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

Research output: Contribution to journalArticle

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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
Publication statusPublished - 1984
Externally publishedYes

Fingerprint

Platelets
Japan
Blood Platelets
Ristocetin
Glycoproteins
Plasmas
Platelet-Rich Plasma
von Willebrand Factor
Platelet Membrane Glycoproteins
Deamino Arginine Vasopressin
Agar Gel Electrophoresis
Platelet Aggregation
Sodium Dodecyl Sulfate
Platelet type Von Willebrand disease
Electrophoresis
Sepharose
Agglomeration
Gels
Chemical analysis

ASJC Scopus subject areas

  • Hematology

Cite this

Takahashi, H., Handa, M., Watanabe, K., Ando, Y., Nagayama, R., Hattori, A., ... Zimmerman, T. S. (1984). Further characterization of platelet-type von Willebrand's disease in Japan. Blood, 64(6), 1254-1262.

Further characterization of platelet-type von Willebrand's disease in Japan. / Takahashi, H.; Handa, M.; Watanabe, K.; Ando, Y.; Nagayama, R.; Hattori, A.; Shibata, A.; Federici, A. B.; Ruggeri, Z. M.; Zimmerman, T. S.

In: Blood, Vol. 64, No. 6, 1984, p. 1254-1262.

Research output: Contribution to journalArticle

Takahashi, H, Handa, M, Watanabe, K, Ando, Y, Nagayama, R, Hattori, A, Shibata, A, Federici, AB, Ruggeri, ZM & Zimmerman, TS 1984, 'Further characterization of platelet-type von Willebrand's disease in Japan', Blood, vol. 64, no. 6, pp. 1254-1262.
Takahashi H, Handa M, Watanabe K, Ando Y, Nagayama R, Hattori A et al. Further characterization of platelet-type von Willebrand's disease in Japan. Blood. 1984;64(6):1254-1262.
Takahashi, H. ; Handa, M. ; Watanabe, K. ; Ando, Y. ; Nagayama, R. ; Hattori, A. ; Shibata, A. ; Federici, A. B. ; Ruggeri, Z. M. ; Zimmerman, T. S. / Further characterization of platelet-type von Willebrand's disease in Japan. In: Blood. 1984 ; Vol. 64, No. 6. pp. 1254-1262.
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