Nonneutralizing IgM and IgG antibodies to von Willebrand factor–cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura

Acquired thrombotic thrombocytopenic purpura (TTP) has been linked to severe deficiency of ADAMTS-13 activity caused by autoantibodies inhibitory to ADAMTS-13. We report data on a patient with confirmed TTP who had severely reduced ADAMTS-13 activity but showed no ADAMTS-13 inhibition in a widely us...

Full description

Saved in:
Bibliographic Details
Published in:Blood 2003-11, Vol.102 (9), p.3241-3243
Main Authors: Scheiflinger, Friedrich, Knöbl, Paul, Trattner, Bettina, Plaimauer, Barbara, Mohr, Gabriele, Dockal, Michael, Dorner, Friedrich, Rieger, Manfred
Format: Article
Language:English
Subjects:
ADAM Proteins
ADAMTS13 Protein
Aged
Autoantibodies - blood
Autoantibodies - immunology
Biological and medical sciences
Enzyme-Linked Immunosorbent Assay
Female
Hematologic and hematopoietic diseases
Humans
Immunoglobulin G - blood
Immunoglobulin M - blood
Medical sciences
Metalloendopeptidases - immunology
Platelet diseases and coagulopathies
Purpura, Thrombotic Thrombocytopenic - etiology
Purpura, Thrombotic Thrombocytopenic - immunology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Acquired thrombotic thrombocytopenic purpura (TTP) has been linked to severe deficiency of ADAMTS-13 activity caused by autoantibodies inhibitory to ADAMTS-13. We report data on a patient with confirmed TTP who had severely reduced ADAMTS-13 activity but showed no ADAMTS-13 inhibition in a widely used fluid phase activity assay. With a newly developed enzyme-linked immunosorbent assay, using immobilized recombinant ADAMTS-13, we found high titers of IgM and IgG antibodies that bound to ADAMTS-13, but did not neutralize protease activity. These autoantibodies probably influenced the half-life of ADAMTS-13 or its binding to the endothelial cell surface, thereby compromising ADAMTS-13 activity in vivo. Given that ADAMTS-13 may interact physiologically with various receptors or ligands, the occurrence, distribution, and the epitope mapping of nonneutralizing antibodies will be an important area for future research.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2003-05-1616