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Type IIB von Willebrand's disease: differential clearance of endogenous
versus transfused large multimer von willebrand factor
ZM Ruggeri, R Lombardi, L Gatti, R Bader, C Valsecchi and TS Zimmerman
The abnormal multimeric composition of plasma von Willebrand factor in type
IIB von Willebrand's disease is transiently corrected after infusion of
1-deamino-[8-D-arginine]-vasopressin. However, the larger multimers
released into the circulation disappear more rapidly in these patients than
in type I von Willebrand's disease or normals. We demonstrate that the
larger multimers of normal von Willebrand factor transfused into a type IIB
patient are cleared from the circulation more slowly than multimers of
similar size endogenously released from tissue stores. The rate of
disappearance of large von Willebrand factor multimers after infusion of
cryoprecipitate is similar in IIB, IIA, and severe homozygous-like von
Willebrand's disease. Platelets from the IIB patient exhibited normal
ristocetin-induced binding of normal von Willebrand factor. However, like
normal platelets, they bound IIB von Willebrand factor at lower ristocetin
concentrations than required for normal von Willebrand factor. These
findings provide evidence that absence of the larger multimers from IIB
plasma is related to a molecular abnormality of von Willebrand factor
rather than to enhanced affinity of abnormal tissue or cellular binding
sites, as is the case in the recently described "pseudo" von Willebrand's
disease and "platelet-type" von Willebrand's disease.
Volume 60,
Issue 6,
pp. 1453-1456,
12/01/1982
Copyright © 1982 by The American Society of Hematology
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