What is the recommended management for a patient in their 60s with a mild phenotype of type 2B von Willebrand disease (VWD) and normal von Willebrand factor (VWF) levels and ratios, who only experiences symptoms during stress like surgery?

Management of Mild Type 2B von Willebrand Disease in Elderly Patients During Surgery

For patients in their 60s with mild type 2B von Willebrand disease (VWD) who have normal von Willebrand factor (VWF) levels and ratios but experience symptoms only during stress like surgery, VWF concentrate should be administered to maintain VWF activity ≥50 IU/dL throughout the perioperative period.

Understanding the Clinical Presentation

• Patients with mild phenotype type 2B VWD may have normal VWF levels at baseline but can develop bleeding complications during periods of stress such as surgery 1
• Type 2B VWD is characterized by an abnormal VWF with increased affinity for platelet glycoprotein Ib-alpha (GPIb-alpha) that may result in moderate to severe thrombocytopenia 2
• Even patients with mild phenotypes who maintain normal laboratory values at baseline can experience significant bleeding during surgery or other stressors 3

Preoperative Assessment

• Target VWF activity level should be ≥50 IU/dL for most procedures, as recommended by the International Society on Thrombosis and Haemostasis 1
• Obtain baseline measurements of VWF:Ag, VWF:RCo, and the ratio of VWF:RCo/VWF:Ag to properly assess the patient's current status 1
• Monitor platelet count, as thrombocytopenia can occur in type 2B VWD and may worsen during stress conditions 2

Treatment Approach

First-line Treatment: VWF Concentrate

• VWF concentrate is the treatment of choice for type 2B VWD patients undergoing surgery, as it provides reliable hemostasis without the risk of worsening thrombocytopenia 4
• Plasma-derived VWF/FVIII concentrates or recombinant VWF should be administered to maintain VWF activity ≥50 IU/dL throughout the perioperative period 1
• For major surgery, maintain VWF activity levels >50 IU/dL for the duration of the procedure and postoperative period 1

Avoid Desmopressin in Type 2B VWD

• Desmopressin is contraindicated in type 2B VWD as it may worsen thrombocytopenia by releasing abnormal VWF that binds to platelets 4
• The FDA label for desmopressin specifically states it is not indicated "when there is evidence of an abnormal molecular form of factor VIII antigen," which includes type 2B VWD 5

Perioperative Management Protocol

1. Preoperative preparation:

   • Administer VWF concentrate 30-60 minutes before the procedure to achieve VWF activity ≥50 IU/dL 1
   • Monitor VWF:RCo, VWF:Ag, and FVIII levels pre-treatment and post-treatment 1

2. During surgery:

   • Maintain VWF activity >50 IU/dL throughout the procedure 1
   • Monitor platelet count, as thrombocytopenia may develop or worsen during surgery 2

3. Postoperative management:

   • Continue VWF concentrate administration to maintain VWF activity >50 IU/dL until wound healing is complete 1
   • For patients with epidural anesthesia, maintain VWF activity >50 IU/dL while the catheter remains in place 1

Special Considerations for Elderly Patients

• Elderly patients may have comorbidities that increase both bleeding and thrombotic risks 1
• Careful monitoring of both VWF levels and platelet counts is essential in this population 2
• Elevated FVIII levels that may occur with age or stress should not be the sole determinant for treatment decisions - VWF activity remains the critical parameter 1

Monitoring Response to Treatment

• Monitor VWF:RCo, VWF:Ag, and FVIII levels pre-treatment, post-treatment, and periodically during treatment 1
• Follow platelet counts closely, as thrombocytopenia may develop or worsen during stress conditions 2
• For persistent mucosal bleeding despite adequate FVIII levels, consider adjunctive treatments like tranexamic acid 1

Potential Complications and Management

• Thrombocytopenia: May worsen during stress and increase bleeding risk (adjusted hazard ratio = 4.57) 2
• Inadequate response to VWF concentrate: Consider increasing the dose or frequency of administration 4
• Thrombotic risk: Monitor for signs of thrombosis, particularly in elderly patients with elevated FVIII levels 1