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. 1995 Mar 28;92(7):2428–2432. doi: 10.1073/pnas.92.7.2428

Platelet von Willebrand factor in inherited and acquired bleeding disorders.

P M Mannucci 1
PMCID: PMC42231  PMID: 7708659

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BIGGS R., MATTHEWS J. M. The treatment of haemorrhage in von Willebrand's disease and the blood level of factor VIII (AHG). Br J Haematol. 1963 Apr;9:203–214. doi: 10.1111/j.1365-2141.1963.tb05458.x. [DOI] [PubMed] [Google Scholar]
  2. Berkowitz S. D., Federici A. B. Sialic acid prevents loss of large von Willebrand factor multimers by protecting against amino-terminal proteolytic cleavage. Blood. 1988 Nov;72(5):1790–1796. [PubMed] [Google Scholar]
  3. Blatt P. M., Brinkhous K. M., Culp H. R., Krauss J. S., Roberts H. R. Antihemophilic factor concentrate therapy in von Willebrand disease. Dissociation of bleeding-time factor and ristocetin-cofactor activities. JAMA. 1976 Dec 13;236(24):2770–2772. [PubMed] [Google Scholar]
  4. Bouma B. N., Hordijk-Hos J. M., DeGraaf S., Sixma J. J., Van Mourik J. A. Presence of factor VIII-related antigen in blood platelets of patients with Von Willebrand's disease. Nature. 1975 Oct 9;257(5526):510–512. doi: 10.1038/257510a0. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Bowie E. J., Solberg L. A., Jr, Fass D. N., Johnson C. M., Knutson G. J., Stewart M. L., Zoecklein L. J. Transplantation of normal bone marrow into a pig with severe von Willebrand's disease. J Clin Invest. 1986 Jul;78(1):26–30. doi: 10.1172/JCI112560. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Castaman G., Rodeghiero F., Lattuada A., La Greca G., Mannucci P. M. Multimeric pattern of plasma and platelet von Willebrand factor is normal in uremic patients. Am J Hematol. 1993 Dec;44(4):266–269. doi: 10.1002/ajh.2830440409. [DOI] [PubMed] [Google Scholar]
  7. Castillo R., Monteagudo J., Escolar G., Ordinas A., Magallón M., Martín Villar J. Hemostatic effect of normal platelet transfusion in severe von Willebrand disease patients. Blood. 1991 May 1;77(9):1901–1905. [PubMed] [Google Scholar]
  8. Coller B. S., Hirschman R. J., Gralnick H. R. Studies on the Factor VIII/von Willebrand factor antigen on human platelets. Thromb Res. 1975 Jun;6(6):469–480. doi: 10.1016/0049-3848(75)90059-6. [DOI] [PubMed] [Google Scholar]
  9. Ewenstein B. M., Inbal A., Pober J. S., Handin R. I. Molecular studies of von Willebrand disease: reduced von Willebrand factor biosynthesis, storage, and release in endothelial cells derived from patients with type I von Willebrand disease. Blood. 1990 Apr 1;75(7):1466–1472. [PubMed] [Google Scholar]
  10. Federici A. B., de Groot P. G., Moia M., Ijsseldijk M. J., Sixma J. J., Mannucci P. M. Type I von Willebrand disease, subtype 'platelet low': decreased platelet adhesion can be explained by low synthesis of von Willebrand factor in endothelial cells. Br J Haematol. 1993 Jan;83(1):88–93. doi: 10.1111/j.1365-2141.1993.tb04636.x. [DOI] [PubMed] [Google Scholar]
  11. Fernandez M. F., Ginsberg M. H., Ruggeri Z. M., Batlle F. J., Zimmerman T. S. Multimeric structure of platelet factor VIII/von Willebrand factor: the presence of larger multimers and their reassociation with thrombin-stimulated platelets. Blood. 1982 Nov;60(5):1132–1138. [PubMed] [Google Scholar]
  12. Fressinaud E., Baruch D., Rothschild C., Baumgartner H. R., Meyer D. Platelet von Willebrand factor: evidence for its involvement in platelet adhesion to collagen. Blood. 1987 Oct;70(4):1214–1217. [PubMed] [Google Scholar]
