Acquired haemophilia A due to the development of auto-antibodies directed a
gainst factor VIII (FVIII) is a rare disorder that may result in serious ha
emorrhagic episodes. Although in many cases no associated underlying disord
ers are apparent, the condition has been reported in association with autoi
mmune disorders, haematological malignancies, treatment with certain drugs
and pregnancy. The reaction kinetics of auto-antibodies to FVIII differ fro
m those observed with allo-antibodies in congenital haemophilia. Previous s
tudies in congenital haemophilia have raised the possibility that inhibitor
y antibodies vary in their reactivity with the factor VIII molecules in dif
ferent concentrates used for treatment. However, the interaction of FVIII i
n concentrates and inhibitors in acquired haemophilia has never been previo
usly studied. In this study, the effect of different FVIII concentrates was
studied on neutralization in vitro by performing inhibitor titres using th
e New Oxford inhibitor assay method.
The inhibitor titre in eight patients with acquired haemophilia A was assay
ed against five commercially available FVIII concentrates of varying purity
. The intermediate purity concentrate 8Y and the high purity concentrate th
at contains normal amounts of von Willebrand's Factor (vWF) (Alphanate) gav
e lower titres than the high purity concentrates with low (Monoclate-P) or
no (Kogenate) von Willebrand content. All but one antibody had very low rea
ctivity with porcine FVIII. Further work will be required to establish whet
her concentrates manifesting a low level of in vitro reactivity with the in
hibitor have a better haemostatic effect in vivo.