[Successful induction of immune tolerance and novel hemostatic effects in a hemophilia A with high-responder inhibitor by regular infusions of factor VIII]

Rinsho Ketsueki. 1996 Nov;37(11):1303-8.
[Article in Japanese]

Abstract

A hemophilia A patient with high responder inhibitor had been treated by (activated) prothrombin complex concentrates (A) PCC and activated factor VII until the occurrence of intracranial bleeding at the age of 6 years. Since the inhibitor titer was decreased less than 1 Bethesda Units/ml, high dose of factor VIII was given followed by the infusions of factor VIII concentrates (100 units/kg) three times a week. In spite of previous episodes of anamnestic responses by factor VIII products before, the inhibitor titer did not increase and disappeared completely 6 months after the FVIII infusion therapy. The specific anti-factor VIII IgG subclasses of the inhibitor were IgG2 and IgG4. The inhibitor recognized both light and heavy chains. He have no bleeding episode for 6 months since the beginning of the prophylactic with factor VIII concentrates.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Blood Coagulation Factors / administration & dosage*
  • Child
  • Factor VIII / administration & dosage*
  • Factor VIII / antagonists & inhibitors
  • Hemophilia A / blood
  • Hemophilia A / immunology*
  • Hemophilia A / therapy*
  • Hemostasis*
  • Humans
  • Immune Tolerance*
  • Immunoglobulin G / analysis
  • Male
  • Quality of Life

Substances

  • Blood Coagulation Factors
  • Immunoglobulin G
  • prothrombin complex concentrates
  • Factor VIII