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Thromb Haemost 1990; 64(02): 227-231
DOI: 10.1055/s-0038-1647290
Original Article
Schattauer GmbH Stuttgart

Platelet Adhesion to Collagen in Subtypes of Type I von Willebrand’s Disease Is Dependent on Platelet von Willebrand Factor

Patrizia d'Alessio
1   The Department of Haematology, University Hospital Utrecht, The Netherlands
,
Jaap Jan Zwaginga
1   The Department of Haematology, University Hospital Utrecht, The Netherlands
,
Hetty C de Boer
1   The Department of Haematology, University Hospital Utrecht, The Netherlands
,
Augusto B Federici
2   The A. Bianchi Bonomi Haemophilia and Thrombosis Center and Institute of Internal Medicine, University of Milano, Italy
,
Francesco Rodeghierio
3   The Hemophilia and Thrombosis Center and Hematology Division Hospital of Vicenza, Italy,
,
Giancarlo Castaman
3   The Hemophilia and Thrombosis Center and Hematology Division Hospital of Vicenza, Italy,
,
Guglielmo Mariani
4   The Institute of Hematology, University of Rome, Italy
,
P Mannuccio Msnnucci
2   The A. Bianchi Bonomi Haemophilia and Thrombosis Center and Institute of Internal Medicine, University of Milano, Italy
,
Phillip G de Groot
1   The Department of Haematology, University Hospital Utrecht, The Netherlands
,
Jan J Sixma
1   The Department of Haematology, University Hospital Utrecht, The Netherlands
› Author Affiliations
Further Information

Publication History

Received 09 February 1990

Accepted after revision 28 May 1990

Publication Date:
26 July 2018 (online)

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Summary

Von Willebrand’s disease type I, characterized by low levels of factor VIII coagulant activity (VTII :C), von Willebrand factor antigen (vWF:Ag) and ristocetin cofactor activity (RiCof) (1), can be subdivided on the basis of platelet von Willebrand factor into subtype platelet normal, platelet discordant, and platelet low (2). We have investigated the contribution of platelet von Willebrand factor in these various subtypes to platelet adhesion using the rectangular perfusion chamber of Sakariassen et al. (3) with fibrillar collagen or a fibroblast matrix as adhesive surfaces. Platelet adhesion to fibrillar collagen was decreased in all subtypes of von Willebrand’s disease, but not as low as in severe von Willebrand’s disease. A close correlation was observed between platelet adhesion to collagen and plasma vWF:Ag in severe von Willebrand’s disease, subtype platelet low, subtype platelet discordant, and normal controls. The platelet adhesion in subtype platelet normal was higher than expected from the plasma vWF: Ag level. Perfusions in which washed platelets were added to a human albumin solution together with red blood cells gave similar adhesion values in subtype platelet normal and normal controls; adhesion was decreased in subtype platelet discordant, and the lowest values were found in subtype platelet low and in severe von Willebrand’s disease. These data indicate that platelet von Willebrand factor may contribute to platelet adhesion, when plasma von Willebrand factor is low. Perfusion studies over a fibroblast matrix gave similar low adhesion values for subtype platelet low and platelet normal, indicating that the contribution of platelet von Willebrand factor can only be observed on a strongly activating surface such as fibrillar collagen.

 

