Premium
Acquired von Willebrand disease in a patient with angiodysplasia resulting from immune‐mediated clearance of von Willebrand factor
Author(s) -
Inbal A.,
Bank I.,
Zivelin A.,
Varon D.,
Dardik R.,
Shapiro R.,
Rosenthal E.,
Shenkman B.,
Gitel S.,
Seligsohn U.
Publication year - 1997
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1997.d01-1987.x
Subject(s) - von willebrand factor , von willebrand disease , medicine , immunology , antibody , hemostasis , immune system , antigen , gastroenterology , platelet
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.