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Summary  Sensitivity to  FVIII  inhibitors of the native plasma‐derived (pd)  FVIII / VWF  complex vs. the complexes formed after exogenous  FVIII  infusion in the haemophilic patient has not been thoroughly studied. The role of  VWF  in the interaction of  FVIII  with inhibitors was studied  in vitro  using different combinations of  VWF  and  FVIII  concentrates. Normal plasma, pd FVIII / VWF  and isolated  FVIII  (recombinant  FVIII , B‐domain deleted and pd FVIII ) were used. Titre ( BU ) was kinetically determined (up to 2 h) in serial dilutions of inhibitor IgG (purified from a pool of plasmas with inhibitors) mixed with  VWF  and then incubated with the different  FVIII . Inhibitor was also added to previously mixed  VWF + FVIII . Residual  FVIII :C was determined.  TGA  assays were performed with  FVIII ‐deficient plasma spiked with the  FVIII ‐ VWF  mixtures with/without an  ESH ‐8 antibody. Inhibitor titres for plasma and pd FVIII / VWF  were comparable at all time points. Titres for all concentrates of isolated  FVIII  were significantly higher than those for plasma or pd FVIII / VWF  (1.4–1.9 fold) even after preincubation with  VWF . At t = 0 h, titres for plasma or pd FVIII / VWF  were unquantifiable, but were detectable for isolated  FVIII  (0.6–1.6  BU ). In contrast to pd FVIII / VWF , the decrease in thrombin generation parameters by isolated  FVIII  in the presence of  ESH ‐8 was significant ( P  < 0.01) even when previously combined with  VWF . In conclusion,  VWF  protection against  FVIII  inhibitor activity might be higher with native pd FVIII / VWF  complex than with the corresponding compound formed from the isolated proteins. Bethesda assay titration using different  FVIII  concentrates would be advisable to guide the treatment of inhibitor patients.

haemophilia

Native plasma‐derived  FVIII / VWF  complex has lower sensitivity to  FVIII  inhibitors than the combination of isolated  FVIII  and  VWF  proteins. Impact on Bethesda assay titration of  FVIII  inhibitors