What treatment would you suggest for a patient with Hemophilia C (factor XI deficiency) experiencing prolonged bleeding after childbirth?

Treatment for Hemophilia C (Factor XI Deficiency) with Postpartum Bleeding

Fresh Frozen Plasma (FFP) is the recommended first-line treatment for a patient with Hemophilia C experiencing prolonged bleeding after childbirth. 1

Understanding Hemophilia C

Hemophilia C, also known as Factor XI deficiency or Rosenthal disease, is a rare bleeding disorder that:

• Is particularly common in individuals of Ashkenazi Jewish descent
• Has variable bleeding tendencies that don't necessarily correlate with Factor XI activity levels
• Often manifests as excessive bleeding following surgery, dental procedures, or childbirth

Treatment Algorithm for Postpartum Bleeding in Hemophilia C

First-Line Treatment:

• Fresh Frozen Plasma (FFP) is the treatment of choice for Factor XI deficiency 1
  • Contains adequate amounts of Factor XI to achieve hemostasis
  • Dosing: 15-20 mL/kg initially, followed by maintenance doses as needed
  • Target: Maintain Factor XI levels above 30-40% for adequate hemostasis

Second-Line Options (if FFP is unavailable or ineffective):

1. Tranexamic Acid (TXA)

   • Antifibrinolytic agent that helps stabilize clots
   • Often used as adjunctive therapy with FFP 2
   • Particularly useful for mucosal bleeding

2. DDAVP (Desmopressin)

   • May provide some benefit in mild cases
   • Less effective than in other bleeding disorders
   • Should not be relied upon as sole therapy for significant bleeding 2

Not Recommended as First-Line for Hemophilia C:

• vWF:FVIII concentrate - Not specifically indicated for Factor XI deficiency
• Cryoprecipitate - Contains minimal Factor XI and is not effective for Hemophilia C

Monitoring and Follow-up

• Monitor clinical response to treatment
• Follow hemostatic parameters including aPTT
• Continue treatment until bleeding is controlled and wound healing is established
• For severe cases, maintain Factor XI levels >30% for 3-5 days after bleeding stops

Special Considerations for Hemophilia C in Postpartum Setting

• Postpartum bleeding risk may persist for up to 2 weeks after delivery
• Consider prophylactic treatment before invasive procedures during this period
• Coordinate care between hematology and obstetrics teams

Common Pitfalls to Avoid

• Misdiagnosis: Hemophilia C can be confused with other coagulation disorders due to prolonged aPTT
• Inadequate dosing: FFP requires larger volumes than other blood products to achieve hemostasis
• Delayed treatment: Early intervention is critical to prevent significant blood loss
• Relying on factor levels alone: Clinical bleeding phenotype may not correlate with Factor XI levels

Conclusion for Clinical Practice

For this 25-year-old woman of Ashkenazi Jewish descent with Hemophilia C and postpartum bleeding, FFP should be administered promptly to replace Factor XI and achieve hemostasis. TXA can be used as an adjunctive therapy, but DDAVP and cryoprecipitate are not recommended as primary treatments for Factor XI deficiency.