Up until recently, von Willebrand Factor (VWF) structure only been studied through in vitro transfer, which allows transient expression of a transgene by mouse hepatocy important shift in VWF research. Indeed this approach has now enabled us to transiently express a number of VWF mutants in VWF residues in different aspects of VWF biolo reproducing various types of von Willebrand disease have b...

Epinephrine infusion causes variable increases in the components of the Factor VIII (antihemophilic factor) complex in patients with von Willebrand's disease. The increase in antihemophilic factor procoagulant activity was greater than that of Factor VIII-related antigen and von Willebrand factor activity in two patients with von Willebrand's disease. Similar increases in the three individual f...

von Willebrand factor (vWF) is a multimeric adhesive glycoprotein essential for normal hemostasis. We have discovered that cultured human umbilical vein endothelial cells incorporate inorganic sulfate into vWF. Following immunoisolation and analysis by polyacrylamide or agarose gel electrophoresis, metabolically labeled vWF was found to have incorporated [35S]-sulfate into all secreted multimer...

A 41-year-old male underwent allogeneic bone marrow transplantation for the treatment of acute myelogenous leukemia. Six months later, he was admitted to a hospital with signs and symptoms consistent with worsening chronic graft-vs-host disease. Despite a negative past history for a bleeding diathesis, the patient was found to have absent factor VIII procoagulant and ristocetin cofactor activit...

Recent progress in the biochemical characterization of coagulation factors VIII and IX has greatly contributed to our understanding of the inheritance of hemophilia and von Willebrand's disease and facilitated the recognition of carriers of these disorders. Factor VIII is a molecular complex which may be quantitated immunologically as factor VIII-related antigen. Within this complex reside the ...

The platelet function analyzer PFA-100 (Dade International Inc., FL, USA) is a relatively novel method for rapid in vitro global evaluation of primary hemostasis.1 Since the PFA-100 has been shown to be very sensitive to platelet and von Willebrand factor (VWF) levels and function, it is useful mainly as a screening test for von Willebrand’s disease (VWD) and several platelet disorders.2,3 Rece...

Hydroxyethyl starch is commonly used as a plasma volume expander in the surgical patient. Although it is generally considered a safe plasma substitution, some reports of an acquired von Willebrand's disease-like syndrome have been documented. To examine this further, von Willebrand factor: ristocetin cofactor activity (RCoF) was measured in two groups of patients perioperatively, following hydr...

This study prospectively evaluated the rate of biologic response to desmopressin (DDAVP) in 66 patients with type 1 or 2 von Willebrand disease (VWD), each of whom had, on the basis of available records, a clinically significant bleeding history and at least one of the following laboratory abnormalities: bleeding time (BT) longer than 15 minutes, ristocetin cofactor activity (VWF:RCo) less than...