title: Three cases of acquired von Willebrand disease associated with systemic lupus erythematosus.
abstract: Acquired von Willebrand disease associated with systemic lupus erythematosus (SLE) has been detected in three middle-aged women. In each case the first clinical manifestation was a bleeding syndrome. Plasma levels of von Willebrand factor (VWF) and ristocetin-induced platelet agglutination were as found in type 1 von Willebrand disease for the first patient, type 3 for the second patient, and type 2 for the third patient. Intraplatelet levels of VWF were normal for all three patients. In all cases a mixture of patient's plasma with normal plasma resulted in inhibition of ristocetin-induced binding of VWF to normal platelets. Intravenous immunoglobulin given to patients 2 and 3 corrected the plasma VWF level of the second patient but not that of the third. Therapy with corticosteroids was partially beneficial for patient 3 and patient 2. For patient 2, the severity of the cutaneous lesions also led to the use of cyclophosphamide, and this therapy resulted in total correction of VWF levels. Our observations confirm previous reports of acquired von Willebrand syndrome associated with SLE and show heterogeneity both in the phenotypic form and in the response to treatment.
authors.affiliation: Clinique de Médecine Interne et Maladies Infectieuses, Hôpital Haut-Lévêque, Pessac, France.
authors.forename: J F
authors.initials: JF
authors.lastname: Viallard
authors.forename: J L
authors.initials: JL
authors.lastname: Pellegrin
authors.forename: C
authors.initials: C
authors.lastname: Vergnes
authors.forename: A
authors.initials: A
authors.lastname: Borel-Derlon
authors.forename: G
authors.initials: G
authors.lastname: Clofent-Sanchez
authors.forename: A T
authors.initials: AT
authors.lastname: Nurden
authors.forename: B
authors.initials: B
authors.lastname: Leng
authors.forename: P
authors.initials: P
authors.lastname: Nurden
mesh_terms: D000368:Aged; D005260:Female; D006474:Hemorrhagic Disorders / Q000209:etiology; D006801:Humans; D016756:Immunoglobulins, Intravenous; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications*; D008875:Middle Aged; D014842:von Willebrand Diseases / Q000097:blood / Q000209:etiology* / Q000628:therapy; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D016756:Immunoglobulins, Intravenous; D014841:von Willebrand Factor