An inhibitor to factor VIII:C in a patient with possible combined haemophilia A and von Willebrand's disease

Thromb Haemost. 1986 Apr 30;55(2):158-61.

Abstract

A factor VIII inhibitor has been found in a patient with an unusual combination of factor VIII-related properties. The inhibitor is directed specifically against the clotting activity (VIII:C) of the factor VIII complex. It behaves in a similar fashion to high responding inhibitors of factor VIII seen in haemophilia A patients and it was characterised as an immunoglobulin of the IgG class. Laboratory results from the patient and his family show considerable variation of factor VIII-related properties between various individuals. Overall, the data suggests the co-existence of haemophilia A and von Willebrand's disease in the family and the presence of both diseases in the patient.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Factor VIII / antagonists & inhibitors*
  • Factor VIII / genetics
  • Factor VIII / isolation & purification
  • Genetic Variation
  • Hemophilia A / blood*
  • Hemophilia A / complications
  • Hemophilia A / pathology
  • Humans
  • Immunoenzyme Techniques
  • Infant
  • Male
  • Pedigree
  • von Willebrand Diseases / blood*
  • von Willebrand Diseases / complications
  • von Willebrand Diseases / genetics
  • von Willebrand Diseases / pathology

Substances

  • Factor VIII