THE BASIC SCIENCE, DIAGNOSIS, AND CLINICAL MANAGEMENT OF VON WILLEBRAND DISEASE Second Edition
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منابع مشابه
The role of exon 45 and 16 in the pathogenesis of Von Willebrand disease in Iranian Patients
Abstract Background Von Willebrand disease (VWD) is an autosomal recessive congenital bleeding disorder with deficiency or dysfunction of von Willebrand factor (VWF). The gene encoding for the VWF is located on chromosome 12, which is 178 Kb with 52 exons. Various mutations of this gene is responsible for the clinical features of VWD, but some single nucleotide polymorphisms make the molecu...
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Von Willebrand disease is an inherited condition characterized by deficiency of von Willebrand factor, which is essential in hemostasis. The National Heart, Lung, and Blood Institute has released new evidence-based guidelines for the diagnosis and management of the disease. There are three major subtypes of von Willebrand disease, classified as partial quantitative deficiency (low levels) of vo...
متن کاملHow I treat How I treat von Willebrand disease
Recent multicenter studies have clarified the molecular basis underlying the different von Willebrand disease (VWD) types, all of which are caused by the deficiency and/or abnormality of von Willebrand factor (VWF). These studies have suggested a unifying pathophysiologic concept. The diagnosis of VWD, remains difficult because its clinical and laboratory phenotype is very heterogeneous and may...
متن کاملNew developments in the diagnosis and treatment of von Willebrand disease
von Willebrand disease (VWD) arises from deficiencies and/or defects in the plasma protein VWF. VWD is reportedly the most common inherited bleeding disorder. The classification scheme for VWD identifies six types, with type 1 VWD defining quantitative deficiencies of VWF, type 3 defining an absence of plasma VWF, and types 2A, 2B, 2M and 2N defining qualitative defects of VWF. Diagnosis requir...
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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...
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