Von Willebrand Disease (VWD) questions
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Questions and Answers

Which of the following is NOT a known function of von Willebrand factor (vWF)?

  • Facilitating platelet aggregation and adhesion
  • Acting as a chaperone protein for Factor VIII
  • Promoting the breakdown of fibrin clots (correct)
  • Inhibiting RANKL-induced osteoclastogenesis

A patient presents with a history of easy bruising, frequent nosebleeds, and heavy menstrual bleeding. These symptoms are most indicative of which bleeding disorder?

  • Hemophilia A
  • Von Willebrand Disease (correct)
  • Factor V Leiden
  • Disseminated Intravascular Coagulation (DIC)

A patient with type O blood is being evaluated for a bleeding disorder. Which of the following statements is most accurate regarding the interpretation of their vWF levels?

  • vWF levels are always consistent across all blood types, but factor VIII levels are altered in type O.
  • Type O individuals typically have lower vWF levels, which should be considered when interpreting results. (correct)
  • Type O individuals typically have higher vWF levels, so a lower threshold should be used for diagnosis.
  • Blood type has no impact on vWF levels; therefore, standard reference ranges should be used.

Desmopressin (DDAVP) is a common treatment for some types of Von Willebrand Disease (VWD). What is the primary mechanism of action of Desmopressin in treating VWD?

<p>Induces the release of vWF and Factor VIII from endothelial cells. (A)</p> Signup and view all the answers

In which type of Von Willebrand Disease (VWD) is agarose gel electrophoresis least likely to detect any vWF multimers?

<p>Type 3 VWD (D)</p> Signup and view all the answers

Which type of VWD is characterized by hyperfunctional platelet binding?

<p>Type 2B (A)</p> Signup and view all the answers

A patient diagnosed with aortic stenosis develops acquired Von Willebrand Disease (VWD). Which pathophysiological mechanism is MOST likely responsible for the development of VWD in this scenario?

<p>Mechanical destruction of vWF multimers due to high shear stress. (A)</p> Signup and view all the answers

A 25-year-old female patient with a history of menorrhagia is suspected of having a bleeding disorder. Initial lab results show normal platelet count, PT, and INR, but prolonged PTT. Further testing reveals decreased levels of vWF antigen and activity. Which of the following genetic inheritance patterns would make Type 3 VWD most likely in this patient?

<p>Autosomal recessive (A)</p> Signup and view all the answers

During an investigation into a qualitative VWD, a lab technician performs an in vitro platelet aggregation study. They observe that ristocetin-induced platelet aggregation is significantly enhanced. Which subtype of VWD is most likely indicated by this finding?

<p>Type 2B (D)</p> Signup and view all the answers

A researcher is investigating novel therapeutic targets for VWD. They hypothesize that inhibiting a specific enzyme could prevent the degradation of vWF multimers under high shear stress conditions. Which of the following enzymes would be the MOST rational target for this therapeutic intervention?

<p>ADAMTS13 (B)</p> Signup and view all the answers

Flashcards

Von Willebrand Disease (VWD)

The most common inherited bleeding disorder, affecting roughly 1% of the population, caused by a defect in the von Willebrand factor (vWF).

Von Willebrand Factor (vWF)

A glycoprotein that facilitates platelet aggregation and adhesion and acts as a chaperone for factor VIII.

Type 1 VWD

Mild quantitative defect where there is a decreased amount and activity of vWF; about 80% of cases.

Type 2 VWD

Qualitative or functional defect where vWF is present but not functioning correctly; further classified into subtypes.

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Type 3 VWD

Severe total quantitative defect where no vWF is produced; the rarest and most severe form.

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Desmopressin (DDAVP)

A synthetic vasopressin analog used for type 1 and some type 2 VWD, as it induces the release of vWF and factor VIII from endothelial cells.

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Acquired VWD

A condition characterized by the development of vWD due to another underlying condition, such as aortic stenosis or Wilms tumor.

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Heyde's Syndrome

A condition associated with aortic stenosis where shear stess destroys vWF.

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Description

Test your knowledge of Von Willebrand Disease (VWD) with these questions. Questions cover symptoms, diagnosis, treatment, and classification of VWD. Review vWF function, DDAVP mechanisms, and disease pathophysiology.

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