FAMILIAL INCIDENCE OF PRECIPITATING ANTIBODIES IN VONWILLEBRANDS DISEASE - STUDY OF 4 CASES

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  • 1 January 1979
    • journal article
    • research article
    • Vol. 94  (1) , 60-75
Abstract
Precipitating antibodies directed toward human F.VIII/WF [factor VIII/von Willebrand factor] were found in the plasma of 4 of 17 multitransfused patients with severe, homozygous-like VWD [von Willebrand''s disease]. The familial incidence was illustrated by the development of these antibodies in 3 patients from the same kindred. Such antibodies, titrated with newly developed quantitative assays of anti-VIIIR:Ag [anti-factor VIII related antigen] and anti-VIIIR:RCo [anti-factor VIII-related ristocetin cofactor] were directed toward only these components of F.VIII/WF. VIII:C [factor VIII procoagulant activity] was neutralized in a time-independent manner in plasma and was not inactivated when separated from F.VIII/WF by solid-phase PE [polyelectrolyte] absorption. Plasma, serum [anti-factor VIII-related ristocetin cofactor] or immunoglobulin [Ig] reacted in precipitation systems (immunodiffusion, EID [electroimmunodiffusion], CID [crossed immunoelectrophoresis]) with human VIIIR:Ag, with some degree of cross-reactivity toward VIIIR:Ag from other mammalian plasmas. When used in IRMA [immunoradiometric assay] these antibodies demonstrated the same abnormalities as heterologous antisera in variant VWD: decreased binding affinity or nonparallelism of the dose-response curves. They are polyclonal IgG with both .kappa. and L chains. In some patients with severe homozygous-like VWD, the synthesis of the component of F.VIII/WF carrying VIIIR:Ag and VIIIR:RCo is apparently suppressed whereas VIII:C production is not completely abolished.