title: [Successful induction of immune tolerance and novel hemostatic effects in a hemophilia A with high-responder inhibitor by regular infusions of factor VIII].
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.
authors.affiliation: Department of Pediatrics, Nara Medical University, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Shima
authors.forename: Y
authors.initials: Y
authors.lastname: Sawamoto
authors.forename: M
authors.initials: M
authors.lastname: Kamisue
authors.forename: Y
authors.initials: Y
authors.lastname: Shibata
authors.forename: S
authors.initials: S
authors.lastname: Tuzi
authors.forename: M
authors.initials: M
authors.lastname: Kuwabara
authors.forename: I
authors.initials: I
authors.lastname: Tanaka
authors.forename: T
authors.initials: T
authors.lastname: Tanizawa
authors.forename: A
authors.initials: A
authors.lastname: Tanaka
authors.forename: M
authors.initials: M
authors.lastname: Ueda
authors.forename: E
authors.initials: E
authors.lastname: Kakishita
authors.forename: A
authors.initials: A
authors.lastname: Yoshioka
mesh_terms: D001779:Blood Coagulation Factors / Q000008:administration & dosage*; D002648:Child; D005169:Factor VIII / Q000008:administration & dosage* / Q000037:antagonists & inhibitors; D006467:Hemophilia A / Q000097:blood / Q000276:immunology* / Q000628:therapy*; D006487:Hemostasis; D006801:Humans; D007108:Immune Tolerance; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D011788:Quality of Life
chemical_list: D001779:Blood Coagulation Factors; D007074:Immunoglobulin G; C025667:prothrombin complex concentrates; D005169:Factor VIII