title: Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia: relationship with platelet count.
abstract: We present a 4-year follow-up of a 42-year-old patient with primary thrombocythemia whose clinical course was complicated by two major mucocutaneous bleeding episodes. On both occasions an acquired functional von Willebrand factor deficiency was demonstrated. In contrast to what is reported in the literature, an inverse relationship between platelet number and plasma high-molecular-weight multimers of von Willebrand factor was established.
authors.affiliation: Department of Hematology, University Hospital Dijkzigt, Erasmus University, Rotterdam, The Netherlands.
authors.forename: P J
authors.initials: PJ
authors.lastname: van Genderen
authors.forename: J J
authors.initials: JJ
authors.lastname: Michiels
authors.forename: S C
authors.initials: SC
authors.lastname: van der Poel-van de Luytgaarde
authors.forename: H H
authors.initials: HH
authors.lastname: van Vliet
mesh_terms: D000328:Adult; D001760:Bleeding Time; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D009092:Mucous Membrane; D010976:Platelet Count / Q000187:drug effects; D012871:Skin Diseases / Q000209:etiology*; D013922:Thrombocytosis / Q000150:complications* / Q000188:drug therapy; D014842:von Willebrand Diseases / Q000150:complications* / Q000503:physiopathology; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D014841:von Willebrand Factor; D006918:Hydroxyurea