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Mechanisms of thrombocytopenia in platelet-type von Willebrand disease
Platelet-type von Willebrand disease is an inherited platelet disorder characterized by thrombocytopenia with large platelets caused by gain-of-function variants in GP1BA leading to enhanced GPIbα-von Willebrand factor (vWF) interaction.
Loredana Bury +5 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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Molecular diagnosis of patients with von Willebrand disease is pending in most populations due to the complexity and high cost of conventional molecular analyses.
Nina Borràs +48 more
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von Willebrand Factor (VWF) Inhibitors in Two Brothers with von Willebrand Disease: A Case Report [PDF]
The development of inhibitors to von Willebrand factor (VWF) is a rare but potentially serious complication of VWF replacement therapy in patients with von Willebrand disease (VWD).
Claudia Djambas Khayat +4 more
doaj +2 more sources
ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease.
BACKGROUND von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Accurate and timely diagnosis presents numerous challenges.
P. James +18 more
semanticscholar +1 more source
ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease.
BACKGROUND von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients.
Nathan T Connell +18 more
semanticscholar +1 more source
Evaluation of a new semi-automated Hydragel 11 von Willebrand factor multimers assay kit for routine use [PDF]
Background: Accurate diagnosis and classification of von Willebrand disease (VWD) are essential for optimal management. The von Willebrand factor multimers analysis (VWF:MM) is an integral part of the diagnostic process in the phenotypic classification ...
Pikta Marika +6 more
doaj +1 more source
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
openaire +5 more sources
1. Stacy Cooper, MD 2. Clifford Takemoto, MD 1. Charlotte Bloomberg Children’s Center, Johns Hopkins Hospital Baltimore, MD 1. 1. Nichols W, 2. Hultin MB, 3. James AH, 4. et al Von Willebrand Disease (VWD): Evidence-Based Diagnosis and Management Guidelines, The National Heart, Lung, and Blood Institute (NHLBI) Expert
Stacy Cooper, Clifford M. Takemoto
+8 more sources

