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Thromb Haemost 1984; 52(03): 267-270
DOI: 10.1055/s-0038-1661193
Original Article
Schattauer GmbH Stuttgart

Agglutination of Formalin-Fixed, Platelet-Type von Willebrand’s Disease Platelets by Human von Willebrand Factor

Hoyu Takahashi
The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
Akira Shibata
The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
› Author Affiliations
Further Information

Publication History

Received 29 June 1984

Accepted 22 August 1984

Publication Date:
19 July 2018 (online)

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Summary

The interaction of platelets and von Willebrand factor (vWF) in platelet-type von Willebrand’s disease (vWD) was characterized using formalin-fixed platelets from the patients. Formalin-fixed patient platelets were agglutinated directly by human vWF in normal plasma and type IIB vWD plasma, but not in type IIA vWD plasma. In the presence of a small amount of normal vWF, ristocetin-induced agglutination of patient platelets was enhanced with low concentrations of ristocetin. Wheat germ agglutinin and EDTA inhibited vWF-induced agglutination, although EDTA had no effect on ristocetin (plus vWF)-induced agglutination. These results demonstrate that vWF-induced agglutination of platelet-type vWD platelets is independent of active platelet metabolism but requires divalent cations, and suggest that platelet membrane glycoprotein I (GPI) would be involved in this agglutination.

Key words

von Willebrand’s disease - Platelet-type von Willebrand’s disease - von Willebrand factor - Platelet agglutination - Ristocetin
 

