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Acquired von Willebrand disease caused by an autoantibody selectively
inhibiting the binding of von Willebrand factor to collagen
PJ van Genderen, T Vink, JJ Michiels, MB van 't Veer, JJ Sixma and HH van Vliet
Department of Hematology, University Hospital Dijkzigt, Rotterdam, The
Netherlands.
An 82-year-old man with a low-grade malignant non-Hodgkin lymphoma and an
IgG3 lambda monoclonal gammopathy presented a recently acquired bleeding
tendency, characterized by recurrent epistaxis, easy bruising, and episodes
of melena, requiring packed red blood cell transfusions. Coagulation
studies showed a von Willebrand factor (vWF) defect (Ivy bleeding time,
> 15 minutes; vWF antigen [vWF:Ag], 0.08 U/mL; ristocetin cofactor
activity [vWF:RCoF], < 0.05 U/mL; collagen binding activity [vWF:CBA],
0.01 U/mL; absence of the high molecular weight multimers of vWF on
multimeric analysis). Mixing experiments suggested the presence of an
inhibitor directed against the vWF:CBA activity of vWF without
significantly inhibiting the FVIII:C, vWF:Ag, and vWF:RCoF activities. The
inhibitor was identified as an antibody of the IgM class by
immunoabsorption of vWF and inhibitor-vWF complexes from the plasma of the
patient. Subsequent immunoprecipitation experiments using recombinant
fragments of vWF showed that the inhibitor reacted with both the
glycoprotein Ib binding domain (amino acids [aa] 422-826) and the A3 (aa
909-1112) domain of vWF, but not with the A2 (aa 716-908) or D4 (aa
1183-1535) domains. We conclude that the IgM autoantibody inhibits the
vWF:CBA activity by reacting with an epitope present on both the
glycoprotein Ib and A3 domains of vWF.
Volume 84,
Issue 10,
pp. 3378-3384,
11/15/1994
Copyright © 1994 by The American Society of Hematology
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