title: Acquired von Willebrand disease in a patient with angiodysplasia resulting from immune-mediated clearance of von Willebrand factor.
abstract: A patient with a severe bleeding tendency due to acquired von Willebrand disease (VWD) is presented. Although no underlying disorder has emerged during 6 years of follow-up, an immune-mediated mechanism was responsible for acquired VWD in this patient as demonstrated by detection of von Willebrand factor (VWF)/anti-VWF complexes in the patient's plasma and their removal by protein A-sepharose beads and resumption of normal haemostasis with correction of VWF antigen, VWF activity and VWF multimeric pattern after treatment of the patient with high-dose gammaglobulin. Detection of anti-VWF antibodies in the patient's plasma had a significant impact on the choice of therapeutic intervention to control bleeding.
authors.affiliation: Department of Haematology, Institute of Thrombosis and Haemostasis, Sheba Medical Centre, Tel-Hashomer, Israel.
authors.forename: A
authors.initials: A
authors.lastname: Inbal
authors.forename: I
authors.initials: I
authors.lastname: Bank
authors.forename: A
authors.initials: A
authors.lastname: Zivelin
authors.forename: D
authors.initials: D
authors.lastname: Varon
authors.forename: R
authors.initials: R
authors.lastname: Dardik
authors.forename: R
authors.initials: R
authors.lastname: Shapiro
authors.forename: E
authors.initials: E
authors.lastname: Rosenthal
authors.forename: B
authors.initials: B
authors.lastname: Shenkman
authors.forename: S
authors.initials: S
authors.lastname: Gitel
authors.forename: U
authors.initials: U
authors.lastname: Seligsohn
mesh_terms: D016888:Angiodysplasia / Q000276:immunology*; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006801:Humans; D007116:Immunization, Passive; D008297:Male; D008875:Middle Aged; D012007:Rectum; D005719:gamma-Globulins / Q000008:administration & dosage; D014842:von Willebrand Diseases / Q000150:complications / Q000276:immunology* / Q000628:therapy; D014841:von Willebrand Factor / Q000276:immunology*
chemical_list: D005719:gamma-Globulins; D014841:von Willebrand Factor