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Platelet-type von Willebrand's disease: characterization of a new bleeding
disorder
JL Miller and A Castella
An autosomally transmitted bleeding diathesis sharing some, but not all,
features previously described in von Willebrand's disease (vWd) was studied
in five patients representing three generations of a single family.
Bleeding times in the upper normal range in conjunction with low-normal
platelet counts, normal factor VIII coagulant activity and VIII-related
antigen, decreased VIII-ristocetin cofactor activity, selective decrease of
the higher molecule weight factor VIII/von Willebrand factor (VIII/vWF)
multimers, and increased ristocetin- induced platelet agglutination at low
ristocetin concentrations were characteristic. Binding of patient VIII/vWF
to washed normal platelets was within normal limits, whereas binding of
normal VIII/vWF to patient platelets was significantly increased (p less
than 0.001 at 0.6 mg/ml ristocetin). This disorder accordingly appears to
involve an intrinsic platelet abnormality affecting platelet-VIII/vWF
interactions. It is proposed that the concept of vWD be broadened to
include patients with this abnormality, which may appropriately be called
"Platelet-type von Willebrand's disease."
Volume 60,
Issue 3,
pp. 790-794,
09/01/1982
Copyright © 1982 by The American Society of Hematology
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