[Successful induction of immune tolerance and novel hemostatic effects in a hemophilia A with high-responder inhibitor by regular infusions of factor VIII].
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.
Shima
M
M
Department of Pediatrics, Nara Medical University, Japan.
Sawamoto
Y
Y
Kamisue
M
M
Shibata
Y
Y
Tuzi
S
S
Kuwabara
M
M
Tanaka
I
I
Tanizawa
T
T
Tanaka
A
A
Ueda
M
M
Kakishita
E
E
Yoshioka
A
A
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
D001779:Blood Coagulation Factors; D007074:Immunoglobulin G; C025667:prothrombin complex concentrates; D005169:Factor VIII