Haemophilia A and B are hereditary X-linked disorders due to deficiency (or absence) of coagulation factor VIII or IX, respectively. Bleeding risk is related to the severity of factor deficiency. Repeated joint bleeding can lead to a severe haemophilic arthropathy resulting in disabilities. Outcome measurements in persons with haemophilia (PWH) have been limited to laboratory evaluation (factor...

Investigations were undertaken of the chemical nature and kinetics of interaction of three acquired inhibitors of Factor VIII. The inhibition was stoichiometric, one molecule of inhibitor (or one site on a molecule) being required to inactivate one molecule of Factor VIII. All three inhibitors were found to be monotypic antibodies of class IgG, Type K (gamma(2) kappa(2)). This appears to be the...

BACKGROUND AND OBJECTIVES In patients with factor XI(FXI) deficiency the bleeding tendency is poorly correlated with plasma factor levels. The purpose of this study was to evaluate whether or not this discrepancy is also present in a large series of patients from Iran, a previously unexplored ethnic group. DESIGN AND METHODS In 28 FXI - deficient patients bleeding symptoms and their relation ...

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 ...