A patient was referred for investigation of heavy bleeding after surgery. He showed several features of hypothyroidism but no goitre. Primary autoimmune hypothyroidism was confirmed by the finding of a low serum thyroxine concentration and a high thyrotropin concentration. Factor VIII concentration was low, and a mild coagulation inhibitor was found. The patient was treated with thyroxine and r...

Factor VII circulates as a single chain inactive zymogen (10 nmol/L) and a trace ( approximately 10-100 pmol/L) circulates as the 2-chain form, factor VIIa. Factor VII and factor VIIa were studied in a coagulation model using plasma concentrations of purified coagulation factors with reactions initiated with relipidated tissue factor (TF). Factor VII (10 nmol/L) extended the lag phase of thromb...

Acquired haemophilia A (AHA) is a rare hemorrhagic disease caused by an autoantibody against coagulation factor VIII. Nonhaemophiliac patients develop autoantibodies (inhibitors) directed against the factor VIII circulating coagulation protein. Disease is associated with an increased morbidity and mortality. Inhibitors against FVIII induce acute and life-threatening hemorrhagic diathesis becaus...

BACKGROUND Elevated plasma levels of coagulation factor VIII are a strong risk factor for pulmonary emboli and deep venous thromboses. OBJECTIVES To identify reversible biomarkers associated with high factor VIII and assess potential significance in a specific at-risk population. PATIENTS/METHODS 609 patients with hereditary haemorrhagic telangiectasia were recruited prospectively in two se...

Coagulation is triggered during the onset of myocardial infarction, resulting in vascular occlusion. However, a causal role for individual haemostatic factors in the development of thrombotic occlusion is not established. Three cases (all relatively young women) are reported of raised factor VIII associated with myocardial infarction. Two patients presented acutely with myocardial infarction at...

Coagulation is triggered during the onset of myocardial infarction, resulting in vascular occlusion. However, a causal role for individual haemostatic factors in the development of thrombotic occlusion is not established. Three cases (all relatively young women) are reported of raised factor VIII associated with myocardial infarction. Two patients presented acutely with myocardial infarction at...

Hemophilia A is a rare inherited bleeding disorder due to mutation of the gene that encodes the coagulation protein factor VIII. Historically, prior to the availability of treatment with factor VIII preparations, most boys died from uncontrolled bleeding, either spontaneous bleeding or after injury, before reaching 20 years of age. One of the most impressive triumphs of modern medicine is that ...

A 51-year-old patient with haemophilia since childhood (usual factor VIII level 14%) developed acute viral hepatitis type B two months after an operation which had been covered by cryoprecipitate. The course of the hepatitis following admission was severe with encephalopathy and ascites. Evidence of intravascular coagulation with an increased radioactive fibrinogen turnover was also present. Th...

Acquired inhibitors to coagulation factors other than factor VIII are extremely rare. We describe a case of a 59-year-old woman with abnormal bleeding, diagnosed with concurrent inhibitor antibodies to factor VIII and IX by Bethesda testing. We demonstrate that anti-FVIII antibodies of a very high titre are capable of disturbing the aPTT-based Bethesda assay, resulting in falsely-positive antib...

Coagulation factor VIII (FVIII) is a cofactor in the intrinsic pathway of blood coagulation for which deficiency results in the bleeding disorder hemophilia A. FVIII contains a domain structure of A1-A2-B-A3-C1-C2 of which the B domain is dispensable for procoagulant activity in vitro. In this report, we compare the properties of B-domain-deleted FVIII (residues 760 through 1639, designated LA-...