Hemophilia - Diagnosis and Management

Diagnosis:

 • 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/



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