  13. Fressinaud E., Federici A. B., Castaman G., Rothschild C., Rodeghiero F., Baumgartner H. R., Mannucci P. M., Meyer D. The role of platelet von Willebrand factor in platelet adhesion and thrombus formation: a study of 34 patients with various subtypes of type I von Willebrand disease. Br J Haematol. 1994 Feb;86(2):327–332. doi: 10.1111/j.1365-2141.1994.tb04734.x. [DOI] [PubMed] [Google Scholar]
  14. Gralnick H. R., McKeown L. P., Williams S. B., Shafer B. C., Pierce L. Plasma and platelet von Willebrand factor defects in uremia. Am J Med. 1988 Dec;85(6):806–810. doi: 10.1016/s0002-9343(88)80025-1. [DOI] [PubMed] [Google Scholar]
  15. Gralnick H. R., Rick M. E., McKeown L. P., Williams S. B., Parker R. I., Maisonneuve P., Jenneau C., Sultan Y. Platelet von Willebrand factor: an important determinant of the bleeding time in type I von Willebrand's disease. Blood. 1986 Jul;68(1):58–61. [PubMed] [Google Scholar]
  16. Gralnick H. R., Williams S. B., Coller B. S. Fibrinogen competes with von Willebrand factor for binding to the glycoprotein IIb/IIIa complex when platelets are stimulated with thrombin. Blood. 1984 Oct;64(4):797–800. [PubMed] [Google Scholar]
  17. Gralnick H. R., Williams S. B., McKeown L. P., Krizek D. M., Shafer B. C., Rick M. E. Platelet von Willebrand factor: comparison with plasma von Willebrand factor. Thromb Res. 1985 Jun 15;38(6):623–633. doi: 10.1016/0049-3848(85)90205-1. [DOI] [PubMed] [Google Scholar]
  18. Green D., Potter E. V. Failure of AHF concentration to control bleeding in von Willebrand's disease. Am J Med. 1976 Mar;60(3):357–360. doi: 10.1016/0002-9343(76)90751-8. [DOI] [PubMed] [Google Scholar]
  19. Griggs T. R., Webster W. P., Cooper H. A., Wagner R. H., Brinkhous K. M. Von Willebrand factor: gene dosage relationships and transfusion response in bleeder swine--a new bioassay. Proc Natl Acad Sci U S A. 1974 May;71(5):2087–2090. doi: 10.1073/pnas.71.5.2087. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Howard M. A., Montgomery D. C., Hardisty R. M. Factor-VIII-related antigen in platelets. Thromb Res. 1974 May;4(5):617–624. doi: 10.1016/0049-3848(74)90218-7. [DOI] [PubMed] [Google Scholar]
  21. Jaffe E. A., Hoyer L. W., Nachman R. L. Synthesis of antihemophilic factor antigen by cultured human endothelial cells. J Clin Invest. 1973 Nov;52(11):2757–2764. doi: 10.1172/JCI107471. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Koutts J., Walsh P. N., Plow E. F., Fenton J. W., 2nd, Bouma B. N., Zimmerman T. S. Active release of human platelet factor VIII-related antigen by adenosine diphosphate, collagen, and thrombin. J Clin Invest. 1978 Dec;62(6):1255–1263. doi: 10.1172/JCI109246. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Larrieu M. J., Caen J. P., Meyer D. O., Vainer H., Sultan Y., Bernard J. Congenital bleeding disorders with long bleeding time and normal platelet count. II. Von Willebrand's disease (report of thirty-seven patients). Am J Med. 1968 Sep;45(3):354–372. doi: 10.1016/0002-9343(68)90070-3. [DOI] [PubMed] [Google Scholar]
  24. Lattuada A., Varanukulsak P., Castaman G. C., Mannucci P. M. The response of plasma von Willebrand factor to desmopressin (DDAVP) is related to the platelet levels of von Willebrand factor. Thromb Res. 1992 Aug 15;67(4):467–471. doi: 10.1016/0049-3848(92)90276-g. [DOI] [PubMed] [Google Scholar]
  25. López-Fernández M. F., López-Berges C., Nieto J., Martín R., Batlle J. Platelet and plasma von Willebrand factor: structural differences. Thromb Res. 1986 Oct 1;44(1):125–128. doi: 10.1016/0049-3848(86)90188-x. [DOI] [PubMed] [Google Scholar]
