Laboratory diagnosis

• 4 APTT with failure to correct with normal plasma.

• Inhibitor assay—patient's plasma reducing the factor VIII in normal plasma over 2h incubation period. Antibody titres reported in BU (Bethesda units). Check titre against porcine VIII as porcine VIII is often an effective treatment.

Differential diagnosis of spontaneous inhibitors—need to exclude nonspecific inhibitors e.g. myeloma paraproteins which bind non-specifically to coagulation plasma proteins. Ensure sample not contaminated with heparin.

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