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

What is the approximate prevalence of von Willebrand Disease (VWD) in the UK population?

<p>1% (A)</p> Signup and view all the answers

Which of the following is the primary function of von Willebrand Factor (vWF)?

<p>Promoting platelet adhesion and migration (D)</p> Signup and view all the answers

Which of the following inheritance patterns is MOST commonly associated with VWD?

<p>Autosomal dominant (D)</p> Signup and view all the answers

A patient with a known bleeding disorder requires a tooth extraction. Which local haemostatic measure is LEAST appropriate for managing bleeding risk?

<p>Aggressive rinsing with tranexamic acid mouthwash (A)</p> Signup and view all the answers

In a patient with VWD undergoing a minor surgical procedure, what is the generally accepted minimum platelet count to proceed safely, assuming no other complications?

<p>50 x 10^9/l (B)</p> Signup and view all the answers

Which of the following best describes the underlying mechanism of Factor V Leiden thrombophilia?

<p>Resistance to activated protein C (D)</p> Signup and view all the answers

A patient with acquired VWD secondary to liver disease is scheduled for a dental extraction. Which of the following coagulation factors is MOST likely to be affected due to the liver dysfunction?

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

What is the primary reason NSAIDs are contraindicated in patients with inherited bleeding disorders?

<p>NSAIDs irreversibly inhibit platelet function, prolonging bleeding time. (C)</p> Signup and view all the answers

A patient on DOACs requires flap-raising procedures. According to SDCEP guidelines, this falls under which risk category for bleeding?

<p>Higher risk (A)</p> Signup and view all the answers

A researcher is investigating a novel treatment for VWD Type 3. The therapeutic strategy aims to enhance the half-life of endogenous Factor VIII. Which of the following mechanisms would be MOST directly relevant to this therapeutic approach?

<p>Stabilizing the vWF-Factor VIII complex (A)</p> Signup and view all the answers

What is the most prevalent inherited bleeding disorder in the UK population?

<p>von Willebrand Disease (VWD) (C)</p> Signup and view all the answers

Von Willebrand Factor (vWF) is primarily found in which of the following locations within the body?

<p>Platelets and endothelial cell walls (C)</p> Signup and view all the answers

In Type 2 Von Willebrand Disease (VWD), what is the primary characteristic of the von Willebrand Factor (vWF) protein?

<p>vWF is present in normal quantities but is dysfunctional. (B)</p> Signup and view all the answers

Which of the following best describes the inheritance pattern most commonly associated with von Willebrand Disease (VWD)?

<p>Autosomal dominant (D)</p> Signup and view all the answers

A patient with von Willebrand Disease (VWD) is scheduled for a minor tooth extraction. Which local haemostatic agent is MOST appropriate for use in managing post-extraction bleeding?

<p>Tranexamic acid mouthwash (C)</p> Signup and view all the answers

Why are non-steroidal anti-inflammatory drugs (NSAIDs) generally contraindicated in patients with inherited bleeding disorders like von Willebrand Disease (VWD)?

<p>NSAIDs interfere with platelet function, exacerbating bleeding tendencies. (B)</p> Signup and view all the answers

In the context of dental procedures for patients on Direct Oral Anticoagulants (DOACs) as per SDCEP guidelines, which of the following procedures is classified as carrying a 'higher risk of bleeding'?

<p>Flap-raising procedures (B)</p> Signup and view all the answers

A patient with acquired von Willebrand Disease (VWD) secondary to severe liver disease is being evaluated. Dysfunction of the liver in this context MOST directly impacts which aspect of coagulation leading to acquired VWD?

<p>Decreased production of coagulation factors that stabilize vWF. (C)</p> Signup and view all the answers

A dental practitioner is considering treating a patient with known Type 3 Von Willebrand Disease (VWD). Which of the following factors is the MOST critical to ascertain prior to undertaking even a minimally invasive procedure, such as a single tooth extraction?

<p>Current platelet count and Factor VIII levels. (A)</p> Signup and view all the answers

In Factor V Leiden thrombophilia, the mutated Factor V protein exhibits resistance to inactivation by Activated Protein C (APC). Considering the role of Protein C in the coagulation cascade, what is the MOST direct consequence of this resistance on haemostasis?

<p>Prolonged activation of Factor V, resulting in sustained thrombin production. (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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Factor V Leiden Thrombophilia

A hypercoagulability disorder due to a mutation in Factor V, common in Europeans.

