Klin Monbl Augenheilkd 2010; 227(4): 345-346
DOI: 10.1055/s-0029-1245253
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© Georg Thieme Verlag KG Stuttgart · New York

Intraocular Haemorrhages in von Willebrand’s Disease

Okuläre Hämorrhagien bei von Willebrand SyndromC. Valmaggia, W. Korte
Further Information

Publication History





Publication Date:
20 April 2010 (online)

Background

Von Willebrand’s disease (vWD) is a hereditary coagulation disorder that was first described by the Finnish physician, Erik von Willebrand in 1926 [7]. It is characterised by an abnormality or deficiency of the von Willebrand factor (vWF), a highly muItimerised glycoprotein that promotes platelet adhesion and aggregation and also acts as a carrier of the coagulation factor VIII [2]. The disease is mainly inherited as an incomplete autosomal dominant trait, although the expressivity of the genetic abnormality is highly variable. Cases of autosomal recessive and X-linked recessive inheritance have also been reported. The prevalence of vWD in the general population is c.1 %. Clinical features range from asymptomatic to severe and include gastrointestinal, cutaneous, and mucosal bleedings [5]. As far as we can ascertain, only two reports have been published that indicate an association between intraocular haemorrhages and any decrease in the vWF [3] [6].

References

  • 1 Budde U, Drewke E, Will K. et al . Diagnostic standards of von Willebrand disease.  Hamostaseologie. 2004;  24 12-26
  • 2 Castaman G, Federici A B, Rodeghiero F. et al . Von Willebrand’s disease in the year 2003: towards complete identification of gene defects for correct diagnosis and treatment.  Haematologica. 2003;  88 94-108
  • 3 Herrmann W A, Lohmann C P, Demmler-Hackenberg M. et al . Von Willebrand’s disease type I as cause for subvitreal, retinal and subretinal haemorrhages.  Graefe’s Arch Clin Exp Ophthalmol. 2005;  243 383-385
  • 4 Kaufmann J E, Vischer U M. Cellular mechanisms of the hemostatic effects of desmopressin.  J Throm Haemost. 2003;  1 682-689
  • 5 Schneppenheim R, Budde U. Classification of von Willebrand disease.  Haemostaseology. 2004;  24 27-36
  • 6 Shiono T, Abe S, Watabe T. et al . Vitreous retinal and subretinal hemorrhages associated with von Willebrand’s syndrome.  Graefe’s Arch Clin Exp Ophthalmol. 1992;  230 496-497
  • 7 Von Willebrand E A. Hereditar pseudohamofil.  Finsk Lakaresallsk Handl. 1926;  68 87-112

Christophe Valmaggia, MD

Department of Ophthalmology, Cantonal Hospital

CH-9007 St. Gallen, Switzerland

Phone: ++ 41/71 94 17 58

Fax: ++ 41/71/4 94 61 67

Email: christophe.valmaggia@kssg.ch

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