  26. Mannucci P. M., Lattuada A., Castaman G., Lombardi R., Colibretti M. L., Ciavarella N., Rodeghiero F. Heterogeneous phenotypes of platelet and plasma von Willebrand factor in obligatory heterozygotes for severe von Willebrand disease. Blood. 1989 Nov 15;74(7):2433–2436. [PubMed] [Google Scholar]
  27. Mannucci P. M., Lombardi R., Bader R., Horellou M. H., Finazzi G., Besana C., Conard J., Samama M. Studies of the pathophysiology of acquired von Willebrand's disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies. Blood. 1984 Sep;64(3):614–621. [PubMed] [Google Scholar]
  28. Mannucci P. M., Lombardi R., Bader R., Vianello L., Federici A. B., Solinas S., Mazzucconi M. G., Mariani G. Heterogeneity of type I von Willebrand disease: evidence for a subgroup with an abnormal von Willebrand factor. Blood. 1985 Oct;66(4):796–802. [PubMed] [Google Scholar]
  29. Mannucci P. M., Moia M., Rebulla P., Altieri D., Monteagudo J., Castillo R. Correction of the bleeding time in treated patients with severe von Willebrand disease is not solely dependent on the normal multimeric structure of plasma von Willebrand factor. Am J Hematol. 1987 May;25(1):55–65. doi: 10.1002/ajh.2830250106. [DOI] [PubMed] [Google Scholar]
  30. Mannucci P. M., Ruggeri Z. M., Ciavarella N., Kazatchkine M. D., Mowbray J. F. Precipitating antibodies to factor VIII/von Willebrand factor in von Willebrand's disease: effects on replacement therapy. Blood. 1981 Jan;57(1):25–31. [PubMed] [Google Scholar]
  31. Mannucci P. M., Tenconi P. M., Castaman G., Rodeghiero F. Comparison of four virus-inactivated plasma concentrates for treatment of severe von Willebrand disease: a cross-over randomized trial. Blood. 1992 Jun 15;79(12):3130–3137. [PubMed] [Google Scholar]
  32. Meschengieser S., Blanco A., Woods A., Maugeri N., Fernandez J., Dupont J., Lazzari M. A. Intraplatelet levels of vWF:Ag and fibrinogen in myeloproliferative disorders. Thromb Res. 1987 Nov 1;48(3):311–319. doi: 10.1016/0049-3848(87)90443-9. [DOI] [PubMed] [Google Scholar]
  33. Nichols T. C., Samama C. M., Bellinger D. A., Roussi J., Reddick R. L., Bonneau M., Read M. S., Bailliart O., Koch G. G., Vaiman M. Function of von Willebrand factor after crossed bone marrow transplantation between normal and von Willebrand disease pigs: effect on arterial thrombosis in chimeras. Proc Natl Acad Sci U S A. 1995 Mar 28;92(7):2455–2459. doi: 10.1073/pnas.92.7.2455. [DOI] [PMC free article] [PubMed] [Google Scholar]
  34. Parker R. I., McKeown L. P., Gallin J. I., Gralnick H. R. Absence of the largest platelet-von Willebrand multimers in a patient with lactoferrin deficiency and a bleeding tendency. Thromb Haemost. 1992 Mar 2;67(3):320–324. [PubMed] [Google Scholar]
  35. Perkins H. A. Correction of the hemostatic defects in Von Willebrand's disease. Blood. 1967 Sep;30(3):375–380. [PubMed] [Google Scholar]
  36. Rodeghiero F., Castaman G., Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood. 1987 Feb;69(2):454–459. [PubMed] [Google Scholar]
  37. Rodeghiero F., Castaman G., Ruggeri M., Tosetto A. The bleeding time in normal subjects is mainly determined by platelet von Willebrand factor and is independent from blood group. Thromb Res. 1992 Mar 1;65(4-5):605–615. doi: 10.1016/0049-3848(92)90210-2. [DOI] [PubMed] [Google Scholar]
  38. Ruggeri Z. M., Mannucci P. M., Bader R., Barbui T. Factor VIII-related properties in platelets from patients with von Willebrand's disease. J Lab Clin Med. 1978 Jan;91(1):132–140. [PubMed] [Google Scholar]
  39. Ruggeri Z. M., Zimmerman T. S. 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 Jun;65(6):1318–1325. doi: 10.1172/JCI109795. [DOI] [PMC free article] [PubMed] [Google Scholar]
  40. Sadler J. E., Gralnick H. R. Commentary: a new classification for von Willebrand disease. Blood. 1994 Aug 1;84(3):676–679. [PubMed] [Google Scholar]
