody, raised in rabbits, did not precipitate the antigen and could only be used in a fluid phase assay to measure the capacity of plasma to neutralize inhibitory activity directed against factor-VII activity. In 1 of the 9 patients normal amounts of factor-VII-CRM could be demonstrated. The CRM+ patient did not show a clinical picture at variance with that of the CRM- patients. The investigation into this CRM+ pedigree revealed heterozygosity in nine gosity in 9 out of 12 persons when using the ratio between biological factor-VII activity and factor-VII-CRM as the criterion.