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A new variant of dominant type II von Willebrand's disease with aberrant
multimeric pattern of factor VIII-related antigen (type IID)
S Kinoshita, J Harrison, J Lazerson and CF Abildgaard
A new type II variant form of von Willebrand's disease has been recognized
in a mother and daughter who have bleeding manifestations typical of von
Willebrand's disease. Laboratory findings include consistently prolonged
bleeding times, with normal levels of factor VIII procoagulant and antigen,
but decreased ristocetin cofactor activity. Electrophoresis in SDS 1.5%
agarose gel and reaction with 125I-labeled anti-factor VIII-related antigen
rabbit IgG, followed by autoradiography, revealed that both plasma and
platelets lack the large multimers of factor VIII-related antigen. In 2.5%
gel, the propositus plasma lacked the normal "triplet" pattern. In 3.0%
gel, a 5-band pattern was observed in normal, type IIA, and type IIB
plasma, whereas type IIC plasma revealed a 2-band pattern. The patient's
plasma revealed a 4-band pattern distinctly different from normal or other
type II variants. We suggest that this new variant be labeled type IID,
until a more appropriate nomenclature is developed.
Volume 63,
Issue 6,
pp. 1369-1371,
06/01/1984
Copyright © 1984 by The American Society of Hematology
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