International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13.
ADAMTS13
VWF
haemostasis
thrombosis
Journal
International journal of laboratory hematology
ISSN: 1751-553X
Titre abrégé: Int J Lab Hematol
Pays: England
ID NLM: 101300213
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
07
04
2020
revised:
15
06
2020
accepted:
02
07
2020
pubmed:
17
7
2020
medline:
4
2
2021
entrez:
17
7
2020
Statut:
ppublish
Résumé
This guidance document was prepared on behalf of the International Council for Standardization in Haematology (ICSH), by the ADAMTS13 Assay Working Group, which comprises an international group of both clinical and laboratory experts. The document provides recommendations on best practice for the performance of ADAMTS13 assays in clinical laboratories. ADAMTS13 assays support the differential diagnosis of thrombotic microangiopathies and have utility in the management of thrombotic thrombocytopenic purpura (TTP). There are three types of assay: activity, antigen and autoantibody/inhibitor assays. Methods for activity assays differ in terms of sensitivity, specificity, precision and turnaround time. The most widely used assays involve VWF peptide substrates and either chromogenic ELISA or FRET techniques, although chemiluminescence assays and rapid screening tests have recently become available. Tests for autoantibodies and inhibitors allow confirmation of acquired, immune-mediated TTP, while antigen assays may be useful in congenital TTP and as prognostic markers. In this document, we have attempted to describe ADAMTS13 assays and the conditions that affect them, as well as: blood collection, sample processing, quality control, standardization and clinical utility; recognizing that laboratories in different parts of the world have varying levels of sophistication. The recommendations are based on expert opinion, published literature and good clinical laboratory practice.
Substances chimiques
ADAMTS13 Protein
EC 3.4.24.87
ADAMTS13 protein, human
EC 3.4.24.87
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
685-696Informations de copyright
© 2020 The Authors. International Journal of Laboratory Hematology published by John Wiley & Sons Ltd.
Références
Sadler JE. Von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. Blood. 2008;112:11-18.
Chen J, Chung DW. Inflammation, von Willebrand factor, and ADAMTS13. Blood. 2018;132:141-147.
Muia J, Gao W, Haberichter SL, et al. An optimized fluorogenic ADAMTS13 assay with increased sensitivity for the investigation of patients with thrombotic thrombocytopenic purpura. J Thromb Haemost. 2013;11:1511-1518.
Muia J, Zhu J, Gupta G, et al. Allosteric activation of ADAMTS13 by von Willebrand factor. Proc Natl Acad Sci U S A. 2014;111(52):18584-18589.
Muia J, Zhu J, Greco SC, et al. Phylogenetic and functional analysis of ADAMTS13 identifies highly conserved domains essential for allosteric regulation. Blood. 2019;133:1899-1908.
South K, Luken BM, Crawley JT, et al. Conformational activation of ADAMTS13. Proc Natl Acad Sci U S A. 2014;111:18578-18583.
Petri A, Kim HJ, Xu Y, et al. Crystal structure and substrate-induced activation of ADAMTS13. Nat Commun. 2019;10:3781.
Joly B, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura. Blood. 2017;129:2836-2846.
Kremer Hovinga JA, Heeb SR, Skowronska M, Schaller M. Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. J Thromb Haemost. 2018;16:618-629.
Hassan S, Westwood JP, Ellis D, et al. The utility of ADAMTS13 in differentiating TTP from other acute thrombotic microangiopathies: results from the UK TTP Registry. Br J Haematol. 2015;171:830-835.
Bendapudi PK, Li A, Hamdan A, Uhl L, et al. Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative. Br J Haematol. 2015;5:836-844.
Masias C, Cataland SR. The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis. Blood. 2018;132:903-910.
Scully M, Hunt BJ, Benjamin S, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Brit J Haematol. 2012;158(3):323-335.
Scully M, Cataland S, Coppo P, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017;15:312-322.
Gosselin RC, Adcock D, Dorgalaleh A, et al. International Council for Standardization in Haematology recommendations for hemostasis critical values, tests, and reporting. Semin Thromb Hemost. 2019;46(04):398-409.
Matsumoto M, Fujimura Y, Wada H, et al. Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. Int J Hematol. 2017;106:3-15.
International Society for Thrombosis and Haemostasis. Recommendations for the diagnosis and treatment of thrombotic thrombocytopenic purpura. In final preparation 2020.
Zini G, D'Onofrio G, Briggs C, et al. ICSH recommendations for identification, diagnostic value, and quantitation of schistocytes. Int Jnl Lab Hem. 2012;34:107-116.
Goodship THJ, Cook HT, Fakhouri F, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. Kidney Int. 2017;91:539-551.
Lippi G, Salvagno GL, Adcock DM, Gelati M, Guidi GC, Favaloro EJ. Right or wrong sample received for coagulation testing? Tentative algorithms for detection of an incorrect type of sample. Int J Lab Hematol. 2010;32:132-138.
Langley K, Fretwell R, Kitchen S, et al. Multiple centre evaluation study of ADAMTS13 activity and inhibitor assays. Int J Lab Hematol. 2018;40:21-25.
