Episode 070: Heme Consults Series: Von Willebrand Disease, Part 2
Aug 23, 2023
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In this episode of the podcast, the hosts continue discussing von Willebrand disease (vWD), focusing on type 2 subtypes. They cover topics such as testing for suspected vWD, management strategies for type 2 vWD, different subtypes of type 2 vWD, and the approach to perioperative management. They also touch on diagnosing and subtyping vWD, types of factor products used for treatment, and understanding the characteristics and management strategies of type 2 vWD subtypes.
Accurate diagnosis of Von Willebrand's disease Type 2 subtypes requires advanced testing, including multimer analysis, Ristocetin-induced platelet aggregation tests, and genetic testing.
Perioperative management for Von Willebrand's disease involves individualized dosing strategies, such as using DDAVP for Type 1 patients and exogenous factor replacement for Type 2B, 2M, 2N, and Type 3 patients to maintain hemostasis.
Deep dives
Overview of Von Willebrand's disease and focus on Type 2
In this episode, the hosts continue their discussion on Von Willebrand's disease, specifically focusing on Type 2. They highlight the difference between Type 2A, which involves a loss of high molecular weight multimers, and Type 2B, which is characterized by a gain of function mutation resulting in enhanced platelet aggregation. They also discuss Type 2M, which involves a loss of platelet binding, and Type 2N, characterized by a decreased factor 8 binding. The hosts emphasize the importance of accurate diagnosis through advanced testing, including multimer analysis and Ristocetin-induced platelet aggregation tests. They also mention the role of genetic testing in identifying specific subtypes. The discussion concludes with insights into perioperative management for patients with Von Willebrand's disease, including the use of DDAVP for Type 1 patients and exogenous factor replacement for Type 2B, 2M, 2N, and Type 3 patients.
Diagnostic considerations and testing options
The hosts discuss the diagnostic considerations in Type 2B Von Willebrand's disease, including the possibility of platelet type Von Willebrand's disease. They highlight the importance of genetic testing, as it provides a reliable way to differentiate between the subtypes. Specific tests, such as multimer analysis, Ristocetin-induced platelet aggregation, Von Willebrand's factor to factor 8 binding assay, and Von Willebrand's factor to collagen binding assay, are mentioned as valuable tools in diagnosing different subtypes. The hosts recommend close monitoring of Von Willebrand factor antigen, Ristocetin cofactor, and factor 8 activity levels to inform dosing strategies for replacement therapy.
Perioperative management for patients with Von Willebrand's disease
The hosts provide insights into perioperative management for patients with Von Willebrand's disease undergoing surgery. They discuss the use of DDAVP for Type 1 patients in minor surgeries or bleeding events. For Type 2B, 2M, 2N, and Type 3 patients, exogenous factor replacement is recommended to maintain factor levels above 50% for several days after minor surgeries and up to 7-14 days after major surgeries. The hosts emphasize the importance of monitoring factor levels and factor 8 activity to prevent dose stacking and maintain hemostasis. Different factor products, including human-derived products like Humate P and lab-derived recombinant products like Von Vendy, are discussed as treatment options.
Case study of perioperative management in a patient with Von Willebrand's disease
The hosts present a case study of a patient with Von Willebrand's disease undergoing extensive cosmetic surgery. They outline the perioperative management plan, including the administration of Humate P prior to surgery and monitoring Von Willebrand factor antigen, Ristocetin cofactor, and factor 8 activity levels. The hosts highlight the importance of individualized monitoring and dosing based on the patient's response and recommend caution to avoid dose stacking that could lead to thrombosis. The case study concludes with the patient achieving successful cosmetic outcomes and optimal recovery.
This week in our hematological consultation series, we continue our discussion on von Willebrand disease (vWD), this time focusing on the type 2 subtypes and we also discuss management.
In case you missed it, we recommend checking out episode 069 for Part I of this vWD series, covering taking a bleeding history and about Type 1 and Type 3 disease.
Content:
- What testing do we send for suspected vWD (refresher)
- What to do if type 2 vWD is suspected?
- What are the different subtypes of Type 2 vWD?
- What is "platelet-type vWD"?
- What is the approach to perioperative management?
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