An optimized fluorogenic ADAMTS13 assay with increased sensitivity for the investigation of patients with thrombotic thrombocytopenic purpura

Journal of Thrombosis and Haemostasis

Volumn 11, Issue 8, 2013, Pages 1511-1518

  Muia, J ^a   Gao, W ^a   Haberichter, S L ^b   Dolatshahi, L ^a   Zhu, J ^a   Westfield, L A ^a   Covill, S C ^b   Friedman, K D ^b   Sadler, J E ^a  

^a WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b BLOOD RESEARCH INSTITUTE   (United States)

Author keywords

ADAMTS13 protein; Human; Kinetics; Recombinant fusion proteins; Substrate specificity; Thrombotic thrombocytopenic purpura; Von Willebrand factor

Indexed keywords

BILIRUBIN; CITRIC ACID; HEMOGLOBIN; HEPARIN; POLYPEPTIDE; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ADULT; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME ASSAY; FEMALE; HUMAN; LIMIT OF DETECTION; MALE; PH; PRIORITY JOURNAL; THROMBOTIC THROMBOCYTOPENIC PURPURA;

ADAMTS13 PROTEIN; HUMAN; KINETICS; RECOMBINANT FUSION PROTEINS; SUBSTRATE SPECIFICITY; THROMBOTIC THROMBOCYTOPENIC PURPURA; VON WILLEBRAND FACTOR;

ADAM PROTEINS; ANTICOAGULANTS; CASE-CONTROL STUDIES; FEMALE; FLUORESCENT DYES; HUMANS; MALE; MUTATION; PEPTIDES; PLASMIDS; PURPURA, THROMBOTIC THROMBOCYTOPENIC; RECOMBINANT FUSION PROTEINS; REPRODUCIBILITY OF RESULTS; SENSITIVITY AND SPECIFICITY; SUBSTRATE SPECIFICITY; THROMBOCYTOPENIA; THROMBOTIC MICROANGIOPATHIES; VON WILLEBRAND FACTOR;

EID: 84881590048     PISSN: 15387933     EISSN: 15387836     Source Type: Journal    
DOI: 10.1111/jth.12319     Document Type: Article

References (35)

1. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lammle B. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998; 339: 1578-84.
2. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339: 1585-94.
3. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 1991; 325: 393-7.
4. Furlan M, Robles R, Lammle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996; 87: 4223-34.
5. Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87: 4235-44.
6. Gerritsen HE, Turecek PL, Schwarz HP, Lammle B, Furlan M. Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP). Thromb Haemost 1999; 82: 1386-9.
7. Kokame K, Nobe Y, Kokubo Y, Okayama A, Miyata T. FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay. Br J Haematol 2005; 129: 93-100.
8. Tripodi A, Peyvandi F, Chantarangkul V, Palla R, Afrasiabi A, Canciani MT, Chung DW, Ferrari S, Fujimura Y, Karimi M, Kokame K, Kremer Hovinga JA, Lammle B, de Meyer SF, Plaimauer B, Vanhoorelbeke K, Varadi K, Mannucci PM. Second international collaborative study evaluating performance characteristics of methods measuring the von Willebrand factor cleaving protease (ADAMTS-13). J Thromb Haemost 2008; 6: 1534-41.
9. Kremer Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010; 115: 1500-11.
10. Palla R, Valsecchi C, Bajetta M, Spreafico M, De Cristofaro R, Peyvandi F. Evaluation of assay methods to measure plasma ADAMTS13 activity in thrombotic microangiopathies. Thromb Haemost 2011; 105: 381-5.
11. Di Stasio E, Lancellotti S, Peyvandi F, Palla R, Mannucci PM, De Cristofaro R. Mechanistic studies on ADAMTS13 catalysis. Biophys J 2008; 95: 2450-61.
12. Meyer SC, Sulzer I, Lammle B, Kremer Hovinga JA. Hyperbilirubinemia interferes with ADAMTS-13 activity measurement by FRETS-VWF73 assay: diagnostic relevance in patients suffering from acute thrombotic microangiopathies. J Thromb Haemost 2007; 5: 866-7.
13. Froehlich-Zahnd R, George JN, Vesely SK, Terrell DR, Aboulfatova K, Dong JF, Luken BM, Voorberg J, Budde U, Sulzer I, Lammle B, Kremer Hovinga JA. Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura. Haematologica 2012; 97: 297-303.
14. Matsushita T, Sadler JE. Identification of amino acid residues essential for von Willebrand factor binding to platelet glycoprotein Ib. Charged-to-alanine scanning mutagenesis of the A1 domain of human von Willebrand factor. J Biol Chem 1995; 270: 13406-14.
15. Aslanidis C, de Jong PJ. Ligation-independent cloning of PCR products (LIC-PCR). Nucleic Acids Res 1990; 18: 6069-74.
16. Kapust RB, Waugh DS. Escherichia coli maltose-binding protein is uncommonly effective at promoting the solubility of polypeptides to which it is fused. Protein Sci 1999; 8: 1668-74.
17. Feys HB, Liu F, Dong N, Pareyn I, Vauterin S, Vandeputte N, Noppe W, Ruan C, Deckmyn H, Vanhoorelbeke K. ADAMTS-13 plasma level determination uncovers antigen absence in acquired thrombotic thrombocytopenic purpura and ethnic differences. J Thromb Haemost 2006; 4: 955-62.
18. Feys HB, Anderson PJ, Vanhoorelbeke K, Majerus EM, Sadler JE. Multi-step binding of ADAMTS-13 to von Willebrand factor. J Thromb Haemost 2009; 7: 2088-95.
19. Anderson PJ, Kokame K, Sadler JE. Zinc and calcium ions cooperatively modulate ADAMTS13 activity. J Biol Chem 2006; 281: 850-7.
20. Studt JD, Kremer Hovinga JA, Antoine G, Hermann M, Rieger M, Scheiflinger F, Lammle B. Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor: in vitro inhibition of ADAMTS13 activity by hemoglobin. Blood 2005; 105: 542-4.
21. NCCLS. Protocols for determination of limits of detection and limits of quantitation; approved guideline. NCCLS document EP17-A. 940 West Valley Road, Suite 1400, Wayne, PA, USA: NCCLS, 2004; 1-39.
22. Raife TJ, Cao W, Atkinson BS, Bedell B, Montgomery RR, Lentz SR, Johnson GF, Zheng XL. Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site. Blood 2009; 114: 1666-74.
23. Kremer Hovinga JA, Mottini M, Lammle B. Measurement of ADAMTS-13 activity in plasma by the FRETS-VWF73 assay: comparison with other assay methods. J Thromb Haemost 2006; 4: 1146-8.
24. Lotta LA, Wu HM, Mackie IJ, Noris M, Veyradier A, Scully MA, Remuzzi G, Coppo P, Liesner R, Donadelli R, Loirat C, Gibbs RA, Horne A, Yang S, Garagiola I, Musallam KM, Peyvandi F. Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura. Blood 2012; 120: 440-8.
25. Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood 2004; 103: 4043-9.
26. Coppo P, Wolf M, Veyradier A, Bussel A, Malot S, Millot GA, Daubin C, Bordessoule D, Pene F, Mira JP, Heshmati F, Maury E, Guidet B, Boulanger E, Galicier L, Parquet N, Vernant JP, Rondeau E, Azoulay E, Schlemmer B; Microangiopathies RdE. Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura. Br J Haematol 2006; 132: 66-74.
27. Peyvandi F, Lavoretano S, Palla R, Feys HB, Vanhoorelbeke K, Battaglioli T, Valsecchi C, Canciani MT, Fabris F, Zver S, Réti M, Mikovic D, Karimi M, Giuffrida G, Laurenti L, Mannucci PM. ADAMTS13 and anti-ADAMTS13 antibodies are markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica 2008; 93: 69-76.
28. Bettoni G, Palla R, Valsecchi C, Consonni D, Lotta LA, Trisolini SM, Mancini I, Musallam KM, Rosendaal FR, Peyvandi F. ADAMTS-13 activity and autoantibodies classes and subclasses as prognostic predictors in acquired thrombotic thrombocytopenic purpura. J Thromb Haemost 2012; 10: 1556-65.
29. Yang S, Jin M, Lin S, Cataland S, Wu H. ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome. Haematologica 2011; 96: 1521-7.
30. Ferrari S, Scheiflinger F, Rieger M, Mudde G, Wolf M, Coppo P, Girma JP, Azoulay E, Brun-Buisson C, Fakhouri F, Mira JP, Oksenhendler E, Poullin P, Rondeau E, Schleinitz N, Schlemmer B, Teboul JL, Vanhille P, Vernant JP, Meyer D, et al. Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity. Blood 2007; 109: 2815-22.
31. Tsai HM, Li A, Rock G. Inhibitors of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura. Clin Lab 2001; 47: 387-92.
32. Scully M, McDonald V, Cavenagh J, Hunt BJ, Longair I, Cohen H, Machin SJ. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood 2011; 118: 1746-53.
33. Froissart A, Buffet M, Veyradier A, Poullin P, Provot F, Malot S, Schwarzinger M, Galicier L, Vanhille P, Vernant J, Bordessoule D, Guidet B, Azoulay E, Rondeau E, Mira J, Wynckel A, Clabault K, Choukroun G, Presne C, Pourrat J, et al. Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French thrombotic microangiopathies reference center. Crit Care Med 2011; 40: 104-11.
34. Knovich MA, Farland A, Owen J. Long-term management of acquired thrombotic thrombocytopenic purpura using serial plasma ADAMTS13 measurements. Eur J Haematol 2012; 88: 518-25.
35. Westwood JP, Webster H, McGuckin S, McDonald V, Machin SJ, Scully M. Rituximab for thrombotic thrombocytopenic purpura: benefit of early administration during acute episodes and use of prophylaxis to prevent relapse. J Thromb Haemost 2013; 11: 481-90.