Hemophilia - Diagnosis and Management

Hemophilia - Diagnosis and Management


 • Consistent bleeding history (unless screening for disease in family members with established disease)

 • aPTT is prolonged, but corrects with a mixing study (aPTT may be normal in mild cases)

 • PT/INR & von Willebrand factor antigen (vWF:Ag) are normal

Individual factor activity levels (VIII, IX, +/- XI) are measured to:

 • Confirm the diagnosis

 • Establish hemophilia type (e.g. A vs. B)

 • Classify severity

Treatment - Acute Bleeding:

 • For serious or moderate bleeding, treat with relevant factor immediately.

 • General targets:

      Serious: 100% factor activity

      Moderate: 50% factor activity

      Mild: Local therapy only is usually sufficient

 • Antifibrinolytic therapy can be a useful adjunct.

Chronic Management:

 • If possible, patients should have multidisciplinary care coordinated by a hemophilia treatment center. Avoid meds that ↑ bleeding risk.

 • Encourage exercise, though safety assessment of athletic regimens should be individualized.

 • Prophylaxis with factors should be considered in those with severe disease, though the cost can be prohibitive.

- Dr. Eric Strong @DrEricStrong - Strong Medicine https://www.youtube.com/c/EricsMedicalLectures/

#Hemophilia #Diagnosis #Management #treatment #hematology
Contributed by

Dr. Gerald Diaz
Board Certified Internal Medicine Hospitalist, GrepMed Editor in Chief 🇵🇭 🇺🇸 - Sign up for an account to like, bookmark and upload images to contribute to our community platform. Follow us on IG:  https://www.instagram.com/grepmed/ | Twitter: https://twitter.com/grepmeded/
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