Tissue factor pathway inhibitor (TFPI) blocks thrombin generation via the extrinsic blood coagulation pathway. Because the severe bleeding in patients with hemophilia occurs from deficiency of intrinsic blood coagulation pathway factor VIII or IX, pharmacological agents that inactivate TFPI and, therefore, restore thrombin generation via the extrinsic pathway, are being developed for treatment ...

Skin necrosis caused by heparins is a rare complication. We report a case of a 71-year-old woman who developed the unusual complication of focal necrotizing skin lesions in the injection site after undergoing thromboprophylaxis with enoxaparin after right knee arthroplasty. Laboratory investigations for heparine-induced thrombocytopenia (HIT), disseminated intravascular coagulation, protein C, ...

Bleeding symptoms are common in healthy children but occasionally may indicate an underlying congenital or acquired bleeding diathesis. The rare bleeding disorders (RBDs) comprise inherited deficiencies of coagulation factors I (congenital fibrinogen deficiencies), II, V, VII, X, XI, and XIII and combined factor deficiencies, most notably of factors V and VIII and of vitamin K-dependent factors...

Factor VIII C-domains are believed to have specific functions in cofactor activity and in interactions with von Willebrand factor. We have previously shown that factor VIII is co-targeted with von Willebrand factor to the Weibel-Palade bodies in blood outgrowth endothelial cells, even when factor VIII carries mutations in the light chain that are associated with defective von Willebrand factor ...

To test the hypothesis that factor VIII expressed in the epidermis can correct hemophilia A, we generated transgenic mice in a factor VIII-deficient background that express human factor VIII under control of the involucrin promoter. Mice from 5 transgenic lines had both phenotypic correction and plasma factor VIII activity. In addition to the skin, however, some factor VIII expression was detec...

factor v (fv) deficiency is a rare bleeding disorder (rbd) that inherit in autosomal recessive manner. diagnosis of fv deficiency (fvd) is made by routine coagulation tests, fv activity and molecular analysis. in patients with fvd, routine coagulation tests including activated partial thromboplastin time (aptt), prothrombin time (pt) and evenbleeding time (bt) are prolongedwhile thrombin time (...

OBJECTIVE Previous work has shown that platelets stimulated with the combination of thrombin and convulxin, a glycoprotein VI agonist, develop 2 populations with different levels of alpha-granule factor V bound to the platelet surface. To evaluate whether this phenomenon is restricted to alpha-granule components or is a feature that can be generalized to other coagulation factors, we studied th...