What is the risk of uncontrolled bleeding in a patient with undiagnosed type 2B von Willebrand disease (VWD) undergoing a dilation and curettage (D&C) procedure without treatment?

Risk of Uncontrolled Bleeding in Undiagnosed Type 2B von Willebrand Disease During D&C

A patient with undiagnosed type 2B von Willebrand disease undergoing a dilation and curettage procedure without treatment faces a significant risk of severe uncontrolled bleeding that could lead to substantial morbidity or mortality.

Understanding Type 2B VWD and Bleeding Risk

Type 2B von Willebrand disease (VWD) is characterized by an abnormal von Willebrand factor (VWF) with increased affinity for platelet glycoprotein Ib-alpha receptors, resulting in:

• Increased platelet clearance
• Preferential loss of high molecular weight VWF multimers
• Moderate to severe thrombocytopenia in approximately 30% of patients at baseline and up to 57% during stress conditions 1

Specific Bleeding Risks During D&C

D&C is considered a minor surgical procedure but involves significant mucosal tissue manipulation, which is particularly problematic in VWD:

• Patients with thrombocytopenia have a 4.57 times higher risk of bleeding compared to those without thrombocytopenia 1
• Surgical interventions without proper hemostatic preparation can lead to critical bleeding events 2
• The risk is amplified because:
  • D&C involves highly vascular endometrial tissue
  • VWD primarily affects mucosal bleeding
  • Absence of diagnosis means no prophylactic measures will be taken

Clinical Consequences of Undiagnosed Bleeding

Without diagnosis and treatment, the patient may experience:

1. Immediate complications:

   • Excessive intraoperative bleeding requiring blood transfusion
   • Prolonged procedure time
   • Incomplete procedure due to poor visualization
   • Need for emergency interventions

2. Post-procedure complications:

   • Continued heavy vaginal bleeding
   • Delayed hemorrhage (which can occur up to 7-14 days post-procedure) 3
   • Anemia requiring transfusion
   • Hemodynamic instability
   • Potential need for additional surgical interventions

Management That Would Have Been Indicated

If diagnosed, appropriate management would include:

• Pre-procedure:

  • Factor VIII/VWF concentrate administration with target VWF activity ≥50 IU/dL 3
  • Avoidance of desmopressin as it may cause further decrease in platelet count in Type 2B VWD 4

• During procedure:

  • Monitoring of VWF levels and platelet count
  • Additional factor concentrate as needed

• Post-procedure:

  • Continued factor replacement for at least 7-14 days due to risk of delayed hemorrhage 3
  • Close monitoring for bleeding for at least 2 weeks 3

Factors That May Worsen Outcomes

Several factors can exacerbate bleeding risk in undiagnosed Type 2B VWD:

• Stress from the procedure itself can worsen thrombocytopenia 1
• Use of NSAIDs for post-procedure pain management would further impair platelet function 5
• Lack of awareness by healthcare providers about abnormal bleeding may delay intervention
• The patient may have variable clinical presentation even with the same genetic mutation, complicating recognition of the problem 4

Conclusion

The absence of diagnosis and appropriate prophylactic treatment places a patient with Type 2B VWD at substantial risk during a D&C procedure. The primary concern is severe mucosal bleeding that could be difficult to control, potentially requiring multiple interventions, blood product support, and possibly resulting in significant morbidity or even mortality in severe cases.