Summary
Laboratory investigations are described in a female patient who suffers from a congenital hæmorrhagic disorder due to deficiency of Factor VII. The one‐stage prothrombin time using brain extract was greatly prolonged, but using viper venom was normal. All tests of the intrinsic blood coagulation mechanism, including the thromboplastin generation test, were normal. The patient provides the eighth recorded case of Factor VII deficiency with this pattern of laboratory findings.
Evidence is presented which supports the concept that Factor VII consists of at least two components, deficiency of either producing a prolonged one‐stage prothrombin time using brain extract. Cases reported in the literature of Factor VII deficiency associated with an abnormal thromboplastin generation test and prolonged plasma venom time probably represent deficiency of the second component. The Factor VII component deficient in this patient is reduced early in therapy with “Dindevan”.
The patient has three children, all with reduced concentrations of Factor VII. It is considered that the children represent the heterozygous state of Factor VII deficiency and that the defect is transmitted in a recessive manner.