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Tranexamic Acid Mouthwash

Medication that can be used in VWD management. Need to be used very Gently and Wisely

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Inherited Bleeding Disorders

Haemophilia A, B, von Willebrand’s Disease, Factor V - Leiden

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Acquired Bleeding Disorders

Bleeding disorders may result secondary to Liver Disease (Factors II, VII, IX, X protein C and S, vitamin K, antithrombin).

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Bleeding Disorders - Avoid NSAIDs

Avoid non-steroidal anti-inflammatory drugs in patients with bleeding disorders.

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Management of VWD Patients

Try to identify cause. Reversible? Delay non-urgent surgery. Safe if Platelet Count > 50 x 10⁹/l.

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DOACs - Low Risk Bleeding

Simple Extractions (1-3 teeth), Incision and Drainage, Detailed Perio Examination, RSI, Restorations with sub-gingival margins

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DOACs - Higher Risk Bleeding

Complex/adjacent extractions, Flap-raising procedures, Gingival re-contouring, Biopsies

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Factor V Leiden

A condition where abnormal Factor V leads to hypercoagulability, affecting about 5% of Europeans.

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Local Haemostatic Measures

Always consider Suturing Sockets, use of Local Haemostatic Agents e.g., Resorbable Oxidised Cellulose ‘Surgicell’, Tranexamic Acid (anti-fibrinolytic) Mouthwash/on Gauze Packs.

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Mouthwash use?

Use Very Gently and Wisely - TRANEXAMIC ACID MOUTHWASH

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Minimally Traumatic Technique

Surgical procedure should be undertaken carefully to preserve tissue integrity and minimize trauma

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Study Notes

Von Willebrand Disease (VWD)

  • Requires consultation with a hematologist.
  • Most common inherited bleeding disorder, affecting 1% of the UK population.
  • Caused by a deficiency in Von Willebrand Factor (VWF), with varying degrees of severity.
  • The range of severities can vary massively.
  • Inheritance is usually autosomal dominant but can be sex-linked recessive, or acquired spontaneously through autoimmune disorders.
  • It can also be gained spontaneously.
  • VWF is a protein found in platelets and endothelial cell walls.
  • Affects both platelet adhesiveness and migration, impairing the formation of the primary platelet plug.
  • Clinical significance varies with the severity of the condition.

VWD Subtypes

  • Subtype 1 is the mildest and most common form (autosomal dominant).
  • Subtype 2: Normal VWF quantity but dysfunctional.
  • Subtype 3: Absence of VWF with low levels of Factor VIII.

VWD Management

  • Depends on: Severity, planned procedure, previous experiences, and subtype.
  • Treatment options: DDAVP, VWF factor replacement, Factor VIII supplementation, and antifibrinolytic drugs.
  • Tranexamic acid is an antifibrinolytic drug used in management.

Factor V Leiden Thrombophilia

  • Hypercoagulability condition caused by abnormal Factor V.
  • Affects 5% of Europeans and is inherited.
  • Deficient Factor 5 results in excessive bleeding, conversely, a broken Factor 5 may stop bleeding too much.

Principles for Bleeding Disorders

  • Effects range from no intervention needed to factor replacement.
  • Hematologist consultation is essential.
  • Always consider local hemostatic measures like suturing sockets, resorbable oxidized cellulose, and tranexamic acid mouthwash/gauze packs.
  • Tranexamic acid mouthwash needs to be used gently and wisely.
  • Employ minimally traumatic techniques; refer if necessary.

Bleeding Disorders - Avoid NSAIDs

  • Inherited disorders include: Hemophilia A, B, von Willebrand’s Disease, Factor V Leiden.
  • Acquired disorders are secondary to liver disease affecting Factors II, VII, IX, X protein C and S, vitamin K, and antithrombin.
  • Review if the patient is on anticoagulant therapy.

Management of VWD Patients

  • For low numbers: Identify the cause and determine if it's reversible.
  • Delay non-urgent surgery where possible.
  • Surgery is safe if platelet count is > 50 x 10⁹/l.

DOACs - Low Risk of Bleeding (SDCEP)

  • Refer to the SDCEP guidelines in examinations.
  • Low-risk procedures: Simple extractions (1-3 teeth), incision and drainage, detailed perio examination, RSI, and restorations with sub-gingival margins.

DOACs - Higher Risk of Bleeding

  • High-risk procedures: Complex/adjacent extractions, flap-raising procedures, gingival re-contouring, and biopsies.

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