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Acquired von Willebrand syndrome: focused for hematologists
The acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings similar to those of inherited von Willebrand disease. However, unlike the inherited disease, AvWS occurs in persons with no personal and family history of ...
Massimo Franchini+1 more
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Diagnosis of platelet-type von Willebrand disease by flow cytometry
Platelet-type von Willebrand disease (PT-VWD) is a rare autosomal dominant bleeding disorder which is due to a mutation in the gene encoding for platelet glycoprotein Ibα (GPIbα) resulting in enhanced affinity for von Willebrand factor (VWF).
Silvia Giannini+3 more
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Considerable progress has been made in characterizing the specific molecular defects responsible for the heterogeneous disorder known as von Willebrand disease (VWD). A large number of molecular defects have been identified and precise characterization may now be possible in the majority of type 2A, type 2B, type 2N, and potentially also type 3 VWD ...
William C. Nichols, David Ginsburg
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Treatment of Von Willebrand Disease [PDF]
SummaryIn von Willebrand disease, there are two main options for the treatment of spontaneous bleeding episodes and for bleeding prophylaxis: desmopressin and transfusional therapy with plasma products. Desmopressin is the treatment of choice for most patients with type 1, who account for approximately 70 to 80 per cent of all cases with the disease ...
P M Mannucci
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Clinical utility of panel-based genetic sequencing for von Willebrand disease [PDF]
Background: von Willebrand disease (VWD) is the most prevalent inherited bleeding disorder with a wide spectrum of causative variants. Next-generation sequencing analyzes the entire VWF gene and provides concomitant assessment of other genes, allowing ...
Radha Ramanan+12 more
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Congenital infiltrating lipoma of the upper limb in a patient with von Willebrand disease [PDF]
Infiltrating lipoma is a rare variety of lipoma, characterized by an infiltration of the adipose tissue of the muscles. Infiltrating lipomas are usually classified in two groups: intermuscular infiltrating lipoma and intramuscular infiltrating lipoma ...
Jose María Lasso+4 more
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Pseudotumor of the Maxillary Sinus in a Child with Von Willebrand Disease [PDF]
Introduction:Mandibular pseudotumors, also known as blood cysts, are rare complications which occur more frequently in patients with an associated bleeding disorder such as hemophilia.Case Report: We present a case of a 2-year and 6-month-old patient ...
Martha Gutierrez Perez+4 more
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Rictor maintains endothelial integrity under shear stress
Background: Endothelial injury induced by low shear stress (LSS) is an initiating factor in the pathogenesis of various cardiovascular diseases, including atherosclerosis, hypertension, and thrombotic diseases.
Hui Li+9 more
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BackgroundAcquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary von Willebrand disease (VWD).
Songmi Wang+6 more
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von Willebrand's disease [PDF]
The common inheritable hemorrhagic disorder, von Willebrand's disease, has been known for only about 50 yr. During these years it has been well established that the cause of the disease is a deficiency of a plasma protein with remarkable biological properties. Future research should focus on details of the molecular structure of this protein.
Lars Holmberg, Inga Marie Nilsson
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