Rottensteiner H, Kaufmann S, Rathgeb A, et al. Temperature-dependent irreversible conformational change of recombinant ADAMTS13 upon metal ion chelation. J Thromb Haemost. 2019;17:995-1002.
Mancini I, Valsecchi C, Lotta LA, et al. FRETS-VWF73 rather than CBA assay reflects ADAMTS13 proteolytic activity in acquired thrombotic thrombocytopenic purpura patients. Thromb Haemost. 2012;112:297-303.
Peyvandi F, Palla R, Lotta LA, Mackie IJ, Scully MA, Machin SJ. ADAMTS-13 assays in thrombotic thrombocytopenic purpura. J Thromb Haemost. 2010;8:631-640.
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;87:4223-4234.
Tsai HM, Lian EC. Antibodies to von Willebrand factor cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339:1585-1594.
Obert B, Tout H, Veyradier A, Fressinaud E, Meyer D, Girma JP. Estimation of the von Willebrand factor-cleaving protease in plasma using monoclonal antibodies to vWF. Thromb Haemost. 1999;82:1382-1385.
Bohm M, Vigh T, Scharrer I. Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13). Ann Hematol. 2002;81:430-435.
Gerritsen HE, Turecek PL, Schwarz HP, Lämmle 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;82:1386-1389.
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.
Zhang L, Lawson HL, Harish VC, Huff JD, Knovich MA, Owen J. Creation of a recombinant peptide substrate for fluorescence resonance energy transfer-based protease assays. Anal Biochem. 2006;358:298-300.
Kato S, Matsumoto M, Matsuyama T, Isonishi A, Hiura H, Fujimura Y. Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity. Transfusion. 2006;46:1444-1452.
Jin M, Casper TC, Cataland SR, et al. Relationship between ADAMTS13 activity in clinical remission and the risk of relapse. Br J Haematol. 2008;141:651-658.
Favresse J, Lardinois B, Chatelain B, Jacqmin H, Mullier F. Evaluation of the fully automated HemosIL Acustar ADAMTS13 activity assay. Thromb Haemost. 2018;118:942-944.
Valsecchi C, Mirabet M, Mancini I, et al. Evaluation of a new, rapid, fully automated assay for the measurement of ADAMTS13 activity. Thromb Haemost. 2019;119:1767-1772.
Stratmann J, Ward J-N, Miesbach W. Evaluation of a rapid turn-over, fully-automated ADAMTS13 activity assay: a method comparison study. J Thromb Thrombolysis. 2020. https://doi.org/10.1007/s11239-020-02086-8
Mancini I, Valsecchi C, Palla R, Lotta LA, Peyvandi F. Measurement of anti-ADAMTS13 neutralizing autoantibodies: a comparison between CBA and FRET assays. J Thromb Haemost. 2014;10:1439-1442.
Moore GW, Meijer D, Griffiths M, et al. A multi-centre evaluation of TECHNOSCREEN ADAMTS13 Activity ELISA as a screening tool for detecting deficiency of ADAMTS-13. J Thromb Haemost. 2020;18:1686-1694.
Studt JD, Bohm M, Budde U, Girma J-P, Varadi K, Lammle B. Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods. J Thromb Haemost. 2003;1:1882-1887.
Tripodi A, Chantarangkul V, Bohm M, et al. Measurement of von Willebrand factor cleaving protease (ADAMTS-13): results of an international collaborative study involving 11 methods testing the same set of coded plasmas. J Thromb Haemost. 2004;2:1601-1609.
Groot E, Hulstein JJJ, Rison CN, De groot Ps, Fijnheer R. FRETS-VWF73: a rapid and predictive tool for thrombotic thrombocytopenic purpura. J Thromb Haemost. 2006;4:698-699.
Mahdian R, Rayes J, Girma J-P, et al. Comparison of FRETS-VWF73 to full length VWF as a substrate for ADAMTS13 activity measurement in human plasma samples. Thromb Haemost. 2006;95:1049-1051.
Shelat SG, Smith P, Ai J, Zheng XL. Inhibitory autoantibodies against ADAMTS-13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS-13 protease and may accelerate its clearance in vivo. J Thromb Haemost. 2006;4:1707-1717.
Starke R, Machin S, Scully M, Purdy G, Mackie I. The clinical utility of ADAMTS13 activity, antigen and autoantibody assays in thrombotic thrombocytopenic purpura. Brit J Haematol. 2007;136:649-655.
Tripodi A, Peyvandi F, Chantarangkul V, et al. Second international collaborative study evaluating performance characteristics of methods measuring the von Willebrand factor cleaving protease (ADAMTS-13). J Thromb Haemost. 2008;6:1534-1541.
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;105:381-385.
Jennings I, Mackie I, Kitchen S, et al. Variability in measurement of ADAMTS13: a UK NEQAS multicentre exercise for ADAMTS13 assays. Brit J Haematol. 2013;161(Suppl. 1):35.
