Acquired von Willebrand disease in a patient with angiodysplasia resulting from immune-mediated clearance of von Willebrand factor.
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.
Inbal
A
A
Department of Haematology, Institute of Thrombosis and Haemostasis, Sheba Medical Centre, Tel-Hashomer, Israel.
Bank
I
I
Zivelin
A
A
Varon
D
D
Dardik
R
R
Shapiro
R
R
Rosenthal
E
E
Shenkman
B
B
Gitel
S
S
Seligsohn
U
U
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*
D005719:gamma-Globulins; D014841:von Willebrand Factor