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Type IIB Tampa: a variant of von Willebrand disease with chronic
thrombocytopenia, circulating platelet aggregates, and spontaneous platelet
aggregation
HI Saba, SR Saba, J Dent, ZM Ruggeri and TS Zimmerman
Type IIB von Willebrand disease is characterized by enhanced ristocetin-
induced platelet aggregation and absence of large von Willebrand factor
multimers from plasma. An alteration of the von Willebrand factor molecule
resulting in increased reactivity with platelets appears to be the basis
for these abnormalities. We have now identified a new variant of type IIB
von Willebrand disease in a family in which the four affected members also
have chronic thrombocytopenia, in vivo platelet aggregate formation, and
spontaneous platelet aggregation in vitro. In spite of repeatedly prolonged
bleeding times and persistent thrombocytopenia, their bleeding diathesis is
only moderate.
Volume 66,
Issue 2,
pp. 282-286,
08/01/1985
Copyright © 1985 by The American Society of Hematology
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