  41. Sakariassen K. S., Aarts P. A., de Groot P. G., Houdijk W. P., Sixma J. J. 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 Oct;102(4):522–535. [PubMed] [Google Scholar]
  42. Sakariassen K. S., Bolhuis P. A., Blombäck M., Thorell L., Blombäck B., Sixma J. J. Association between bleeding time and platelet adherence to artery subendothelium. Thromb Haemost. 1984 Oct 31;52(2):144–147. [PubMed] [Google Scholar]
  43. Sakariassen K. S., Joss R., Muggli R., Kuhn H., Tschopp T. B., Sage H., Baumgartner H. R. Collagen type III induced ex vivo thrombogenesis in humans. Role of platelets and leukocytes in deposition of fibrin. Arteriosclerosis. 1990 Mar-Apr;10(2):276–284. doi: 10.1161/01.atv.10.2.276. [DOI] [PubMed] [Google Scholar]
  44. Sakariassen K. S., Nievelstein P. F., Coller B. S., Sixma J. J. The role of platelet membrane glycoproteins Ib and IIb-IIIa in platelet adherence to human artery subendothelium. Br J Haematol. 1986 Aug;63(4):681–691. doi: 10.1111/j.1365-2141.1986.tb07552.x. [DOI] [PubMed] [Google Scholar]
  45. Sixma J. J., Schiphorst M. E., Verweij C. L., Pannekoek H. Effect of deletion of the A1 domain of von Willebrand factor on its binding to heparin, collagen and platelets in the presence of ristocetin. Eur J Biochem. 1991 Mar 14;196(2):369–375. doi: 10.1111/j.1432-1033.1991.tb15826.x. [DOI] [PubMed] [Google Scholar]
  46. Smiley R. K., Tittley P., Rock G. Studies on the prolonged bleeding time in von Willebrand's disease. Thromb Res. 1989 Mar 1;53(5):417–426. doi: 10.1016/0049-3848(89)90196-5. [DOI] [PubMed] [Google Scholar]
  47. Sporn L. A., Chavin S. I., Marder V. J., Wagner D. D. Biosynthesis of von Willebrand protein by human megakaryocytes. J Clin Invest. 1985 Sep;76(3):1102–1106. doi: 10.1172/JCI112064. [DOI] [PMC free article] [PubMed] [Google Scholar]
  48. Sultan Y., Jeanneau C., Lamaziere J., Maisonneuve P., Caen J. P. Platelet factor VIII-related antigen: studies in vivo after transfusion in patients with von Willebrand disease. Blood. 1978 Apr;51(4):751–761. [PubMed] [Google Scholar]
  49. Takahashi H., Shibata A. Platelet factor VIII-related antigen in patients with chronic myeloproliferative disorders. Thromb Res. 1980 Aug 1;19(3):423–427. doi: 10.1016/0049-3848(80)90271-6. [DOI] [PubMed] [Google Scholar]
  50. Weiss H. J., Pietu G., Rabinowitz R., Girma J. P., Rogers J., Meyer D. Heterogeneous abnormalities in the multimeric structure, antigenic properties, and plasma-platelet content of factor VIII/von Willebrand factor in subtypes of classic (type I) and variant (type IIA) von Willebrand's disease. J Lab Clin Med. 1983 Mar;101(3):411–425. [PubMed] [Google Scholar]
  51. Wencel-Drake J. D., Painter R. G., Zimmerman T. S., Ginsberg M. H. Ultrastructural localization of human platelet thrombospondin, fibrinogen, fibronectin, and von Willebrand factor in frozen thin section. Blood. 1985 Apr;65(4):929–938. [PubMed] [Google Scholar]
  52. Williams S. B., McKeown L. P., Krutzsch H., Hansmann K., Gralnick H. R. Purification and characterization of human platelet von Willebrand factor. Br J Haematol. 1994 Nov;88(3):582–591. doi: 10.1111/j.1365-2141.1994.tb05077.x. [DOI] [PubMed] [Google Scholar]
  53. d'Alessio P., Zwaginga J. J., de Boer H. C., Federici A. B., Rodeghiero F., Castaman G., Mariani G., Mannucci P. M., de Groot P. G., Sixma J. J. Platelet adhesion to collagen in subtypes of type I von Willebrand's disease is dependent on platelet von Willebrand factor. Thromb Haemost. 1990 Oct 22;64(2):227–231. [PubMed] [Google Scholar]

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