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Thromb Haemost 2006; 95(02): 212-220
DOI: 10.1160/TH05-08-0550
Blood Coagulation, Fibrinolysis and Cellular Haemostasis
Schattauer GmbH

Relation between ADAMTS13 activity and ADAMTS13 antigen levels in healthy donors and patients with thrombotic microangiopathies (TMA)

Manfred Rieger   *
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
,
Silvia Ferrari   *
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
,
Johanna A. Kremer Hovinga
2   Department of Hematology and Central Hematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
,
Christian Konetschny
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
,
Andrea Herzog
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
,
Letizia Koller
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
,
Alfred Weber
3   Baxter BioScience, Department of Preclinical Research and Development, Vienna, Austria
,
Giuseppe Remuzzi
4   Mario Negri Institute for Pharmacological Research, and Division of Nephrology and Dialysis, Azienda Ospedaliera, Ospedali Riuniti di Bergamo, Bergamo, Italy
,
Michael Dockal
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
,
Barbara Plaimauer
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
,
Friedrich Scheiflinger
1   Baxter Bioscience, Department of Discovery Research, Vienna, Austria
› Author AffiliationsFinancial support: This work was in part supported bya grant from Telethon (Grant No. GGPO2162) to GR.
Further Information

Publication History

Received 10 August 2005

Accepted after revision 08 January 2005

Publication Date:
28 November 2017 (online)

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Summary

We have established a new, enzyme-linked immunosorbent assay (ELISA) for the detection of ADAMTS13 antigen using purified polyclonal rabbit anti-human ADAMTS13 IgG. Normal plasma ADAMTS13 antigen levels span a concentration of 740 –1420ng/ml (median 1080ng/ml) resulting in an ADAMTS13 activity to antigen ratio of 0.48 to 1.68 U/µg. In a cohort of HUS patients, ADAMTS13 antigen was in the normal range, whereas in hereditary TTP patients antigen levels were low to undetectable, in concordance with severe deficient ADAMTS13 activity. Plasma of acquired TTP patients was found to contain free as well as autoantibody-bound ADAMTS13. We also present evidence for circulating anti-ADAMTS13 antibody/ADAMTS13 antigen immune complexes not only in acutely ill or actively treated patients but also in patients who have already achieved clinical remission. This new developed ADAMTS13 antigen ELISA assay allows rapid determination of ADAMTS13 antigen levels in human plasma but is of limited predictive value for the diagnosis or treatment of acquired TTP due to the detection of ADAMTS13 in antibody complexes.

Keywords

ADAMTS13 - ADAMTS13-antigen - ELISA - TTP - HUS

* Denotes equal contribution of S. F and M. R.


 

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