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The heterogeneity of type IIA von Willebrand's disease: studies with
protease inhibitors
J Batlle, MF Lopez Fernandez, M Campos, B Justica, C Berges, JL Navarro, JM Diaz Cremades, CK Kasper, JA Dent and ZM Ruggeri
The absence of large von Willebrand factor (vWF) multimers from plasma is a
characteristic of Type IIA von Willebrand's disease (vWD) and is thought to
contribute to the clinical expression of this disorder. Recently, three IIA
patients have been reported in whom intermediate and large multimers could
be restored if blood were collected in 5 mm EDTA, 6 mmol/L
N-ethylmaleimide, and 1 mmol/L leupeptin. This suggested that absence of
large multimers resulted from in vitro proteolysis. We have now collected
blood from ten Type IIA vWD patients in these inhibitors but were not able
to detect large multimers in the plasma of any of them. In addition,
intermediate-sized multimers were reduced or completely absent in all. The
inclusion of inhibitors in the citrate anticoagulant, as compared to
citrate alone, was found to increase the relative proportion of
intermediate multimers in some patients but had no effect in others, and in
none did it restore large multimers to plasma. The results with platelet
vWF were more varied. Four patients showed an absence or decrease of large
multimers, whereas in seven patients large multimers were present. When
compared with citrate anticoagulant alone, the inclusion of inhibitors in
the anticoagulant had little or no effect on the platelet multimeric
pattern. 1-Deamino-8- D-Arginine Vasopressin (DDAVP) was administered to
six patients from five families. Two patients from one family showed
complete correction and a third patient showed almost complete correction
of her bleeding time. Two patients showed minimal correction and one showed
no detectable correction. An increase in multimer size after DDAVP tended
to be associated with correction of the bleeding time. However, in no case
did the largest multimers appear in plasma even in patients with complete
bleeding time correction. The presence or absence of inhibitors in the
anticoagulant had little or no effect on the multimeric pattern after
DDAVP. These results indicate that Type IIA vWD is a heterogeneous disorder
in which absence of largest and intermediate multimers is an in vivo
phenomenon.
Volume 68,
Issue 6,
pp. 1207-1212,
12/01/1986
Copyright © 1986 by The American Society of Hematology
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