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Thromb Haemost 1993; 70(05): 758-761
DOI: 10.1055/s-0038-1649665
Clinical Studies
Schattauer GmbH Stuttgart

Autopsy Findings in Three Patients with von Willebrand Disease Type IIB and Type III: Presence of Atherosclerotic Lesions without Occlusive Arterial Thrombi

A B Federici
1   Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Institute of Internal Medicine, Iatly
,
P M Mannucci
1   Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Institute of Internal Medicine, Iatly
,
E Fogato
2   Institute of Pathology, I. R. C. C. S. Maggiore Hospital and University of Milan, Italy
,
P Ghidoni
2   Institute of Pathology, I. R. C. C. S. Maggiore Hospital and University of Milan, Italy
,
L Matturri
2   Institute of Pathology, I. R. C. C. S. Maggiore Hospital and University of Milan, Italy
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Publikationsverlauf

Received 05. Februar 1992

Accepted after revision 29. Juni 1993

Publikationsdatum:
05. Juli 2018 (online)

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Summary

It has been reported that pigs with severe von Willebrand disease (vWd) are protected from spontaneous and diet-induced atherosclerosis, but there are very few studies in human patients. Autopsies were carried out on three patients with vWd (one case with the type IIB variant and two cases with type III, a variant similar to that protecting pigs from atherosclerosis), aged 73, 44 and 52 years at the time of death. Hemorrhagic diathesis was an important cause of death for these patients. Atherosclerosis lesions were found in all patients, though of different extent and localization: severe lesions in all vascular areas in the patient with type IIB, few and slighter in all areas in the two patients with type III, except that in one of them there were moderately severe lesions in the coronary arteries. There was no clinical or pathologic evidence of thrombosis in the coronary arteries or other arteries. These data indicate that severe vWd did not protect completely against the development of atherosclerosis, although it is possible that intensive lifelong treatment with plasma concentrates had a modifying influence in these patients.

 

  • References

  • 1 Ruggeri ZM, Ware J. The structure and function of von Willebrand factor. Thromb Haemostas 1992; 67: 594-569
    PubMedGoogle Scholar
  • 2 Ruggeri ZM. Structure and function of vWf: relationship to vWd. Mayo Clin Proc 1991; 66: 847-861
    CrossrefPubMedGoogle Scholar
  • 3 Tschopp TP, Weiss HJ, Baumgartner HR. Decreased adhesion of platelets to subendothelium in von Willebrand’S disease. J Lab Clin Med 1974; 35: 124-138
    PubMedGoogle Scholar
  • 4 Ikeda Y, Handa M, Kawano K, Kamata T, Murata M, Araki Y, Anbo H, Kawai W, Itagaki I, Sakai K, Ruggeri ZM. The role of von Willebrand factor and fibrinogen in platelet aggregation under varying shear stress. J Clin Invest 1991; 87: 1234-1240
    CrossrefPubMedGoogle Scholar
  • 5 Fuster V, Bowie EJW, Lewis JC, Fass DN. Resistance to arteriosclerosis in pigs with von Willebrand’S disease. Spontaneous and high cholesterol diet-induced arteriosclerosis. J Clin Invest 1978; 61: 722-730
    CrossrefPubMedGoogle Scholar
  • 6 Badimon L, Steele P, Badimon JJ, Bowie EJW, Fuster V. Aortic atherosclerosis in pigs with heterozygous von Willebrand disease. Comparison with homozygous von Willebrand and normal pigs. Arteriosclerosis 1985; 5: 366-370
    CrossrefPubMedGoogle Scholar
  • 7 Nichols TC, Bellinger DA, Tate DA, Reddick RL, Read MS, Koch GG, Brinkhous M, Griggs TR. Von Willebrand factor and occlusive arterial thrombosis. A study in normal and von Willebrand’S disease pigs with diet-induced hypercholesterolemia and atherosclerosis. Arteriosclerosis 1990; 10: 449-461
    CrossrefPubMedGoogle Scholar
  • 8 Bellinger DA, Nichols TC, Read MS, Reddick RL, Lamb MA, Brinkhous KM, Evatt BL, Griggs TR. Prevention of occlusive coronary artery thrombosis by a murine monoclonal antibody to porcine von Willebrand factor. Proc Natl Acad Sci USA 1987; 84: 8100-8104
    CrossrefPubMedGoogle Scholar
  • 9 Riddick RL, Read MS, Brinkhous KM, Bellinger D, Nichols T, Griggs TR. Coronary atherosclerosis in the pig. Induced plaque injury and platelet response. Arteriosclerosis 1990; 10: 541-550
    CrossrefPubMedGoogle Scholar
  • 10 Fuster V, Badimon L, Badimon JJ, John HIP, Chesebro JH. The porcine model for the understanding of thrombogenesis and atherogenesis. Mayo Clin Proc 1991; 66: 818-831
    CrossrefPubMedGoogle Scholar
  • 11 Nichols TC, Bellinger DA, Reddick RL, Read MS, Koch GG, Brinkhous KM, Griggs TR. Role of von Willebrand factor in artherial thrombosis. Studies in normal and von Willebrand disease pigs. Circulation 1991; 83: 56-64
    PubMedGoogle Scholar
  • 12 Kernoff LM, Rose AG, Hughes J, Jacobs P. Autopsy findings in an elderly man suffering from severe von Willebrand’S disease. Thromb Haemost 1981; 46: 714-716
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 13 Mannucci PM, Federici AB, Cattaneo M, Fassati R, Galmarini D. Liver transplantation in severe von Willebrand disease. Lancet 1991; 337: 1105
    PubMedGoogle Scholar
  • 14 Silwer J, Cronberg S, Nilsson IM. Occurrence of arteriosclerosis in von Willebrand’S disease. Acta Med Scand 1966; 180: 475-484
    PubMedGoogle Scholar
  • 15 De GrootPG, Federici AB, De BoerHC, D’Alessio P, Mannucci PM, Sixma JJ. von Willebrand factor synthesized by endothelial cells from a patient type IIB von Willebrand disease supports platelet adhesion normally but has an increased affinity for platelets. Proc Natl Acad Sci USA 1992; 86: 3793-3797
    PubMedGoogle Scholar
  • 16 Dulhoste MN, Bonnet J, Vergnes C, Choussat A, Bricaud H. Maladie de Willebrand et atherosclerose coronarienne. Arch Mal Coeur 1989; 82: 1875-1878
    PubMedGoogle Scholar
  • 17 Moake JL, Turner NA, Stathopoulos NA, Nolasco LH, Heliums JD. Involvement of large plasma von Willebrand factor (vWf) multimers and unusually large vWf forms derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest 1986; 78: 1456-1461
    CrossrefPubMedGoogle Scholar