Further characterization of platelet-type von Willebrand's disease in Japan
H Takahashi, M Handa, K Watanabe, Y Ando, R Nagayama, A Hattori, A Shibata, AB Federici, ZM Ruggeri and TS Zimmerman
We studied four patients who showed aggregation of platelets in
platelet-rich plasma at lower concentrations of ristocetin than those
required for normal platelet-rich plasma and who demonstrated an increased
capacity of the platelets to bind normal von Willebrand factor. The four
patients were from two Japanese families. Platelets from one family
aggregated spontaneously in vitro, and platelets from both families
aggregated upon the addition of normal plasma and cryoprecipitate, in the
absence of ristocetin or other agonists. Analysis of the multimeric
composition of von Willebrand factor by sodium dodecyl sulfate-agarose gel
electrophoresis revealed a decrease in large multimers or a decrease in
both large and intermediate multimers in plasma, but normal multimers in
platelets. 1-Deamino-[8-D- arginine]-vasopressin caused by an immediate
appearance of larger multimers in plasma, followed by the rapid
disappearance of these multimers from circulating plasma. Analysis of
platelet membrane glycoproteins from the patients showed that there were
two distinct bands in the glycoprotein I region; one migrated in a slower
region and the other in a faster region than normal glycoprotein Ib. We
suggest that the platelet receptor abnormality in these patients is related
to this abnormality of glycoprotein Ib.
Volume 64,
Issue 6,
pp. 1254-1262,
12/01/1984
Copyright © 1984 by The American Society of Hematology
