Intraocular Haemorrhages in von Willebrand’s Disease

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

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