  • References

  • 1 Zimmerman TS, Ruggeri ZM. Von Willebrand’s disease. Clin Haematol 1983; 12: 175-200
    PubMedSearch in Google Scholar
  • 2 Mannucci PM, Lombardi R, Bader R, Vianello C, Federici AB, Solinas S, Mazzucconi MG, Mariani G. Heterogeneity of type I von Willebrand disease: Evidence for a subgroup with an abnormal von Willebrand factor. Blood 1985; 66: 796-802
    CrossrefPubMedSearch in Google Scholar
  • 3 Sakariassen KS, Aarts P AM M, DeGroot PG, Houdijk W PM, Sixma JJ. A perfusion chamber developed to investigate platelet interaction in flowing blood with human vessel wall cells, their extracellular matrix, and purified components. J Lab Clin Med 1983; 102: 522-535
    PubMedSearch in Google Scholar
  • 4 Muggli R, Baumgartner HR, Tschopp TB, Keller H. Automated microdensitometry and protein assays as a measure for platelet adhesion and aggregation on collagen-coated slides under controlled flow conditions. J Lab Clin Med 1980; 95: 195-207
    PubMedSearch in Google Scholar
  • 5 Sakariassen KS, Bolhuis PA, Sixma JJ. Adhesion of human blood platelets to human artery subendothelium is mediated by factor VIII-von Willebrand factor bound to subendothelium. Nature 1979; 279: 636-638
    CrossrefPubMedSearch in Google Scholar
  • 6 Steven FS. The effect of chelating agents on collagen interfibrillar matrix interactions in connective tissue. Biochim Biophys Acta 1967; 140: 522-528
    CrossrefPubMedSearch in Google Scholar
  • 7 Houdijk W PM, Sakariassen KS, Nievelstein P FE M, Sixma JJ. Role of factor VUI-von Willebrand factor and fibronectin in the interaction of platelets in flowing blood with monomeric and fibrillar collagen type I and . J Clin Invest 1985; 75: 531-540
    CrossrefPubMedSearch in Google Scholar
  • 8 Nievelstein P FE M, de Groot PhG, d’Alessio P, Heijnen H FG, Orlando E, Sixma JJ. Platelet adhesion to vascular cells: the matrix of cultured fibroblast as model to study the role of exogenous von Willebrand factor in platelet adhesion. Arteriosclerosis 1990; 10: 467-469
    PubMedSearch in Google Scholar
  • 9 Laurell CB. Electroimmunoassay. Scand J Clin Lab Invest 1972; 29: 21-37
    CrossrefPubMedSearch in Google Scholar
  • 10 Ruggeri ZM, Mannucci PM, Jeffcoate SC, Ingram G IC. Immunoradiometric assay of factor VIII related antigen, with observations in 32 patients with von Willebrand’s disease. Br J Haematol 1976; 33: 221-232
    CrossrefPubMedSearch in Google Scholar
  • 11 Sakariassen KS, Cattaneo M, Van den BergA, Ruggeri ZM, Mannucci PM, Sixma JJ. DDAVP enhances adherence and platelet aggregate growth on human artery subendothelium. Blood 1984; 64: 229-236
    PubMedSearch in Google Scholar
  • 12 Gralnick HR, Rick ME, Ruggeri ZM, McKneown LP, Williams SB, Parker RI, Maisonneuve P, Jeanneau C, Sultan Y. Platelet von Willebrand factor: An important determinant of the bleeding time in type I von Willebrand’s disease. Blood 1986; 68: 58-61
    CrossrefPubMedSearch in Google Scholar
  • 13 Sakariassen KS, Bolhuis PA, Blomback M, Thorell L, Blomback B, Sixma JJ. Association between bleeding time and platelet adherence to artery subendothelium. Thromb Haemostas 1984; 52: 144-148
    PubMedSearch in Google Scholar
  • 14 Fressinaud E, Baruch D, Rothschild C, Baumgartner HR, Meyer D. Platelet von Willebrand factor: evidence for its involvement in platelet adhesion to collagen. Blood 1987; 70: 1214-1217
    PubMedSearch in Google Scholar
  • 15 Castillo R, Escolar G, Monteagudo Y, Ordinas A, Garrido M, Moia M, Federici AB, Mannucci PM. Role for platelet von Willebrand factor in supporting platelet-vessel wall interactions in von Willebrand disease. Am J Hematol 1989; 31: 153-158
    CrossrefPubMedSearch in Google Scholar
  • 16 Bowie E JW, Solberg LA, Fass DN, Johnson CM, Knutson GJ, Stewart ML, Zoecklein LJ. Transplantation of normal bone marrow into a pig with severe von Willebrand’s disease. J Clin Invest 1986; 78: 26-30
    CrossrefPubMedSearch in Google Scholar
  • 17 Sixma JJ, Sakariassen KS, Beeser-Visser NH, Ottenhof-Rover M, Bolhuis PA. Adhesion of platelets to human artery subendothelium: effect of factor VUI-von Willebrand factor of various multimeric composition. Blood 1984; 63: 128-139
    PubMedSearch in Google Scholar