Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura-Reference-Cited by-同舟云学术

Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura

Published:2017-07-27 Issue:4 Volume:130 Page:466-471
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Alwan Ferras¹,Vendramin Chiara²,Vanhoorelbeke Karen³,Langley Katy²,McDonald Vickie⁴,Austin Steve⁵,Clark Amanda⁶,Lester William⁷,Gooding Richard⁸,Biss Tina⁹,Dutt Tina¹⁰,Cooper Nichola¹¹,Chapman Oliver¹²,Cranfield Tanya¹³,Douglas Kenny¹⁴,Watson H. G.¹⁵,van Veen J. J.¹⁶,Sibson Keith¹⁷,Thomas William¹⁸,Manson Lynn¹⁹,Hill Quentin A.²⁰,Benjamin Sylvia²¹,Ellis Debra¹,Westwood John-Paul¹,Thomas Mari¹²²,Scully Marie¹²²

Affiliation:

1. Department of Haematology and

2. Haemostasis Research Unit, University College London, London, United Kingdom;

3. Laboratory for Thrombosis Research, Katholieke Universiteit Leuven Campus Kulak Kortrijk, Kortrijk, Belgium;

4. Department of Haematology, Guys and St Thomas National Health Service (NHS) Trust, London, United Kingdom;

5. Department of Haematology, St George's NHS Trust, London, United Kingdom;

6. Bristol Haemophilia Centre, University Hospitals Bristol Foundation Trust, Bristol, United Kingdom;

7. Department of Haematology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom;

8. Department of Haematology, University Hospitals of Leicester, Leicester, United Kingdom;

9. Department of Haematology, The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, United Kingdom;

10. Roald Dahl Haemostasis and Thrombosis Centre, Royal Liverpool University Hospital, Liverpool, United Kingdom;

11. Department of Haematology, Imperial College NHS Trust, London, United Kingdom;

12. Department of Haematology, University Hospitals Coventry and Warwickshire NHS Trust, Coventry, United Kingdom;

13. Department of Haematology, Queen Alexandra Hospital, Portsmouth, United Kingdom;

14. Scottish National Blood Transfusion Service (SNBTS) Clinical Apheresis Unit, Beatson West of Scotland Cancer Centre, Glasgow, United Kingdom;

15. Department of Haematology, Aberdeen Royal Infirmary, Aberdeen, United Kingdom;

16. Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom;

17. Department of Haematology, Great Ormond Street Hospital, London, United Kingdom;

18. Department of Haematology, Addenbrookes Hospital, Cambridge, United Kingdom;

19. SNBTS Department of Transfusion Medicine, Royal Infirmary of Edinburgh, Edinburgh, United Kingdom;

20. Department of Haematology, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom;

21. Department of Clinical Haematology, Oxford University Hospitals, Oxford, United Kingdom; and

22. National Institute for Health Research Cardiometabolic Programme, University College London (UCL) Hospitals/UCL Biomedical Research Centre, London, United Kingdom

Abstract

Key Points High anti-ADAMTS13 antibody and low ADAMTS13 antigen levels adversely affect outcome in immune-mediated TTP with greater mortality seen. A raised troponin at presentation confers a sixfold increase and reduced GCS a nine-fold increase in mortality in acute TTP.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/130/4/466/1404888/blood758656.pdf

Reference30 articles.

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4. Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy;Mariotte;Lancet Haematol,2016

5. The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry: a community perspective of patients with clinically diagnosed TTP-HUS;George;Semin Hematol,2004

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