Absence of ristocetin aggregation factor from the skin of a patient with von Willebrand's disease.
Samples of apparently normal skin from one patient with von Willebrand's disease (vWD) and five patients without vWD were examined with fluorescein-tagged antiserum to the component of factor VIII required for aggregation of platelets by ristocetin (VIII-R.A.F.). No evidence of VIII-R.A.F. was found in the vWD skin, while bright granules were seen on and/or in the endothelial cells of dermal capillaries in all patients without vWD. VIII-R.A.F. granules were also found in the interstitial vasculature of all of twelve renal-biopsy specimens from patients without vWD. These observations support the concept that an abnormality of the vascular endothelium is involved in the pathogenesis of vWD.
D001792:Blood Platelets / Q000032:analysis; D002196:Capillaries / Q000032:analysis / Q000473:pathology*; D005169:Factor VIII; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007106:Immune Sera; D007668:Kidney / Q000473:pathology; D010974:Platelet Aggregation / Q000187:drug effects; D012310:Ristocetin / Q000494:pharmacology*; D012867:Skin / Q000098:blood supply*; D014842:von Willebrand Diseases / Q000097:blood / Q000209:etiology / Q000473:pathology*
D007106:Immune Sera; D012310:Ristocetin; D005169:Factor VIII