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2,102 results
  • Updated overview on von Willebrand disease: focus on the interest of genotyping. [Journal Article]
    Expert Rev Hematol 2019Itzhar-Baikian N, Boisseau P, … Veyradier A
  • Introduction: Von Willebrand disease (VWD) is the most common inherited bleeding disorder, characterized by a quantitative or qualitative defect of von Willebrand factor (VWF), a multimeric glycoprotein crucial for primary hemostasis and coagulation. VWD pathophysiology is heterogeneous as it includes several types and subtypes which therapeutic management is different. The mainstays of VWD treat…
  • Successful Desensitization of a Patient with Possible IgE-Mediated Anaphylactic Reaction to FVIII/VWF Concentrate. [Case Reports]
    Pediatr Allergy Immunol Pulmonol 2019; 32(2):81-84Beken B, Celik V, … Yazicioglu M
  • CONCLUSIONS: Anaphylactic reaction to factor replacement products is a major problem for patients with VWD, especially type 3 VWD requiring multiple factor infusions. We achieved a successful desensitization with FVIII/VWF concentrate in a patient who had an anaphylactic reaction during the infusion of this product. Our patient is important since she represents the first case of IgE-mediated anaphylaxis against VWF concentrate reported in the literature.
  • How I manage severe von Willebrand disease. [Review]
    Br J Haematol 2019Leebeek FWG, Atiq F
  • Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Most patients with mild and moderate VWD can be treated effectively with desmopressin. The management of severe VWD patients, mostly affected by type 2 and type 3 disease, can be challenging. In this article we review the current diagnosis and treatment of severe VWD patients. We will also discuss the management of sever…
  • A personalized approach to the management of VWD. [Journal Article]
    Transfus Apher Sci 2019Phua CW, Berntorp E
  • The goal of treating Von Willebrand Disease (VWD) is to replace deficient or dysfunctional Von Willebrand Factor (VWF) protein. However, the choice of treatment has to be considered carefully in view of patient factors and the unique properties of replacement products. Tailoring a treatment plan to an individual patient's bleeding challenge is an intricate process. This review describes personali…
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