Abstract:
Haemophilia A severity is closely correlated to the factor VIII (FVIII) activity, which can be measured in
different ways. The original one-stage clotting assay is still the most widely used. The two-stage
coagulation assay eliminated many of the drawbacks of the one-stage assay and was further developed
into the chromogenic assay, a two-staged test with purified coagulation factors in the first stage, and a
FXa-specific chromogenic substrate in the second stage. In many patients with mild or moderate
haemophilia A, there is a discrepancy between the one-stage and the two-stage assays. If only the onestage
assay is used, some patients will have normal FVIII levels and not be diagnosed as having
haemophilia or be considered to have a milder bleeding risk than is the case. Other patients who have
normal FVIII activity will be diagnosed as haemophilia A. All haemophilia treatment centre laboratories
should have access to both one-stage and chromogenic FVIII:C assays. Appropriate standards should be
employed to enable accurate FVIII:C measurement. Different assays to measure inhibitor activity to
infused FVIII have been developed since 1959. Inhibitor results based on the one-stage or chromogenic
FVIII:C assays are well correlated, but the one-stage assay may be influenced by nonspecific inhibition.
