We report a case of patient with recurrent hematomas while on anticoagulation for pulmonary embolism and prolonged hospital stay due to delayed diagnosis acquired hemophilia A. Acquired A is rare autoimmune bleeding disorder autoantibodies directed against coagulation factor VIII (FVIII), leading an FVIII deficiency. isolated activated partial thromboplastin time (aPTT) in warrants workup This ...

OBJECTIVE Both soluble endothelial protein C receptor (sEPCR) and factor VIII (FVIII) seem to be potential mediators in thrombotic and inflammatory states. The aim of the present study was to determine the relation between plasma sEPCR and FVIII levels in a group of healthy Turkish infants. METHODS The study population consisted of 50 healthy infants aged 6 months (Group 1, n=23) and 12 month...

Haemophilia A is a rare inherited bleeding disorder characterized by an inability of the blood to clot normally. Patients can experience spontaneous or trauma-induced joint and soft tissue bleeding and must keep coagulation factor VIII (FVIII) accessible at all times; thus, FVIII product storage and stability are critical. Our primary objective was to assess haemophilia A patients' and caregive...

UNLABELLED BACKGROUND & OBJECTIVE  The factor VIII (FVIII) B domain shares very little amino acid homology with other known proteins and is not directly necessary for procoagulant activity. Despite this, missense mutations within the B domain have been reported in patients with hemophilia A. Given that the B domain is dispensable for secretion and function of FVIII, we hypothesized that thes...

Preventive subcutaneous treatment of severe hemophilia A with bispecific antibodies that mimic the action coagulation factor VIII (FVIII) is emerging as an effective alternative to replacement therapy intravenous administration FVIII concentrates, either derived from plasma or produced by biotechnology. Access this innovative therapeutic approach for a growing number patients worldwide increasi...

The immunogenicity of therapeutic factor VIII (FVIII) in patients with haemophilia A remains a critical issue in patient management. This review describes the immunological processes involved in the activation of the immune system against FVIII, with a particular focus on the role of endocytic receptors for the recognition of FVIII by antigen-presenting cells.

SUMMARY An EMEA expert meeting on FVIII products and inhibitor development was held on 28 February to 2 March 2006 to provide a forum to discuss the international standardisation and harmonisation of requirements for clinical studies on FVIII inhibitor development in haemophilia A patients. The long-term objective is to collect comparable clinical data on the immunogenicity of recombinant and p...

Approximately 25% of hemophilia A patients infused with hemophilic plasmas, but only 33% (7 of 21) of autoantibody factor VIII (fVIII) mount an immune response, which leads plasmas. These results demonstrated that the inhibitor reto its inactivation. Anti-fVIII autoantibodies are also seen sponse in hemophiliacs was more complex and the epitope rarely in individuals with normal fVIII. We have p...

In mild/moderate haemophilia A (MHA) patients, many factor VIII (FVIII) gene defects, mainly missense mutations, have been identified and greatly improved the understanding of the structure and function of FVIII molecule. Characterization of the molecular mechanisms involved in MHA has helped to identify regions critical for proper FVIII biosynthesis, thrombin activation, intramolecular stabili...