Confirm type and severity of hemophilia (A or B) via factor assay. Baseline factor levels determine severity and guide treatment approach.
Patients should be managed in comprehensive hemophilia treatment centers with access to hematologists, nurses, physiotherapists, and psychosocial support.
Monitor factor levels, inhibitor development, joint health, and treatment adherence.
Administer recombinant or plasma-derived clotting factor VIII (Hemophilia A) or factor IX (Hemophilia B) during active bleeding episodes. Dose and duration depend on bleed location and severity.
Used in mild Hemophilia A. Stimulates release of endogenous factor VIII. Not effective in Hemophilia B. Avoid in children under 2 years and patients with cardiovascular disease.
Tranexamic acid and aminocaproic acid help prevent clot breakdown. Useful in mucosal bleeds (e.g., oral, nasal, menorrhagia). Often used alongside factor therapy.
Standard of care for children and adults with severe Hemophilia. Prevents spontaneous bleeding and protects joint health. Typically 2–3 infusions/week.
Allow less frequent dosing. Includes modified recombinant factors (e.g., Fc fusion, PEGylation). Improves adherence and quality of life.
A bispecific monoclonal antibody mimicking factor VIII function. Given subcutaneously once weekly or less. Can be used in patients with or without inhibitors.
Standard approach to eradicate inhibitors. Involves frequent high-dose factor administration over months. Success rates vary by patient.
Includes activated prothrombin complex concentrate (aPCC) and recombinant activated factor VII (rFVIIa). Used during bleeds in patients with high-titer inhibitors.
Factor levels must be corrected preoperatively. Maintain adequate levels intra- and post-op. Use antifibrinolytics and local hemostatic agents as needed.
Immediately treat with factor replacement before imaging. Suspect intracranial bleed in any head trauma, even if symptoms are mild.
One-time intravenous infusion delivering a functioning F8 or F9 gene using viral vectors. Approved for Hemophilia B in some countries; under investigation for Hemophilia A.
Research ongoing for therapies targeting natural anticoagulants (e.g., anti-TFPI, antithrombin) to restore hemostatic balance.
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