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/EricsMedi
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