Selective absence of large forms of factor VIII/von Willebrand factor in
acquired von Willebrand's syndrome. Response to transfusion
D Meyer, D Frommel, MJ Larrieu and TS Zimmerman
A previously healthy elderly man with mucocutaneous bleeding was found to
have a benign monoclonal IgG gammapathy associated with criteria for severe
von Willebrand disease (Factor VIII procoagulant activity,
Factor-VIII-related antigen, and ristocetin cofactor activity, less than
10% of normal). Associated qualitative abnormalities of factor VIII/von
Willebrand factor were demonstrated by radiocrossed immunoelectrophoresis
and immunoradiometric assay. The late clinical onset and negative family
history are in favor of an acquired form of vWD. The monoclonal gammapathy
and abnormalities of factor VIII/von Willebrand factor have been stable
over a 10-yr period. No inhibitor to Factor VIII procoagulant activity,
ristocetin cofactor activity, or Factor-VIII-related antigen could be
demonstrated. Following transfusion of cryoprecipitate (with a normal cross
immunoelectrophoretic pattern), there was a rapid removal of the large
forms of Factor.-VIII-related antigen, paralleled by a decay of ristocetin
cofactor activity. The transfusion study of this patient with acquired von
Willebrand disease type II (variant of von Willebrand disease) serves to
emphasize the relationship between polydispersity of Factor VIII/von
Willebrand Factor and functional heterogeneity.
Volume 54,
Issue 3,
pp. 600-606,
09/01/1979
Copyright © 1979 by The American Society of Hematology