Mackie I, Langley K, Chitolie A, et al. Discrepancies between ADAMTS13 activity assays in patients with thrombotic microangiopathies. Thromb Haemost. 2013;109:488-496.
Thouzeau S, Capdenat S, Stepanian A, Coppo P, Veyradier A. Evaluation of a commercial assay for ADAMTS13 activity measurement. Thromb Haemost. 2013;110:852-853.
Joly B, Stepanian A, Hajage D, et al. Evlauation of a chromogenic commercial assay using VWF-73 peptide for ADAMTS13 activity measurement. Thromb Res. 2014;134:1074-1080.
Nakashima MO, Zhang X, Rogers HJ, et al. Validation of a panel of ADAMTS13 assays for diagnosis of thrombotic thrombocytopenic purpura: activity, functional inhibitor, and autoantibody test. Int Jnl Lab Hem. 2016;38:550-559.
Kremer Hovinga JA, Mottini M, Laamle B. Measurement of ADAMTS-13 activity in plasma by the FRETS-VWF73 assay: comparison with other assay methods. J Thromb Haemost. 2006;4:1146-1148.
Hubbard AR, Heath AB, Kremer Hovinga JA. Establishment of the WHO 1st International Standard ADAMTS13, plasma (12/252): communication from the SSC of the ISTH. J Thromb Haemost. 2015;13(6):1151-1153.
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;5:866-867.
Lu RN, Yang S, Wu HM, Zheng XL. Unconjugated bilirubin inhibits proteolytic cleavage of von Willebrand factor by ADAMTS13 protease. J Thromb Haemost. 2015;13:1064-1072.
Eckmann CM, De Laaf RT, van Keulen JM, Van Mourik JA, De Laat B. Bilirubin oxidase as a solution for the interference of hyperbilirubinaemia with ADAMTS-13 activity measurement by FRETS-VWF73 assay. J Thromb Haemost. 2007;5:1330-1331.
Zhou Z, Han H, Cruz MA, López J, Dong JF, Guchhait P. Haemoglobin blocks von Willebrand factor proteolysis by ADAMTS-13: a mechanism associated with sickle cell disease. Thromb Haemost. 2009;101:1070-1077.
Studt JD, Kremer Hovinga JA, Antoine G, et al. Fatal congenital thrombotic thrombocytopenic pupura with apparent ADAMTS13 inhibitor: in vitro inhibition of ADAMTS13 activity by haemoglobin. Blood. 2005;105:542-544.
Scheiflinger F, Knöbl P, Trattner B, et al. Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura. Blood. 2003;102:3241-3243.
Peyvandi F, Lavoretano S, Palla R, et al. ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica. 2008;93:232-239.
Grillberger R, Gruber B, Skalicky S, et al. A novel flow-based assay reveals discrepancies in ADAMTS-13 inhibitor assessment as compared with a conventional clinical static assay. J Thromb Haemost. 2014;12:1523-1532.
CLSI. Quality System Regulation for Laboratory-Developed Tests: A Practical Guide for the Laboratory. CLSI Document QSRLDT. Wayne, PA: Clinical and Laboratory Standards Institute; 2015.
White GH, Farrance I. Uncertainty of measurement in quantitative medical testing - a laboratory implementation guide. Clin Biochem Rev. 2004;25:S1-S24.
Bendapudi PK, Hurwitz S, Fry A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol. 2017;4:e157-e164.
Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019;380:335-346.
Scully M, Cohen H, Cavenagh J, et al. Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13. Br J Haematol. 2007;136:451-461.
Page EE, Kremer Hovinga JA, Terrell DR, Vesely SK, George JN. Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2016;127:3092-3094.
Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368:2169-2181.
Wu N, Liu J, Yang S, et al. Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura. Transfusion. 2015;55:18-24.
Alwan F, Vendramin C, Liesner R, et al. Characterization and treatment of congenital thrombotic thrombocytopenic purpura. Blood. 2019;133:1644-1651.
van Dorland HA, Taleghani MM, Sakai K, et al. The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017. Haematologica. 2019;104:2107-2115.
Thomas MR, de Groot R, Scully MA, et al. Pathogenicity of anti-ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura. EBioMedicine. 2015;2:942-952.
Ferrari S, Scheiflinger F, Rieger M, 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;109:2815-2822.
Peyvandi F, Scully M, Kremer Hovinga JA, et al. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016;374:511-522.
Alwan F, Vendramin C, Vonhoorelbeke K, et al. Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura. Blood. 2017;130:466-471.
Yarranton H, Lawrie AS, Mackie IJ, et al. Coagulation factor levels in cryosupernatant prepared from plasma treated with amotosalen hydrochloride (S-59) and ultraviolet A light. Transfusion. 2005;45:1453-1458.
Froehlich-Zahnd R, George JN, Vesely SK, et al. Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura. Haematologica. 2012;97:297-303.
Lotta LA, Valsecchi C, Pontiggia S, et al. Measurement and prevalence of circulating ADAMTS13-specific immune complexes in autoimmune thrombotic thrombocytopenic purpura. J Thromb Haemost. 2014;12:329-336.