Abstract
WE report the presence of precipitating antibodies in three patients with von Willebrand's disease (VWD), an inherited deficiency of factor VIII (anti-haemophilic factor, AHF). To our knowledge this is the first description of patients with complete biological and immunological deficiency of a plasma-clotting protein responding to replacement therapy by the production of antibodies with properties indistinguishable from those elicited in animals. AHF is a high molecular weight (> 106) glycoprotein complex1 associated with two biological activities; one necessary for normal clotting (VIII: C) and the other for normal platelet function (Willebrand factor, VIIIR: WF). Specific antigenic determinants (VIIIR: AG) can be reliably quantified using heterologous antisera2,3. Any concept of the structure or the molecular genetics of AHF needs to incorporate the clinical and immunological findings in the two congenital disorders associated with AHF deficiency: haemophilia A and VWD. The former is a sex-linked deficiency of VIII: C alone whereas the latter represents a deficiency of all three parameters of the AHF complex and is inherited as an autosomal trait. Non-precipitating antibodies to AHF, acquired after replacement therapy, are common in haemophilia A (ref. 4) but only one case has been reported in VWD5,6.
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MANNUCCI, P., MEYER, D., RUGGERI, Z. et al. Precipitating antibodies in von Willebrand's disease. Nature 262, 141–142 (1976). https://doi.org/10.1038/262141a0
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DOI: https://doi.org/10.1038/262141a0
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