title: Pathogenetic role of Factor VII deficiency and thrombosis in cross-reactive material positive patients.
abstract: Congenital Factor VII (FVII) deficiency can be divided into two groups: cases of "true" deficiency, or cross-reactive material (CRM) negative and variants that are cross-reactive material positive.The first form is commonly recognized as Type I condition whereas the second one is known as Type II. FVII deficiency has been occasionally associated with thrombotic events, mainly venous. The reasons underlying this peculiar manifestation are unknown even though in the majority of associated patients thrombotic risk factors are present. The purpose of the present study was to investigate if a thrombotic event was more frequent in Type I or in Type II defect.The majority of patients with FVII deficiency and thrombosis belong to Type II defects. In the following paper we discuss the possible role of the dysfunctional FVII cross-reaction material as a contributory cause for the occurrence of thrombosis.
authors.affiliation: Department of Medicine, University of Padua Medical School, Padua, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Girolami
authors.affiliation: Department of Medicine, University of Padua Medical School, Padua, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Sambado
authors.affiliation: Department of Medicine, University of Padua Medical School, Padua, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Bonamigo
authors.affiliation: Department of Medicine, University of Padua Medical School, Padua, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Ferrari
authors.affiliation: Department of Medicine, University of Padua Medical School, Padua, Italy.
authors.forename: A M
authors.initials: AM
authors.lastname: Lombardi
mesh_terms: D000293:Adolescent; D000328:Adult; D019943:Amino Acid Substitution; D005091:Exons; D005167:Factor VII / Q000235:genetics / Q000378:metabolism; D005168:Factor VII Deficiency / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D005260:Female; D005838:Genotype; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D013927:Thrombosis / Q000150:complications* / Q000175:diagnosis / Q000209:etiology; D055815:Young Adult
chemical_list: D005167:Factor VII
keywords: FVII activity; FVII antigen; FVII mutations; Thrombosis