  • References

  • 1 Hoyer LW, Shainoff JR. Factor-VIII-related protein circulates in normal human plasma as high molecular weight multimers. Blood 1980; 55: 1056-1059
    PubMedGoogle Scholar
  • 2 Ruggeri ZM, Zimmerman TS. Variant von Willebrand’s disease. Characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets J Clin Invest 1980; 65: 1318-1325
    CrossrefPubMedGoogle Scholar
  • 3 Meyer D, Obert B, Pietu G, Lavergne JM, Zimmerman TS. Multimeric structure of factor VIII/von Willebrand factor in von Willebrand’s disease. J Lab Clin Med 1980; 95: 590-602
    PubMedGoogle Scholar
  • 4 Zimmerman TS, Ruggeri ZM. von Willebrand’s disease. Prog Hemostas Thromb 1982; 6: 203-236
    PubMedGoogle Scholar
  • 5 Zimmerman TS, Ruggeri ZM, Fulcher CA. Factor VIII/von Willebrand factor. Prog Hematol 1983; 13: 279-309
    PubMedGoogle Scholar
  • 6 Ruggeri ZM, Pareti FI, Mannucci PM, Ciavarella N, Zimmerman TS. Heightened interaction between platelets and factor VIII/von Willebrand factor in a new subtype of von Willebrand’s disease. N Engl J Med 1980; 302: 1047-1051
    CrossrefPubMedGoogle Scholar
  • 7 Takahashi H, Sakuragawa N, Shibata A. von Willebrand disease with an increased ristocetin-induced platelet aggregation and a qualitative abnormality of the factor VIII protein. Am J Hematol 1980; 8: 299-308
    CrossrefPubMedGoogle Scholar
  • 8 Takahashi H. Studies on the pathophysiology and treatment of von Willebrand’s disease. IV. Mechanism of increased ristocetin-induced platelet aggregation in von Willebrand’s disease. Thromb Res 1980; 19: 857-867
    CrossrefPubMedGoogle Scholar
  • 9 Takahashi H, Nagayama R, Hattori A, Ihzumi T, Tsukada T, Shibata A. von Willebrand disease associated with familial thrombocytopenia and increased ristocetin-induced platelet aggregation. Am J Hematol 1981; 10: 89-99
    CrossrefPubMedGoogle Scholar
  • 10 Weiss HJ, Meyer D, Rabinowitz R, Pietu G, Girma J-P, Vicic WJ, Rogers J. Pseudo-von Willebrand’s disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII/von Willebrand factor and enhanced adsorption of its high-molecular-weight multimers N Engl J Med 1982; 306: 326-333
    CrossrefPubMedGoogle Scholar
  • 11 Miller JL, Castella A. Platelet-type von Willebrand’s disease: Characterization of a new bleeding disorder. Blood 1982; 60: 790-794
    PubMedGoogle Scholar
  • 12 Read MS, Shermer RW, Brinkhous KM. Venom coagglutinin. An activator of platelet aggregation dependent on von Willebrand factor Proc Natl Acad Sci USA 1978; 75: 4514-4518
    CrossrefPubMedGoogle Scholar
  • 13 Takahashi H, Nagayama R, Hattori A, Shibata A. Botrocetin- and Polybrene- induced platelet aggregation in platelet-type von Willebrand disease. Am J Hematol. (in press)
    PubMedGoogle Scholar
  • 14 Miller JL, Kupinski JM, Castella A, Ruggeri ZM. von Willebrand factor binds to platelets and induces aggregation in platelet-type but not type II B von Willebrand disease. J Clin Invest 1983; 72: 1532-1542
    CrossrefPubMedGoogle Scholar
  • 15 Takahashi H, Handa M, Watanabe K, Ando Y, Nagayama R, Hattori A, Shibata A, Federici AB, Ruggeri ZM, Zimmerman TS. Further characterization of platelet-type von Willebrand’s disease in Japan. Blood (in press).
    PubMedGoogle Scholar
  • 16 Born GV R. Aggregation of blood platelets by adenosine diphosphate and its reversal. Nature 1962; 194: 927-929
    PubMedGoogle Scholar
  • 17 Hardisty RM, Macpherson JC. A one-stage factor VIII (antihaemophilic globulin) assay and its use on venous and capillary plasma. Thrombos Diathes Haemorrh 1962; 7: 215-219
    PubMedGoogle Scholar
  • 18 Laurell CB. Quantitative estimation of protein by electrophoresis in agarose gel containing antibodies. Anal Biochem 1966; 15: 45-52
    CrossrefPubMedGoogle Scholar
  • 19 Macfarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, McPherson J. A method for assaying von Willebrand factor (ristocetin cofactor). Thrombos Diathes Haemorrh 1975; 34: 306-308
    PubMedGoogle Scholar
  • 20 Moake JL, Olson JD, Troll JH, Tang SS, Funicella T, Peterson DM. Binding of radioiodinated human von Willebrand factor to Bernard-Soulier, thrombasthenic and von Willebrand’s disease platelets. Thromb Res 1980; 19: 21-27
    CrossrefPubMedGoogle Scholar
  • 21 Jenkins CS, Phillips DR, Clemetson KJ, Meyer D, Larrieu M-J, Lüscher EF. Platelet membrane glycoproteins implicated in ristocetin-induced aggregation. Studies of the proteins on platelets from patients with Bernard-Soulier syndrome and von Willebrand’s disease J Clin Invest 1976; 57: 112-124
    CrossrefPubMedGoogle Scholar
  • 22 Nurden AT, Dupuis D, Kunicki TJ, Caen JP. Analysis of the glycoprotein and protein composition of Bernard-Soulier platelets by single and two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis. J Clin Invest 1981; 67: 1431-1440
    CrossrefPubMedGoogle Scholar
  • 23 Coller BS, Peerschke EI, Scudder LE, Sullivan CA. Studies with a murine monoclonal antibody that abolishes ristocetin-induced binding of von Willebrand factor to platelets: Additional evidence in support of GPIb as a platelet receptor for von Willebrand factor. Blood 1983; 61: 99-110
    PubMedGoogle Scholar
  • 24 Nagata Y, Burger MM. Wheat germ agglutinin. Molecular characteristics and specificity for sugar binding J Biol Chem 1974; 249: 3116-3122
    PubMedGoogle Scholar
  • 25 Bhavanandan VP, Katlic AW. The interaction of wheat germ agglutinin with sialoglycoproteins. The role of sialic acid J Biol Chem 1979; 254: 4000-4008
    PubMedGoogle Scholar
  • 26 Wright CS. Crystallographic elucidation of the saccharide binding mode in wheat germ agglutinin and its biological significance. J Mol Biol 1980; 141: 267-291
    CrossrefPubMedGoogle Scholar
  • 27 Nachman RL, Tarasov E, Weksler BB, Ferris B. Wheat germ agglutinin affinity chromatography of human platelet membrane glycoproteins. Thromb Res 1977; 12: 91-104
    PubMedGoogle Scholar
  • 28 Ganguly P, Gould NL, Sidhu P. Interaction of lectins with human platelets. Effects on platelet stimulation by thrombin and ristocetin Biochim Biophys Acta 1979; 586: 574-583
    CrossrefPubMedGoogle Scholar
  • 29 Miller JL, Boselli BD, Kupinski JM. In vivo interaction of von Willebrand factor with platelets following cryoprecipitate transfusion in platelet-type von Willebrand’s disease. Blood 1984; 63: 226-230
    PubMedGoogle Scholar