background: factor viii administration to hemophilia a patients results in an immune response (inhibitor formation) which significantly complicates the therapy. the present study was performed to determine the prevalence of inhibitor development in hemophilia a patients receiving recombinant factor viii therapy. materials and methods: this was an observational descriptive study. clotting factor...

Background: Factor VIII administration to hemophilia A patients results in an immune response (inhibitor formation) which significantly complicates the therapy. The present study was performed to determine the prevalence of inhibitor development in hemophilia A patients receiving recombinant factor VIII therapy. Materials and Methods: This was an observational descriptive study. Clotting fac...

Data have been collected from 47 centers in Europe and North America on the treatment with porcine factor VIII concentrate of 74 acute bleeding episodes in 65 patients with acquired hemophilia. The median initial anti-human factor VIII auto-antibody inhibitor level was 38 Bethesda unit (BU)/mL (range 1.2 to 1,024) whereas that against porcine was 1 BU/mL (range 0 to 15). The mean initial dose o...

A decrease in inhibitor titer has been reported in some patients with acquired factor VIII inhibitors treated with intravenous gammaglobulin (IGIV). We have conducted a prospective, multicenter study of high-dose IGIV in treatment of this disorder to determine efficacy. Nineteen patients received induction therapy with IGIV, 1,000 mg/kg x 2 consecutive days or 400 mg/kg x 5 consecutive days, fo...

Autoantibodies against factor VIII (FVIII) are rare but can cause life-threatening bleeding requiring costly factor replacement and prolonged immunosuppression. We report 4 consecutively treated patients whose acquired FVIII inhibitors responded rapidly to immunosuppressive regimens that included rituximab, a monoclonal antibody against CD20(+) B cells. Three patients had spontaneously occurrin...

daily for 1 week), pulse therapy with methylprednisolone (1000 mg/day for 3 days) and rituximab (100 mg/week for 2 weeks) were initially used for correcting the coagulopathy. Prednisolone (1 mg/kg/day) and cyclophosphamide (100 mg/ day) were prescribed as maintenance therapy. The coagulatory disorder improved after 2 months of treatment associated with reduction of factor VIII inhibitor to 35 B...

OBJECTIVE To determine the frequency of factor VIII specific inhibitors in haemophilia A. STUDY DESIGN Cross-sectional study. PLACE AND DURATION OF STUDY National Institute of Blood Disease and Bone Marrow Transplantation, Karachi, from August 2007 to March 2009. METHODOLOGY Venous blood samples of diagnosed haemophilia A patients were collected in tubes containing 0.109 M (3.2%) trisodiu...

The factor VIII C2 domain is essential for binding to activated platelet surfaces as well as the cofactor activity of factor VIII in blood coagulation. Inhibitory antibodies against the C2 domain commonly develop following factor VIII replacement therapy for hemophilia A patients, or they may spontaneously arise in cases of acquired hemophilia. Porcine factor VIII is an effective therapeutic fo...

Variations of the partial thromboplastin time (PTT) were tested to determine the best screening method for detection of inhibitors of factor VIII. Variables tested included the duration of preincubation of a mixture of patient plasma and factor VIII source (normal plasma), the ratio of the patient plasma to the normal plasma, and the duration of incubation of the normal plasma-patient plasma mi...