Haemophilia with Inhibitors — RACP Paediatrics MCQ

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HardHaematology & OncologyHaemophilia with InhibitorsRACP Paediatrics

A 15-year-old boy has severe haemophilia A (FVIII <1%) and develops an inhibitor (antibody against FVIII). He has a joint bleed. Standard FVIII replacement is ineffective. What is the treatment for acute bleeding in a patient with high-titre inhibitors?

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Correct answer: D — Bypassing agents: recombinant activated factor VII (rFVIIa) or activated prothrombin complex concentrate (FEIBA)

High-titre FVIII inhibitors (>5 BU) render standard FVIII replacement ineffective. Bypassing agents (rFVIIa or FEIBA) are used for acute bleeds. Emicizumab (bispecific antibody mimicking FVIII) provides effective prophylaxis in patients with inhibitors and has transformed management.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Haemophilia with Inhibitors