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, A. B. Federici, Z. M. Ruggeri, T. S. Zimmerman

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Abstract

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 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.

Original languageEnglish
Pages (from-to)1254-1262
Number of pages9
JournalBlood
Volume64
Issue number6
DOIs
Publication statusPublished - 1984

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Takahashi, H., Handa, M., Watanabe, K., Ando, Y., Nagayama, R., Hattori, A., Shibata, A., Federici, A. B., Ruggeri, Z. M., & Zimmerman, T. S. (1984). Further characterization of platelet-type von Willebrand's disease in Japan. Blood, 64(6), 1254-1262. https://doi.org/10.1182/blood.v64.6.1254.bloodjournal6461254