Factor VII congenital deficiency. Clinical picture and classification of the variants

Haemostasis. 1983;13(3):169-77. doi: 10.1159/000214723.


In congenital factor VII deficiency the clinical picture is related to the levels of factor VII coagulant activity; when factor VII:C levels are very low the bleeding episodes can occur frequently. The most frequent bleedings are menorrhagia and metrorrhagia in females and hemarthrosis in both sexes. There are 3 immunological variants of factor VII deficiency: VII-, VIIR and VII+. Conversely, the genetic variants are 2: one characterized by no discrepancy between VII:C and VII:Ag (found in the heterozygotes of VII- and VIIR variants) and the other, in which a discrepancy between VII:C and VII:Ag is found (heterozygotes from VII+ kindreds). In factor VII deficiency, most commonly the human and ox tissue factors show the highest sensitivity to the coagulation defect, whereas the one extracted from rabbits is definitely less sensitive; the definition of functional variants is based upon a different reactivity to homologous and/or heterologous tissue thromboplastins. In no case was a PIVKA-VII-like protein found and none of the factor VII defective molecules reacted to the generation of important kallikrein activity.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Antigens / analysis
  • Antigens / genetics
  • Blood Coagulation Tests
  • Cattle
  • Chromosome Aberrations / diagnosis
  • Chromosome Disorders
  • Chromosomes, Human, 6-12 and X
  • Cross Reactions
  • Factor VII / genetics*
  • Factor VII / immunology
  • Factor VII Deficiency / congenital*
  • Factor VII Deficiency / diagnosis
  • Factor VII Deficiency / genetics
  • Female
  • Genetic Carrier Screening
  • Genetic Variation*
  • Humans
  • Male
  • Rabbits
  • Thromboplastin / physiology


  • Antigens
  • Factor VII
  • Thromboplastin