Laboratory testing for ADAMTS13: Utility for TTP diagnosis/exclusion and beyond.
ADAMTS13 Protein
/ blood
Autoantibodies
/ blood
COVID-19
/ blood
Enzyme-Linked Immunosorbent Assay
Female
Fluorescence Resonance Energy Transfer
Humans
Luminescent Measurements
Male
Multicenter Studies as Topic
Pre-Eclampsia
/ diagnosis
Predictive Value of Tests
Pregnancy
Purpura, Thrombotic Thrombocytopenic
/ blood
Recombinant Proteins
/ metabolism
SARS-CoV-2
Sensitivity and Specificity
von Willebrand Diseases
/ diagnosis
von Willebrand Factor
/ metabolism
Journal
American journal of hematology
ISSN: 1096-8652
Titre abrégé: Am J Hematol
Pays: United States
ID NLM: 7610369
Informations de publication
Date de publication:
01 08 2021
01 08 2021
Historique:
revised:
10
05
2021
received:
09
04
2021
accepted:
12
05
2021
pubmed:
16
5
2021
medline:
16
7
2021
entrez:
15
5
2021
Statut:
ppublish
Résumé
The metalloproteinase ADAMTS13 (a disintegrin with a thrombospondin type 1 motif, member 13), also known as VWF (von Willebrand factor) protease, may be assessed in a vast array of clinical conditions. Notably, a severe deficiency of ADAMTS13 characterizes TTP (thrombotic thrombocytopenic purpura), a rare but potentially fatal disorder associated with thrombosis due to accumulation of prothrombotic ultra-large VWF multimers. Although prompt identification/exclusion of TTP can be facilitated by rapid ADAMTS13 testing, the most commonly utilized assays are based on ELISA (enzyme linked immunosorbent assay) and require long turnaround time and have relatively limited throughput. Nevertheless, several rapid ADAMTS13 assays are now available, at least in select geographies. The current mini-review discusses these issues, as well as the potential utility of ADAMTS13 testing in a range of other conditions, including coronavirus disease 2019 (COVID-19).
Substances chimiques
Autoantibodies
0
Recombinant Proteins
0
von Willebrand Factor
0
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
1049-1055Informations de copyright
© 2021 Wiley Periodicals LLC.
Références
Lautz WE, Raval JS, Dyer MR, Rollins-Raval MA, Zuckerbraun BS, Neal MD. ADAMTS13: origins, applications, and prospects. Transfusion. 2018;58(10):2453-2462.
Blennerhassett R, Curnow J. Pasalic L immune-mediated thrombotic thrombocytopenic purpura: a narrative review of diagnosis and treatment in adults. Semin Thromb Hemost. 2020;46(3):289-301.
Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenia purpura. Blood. 1997;89(9):3097-3103.
Singer K, Bornstein F, Wiles A. Thrombotic thrombocytopenia purpura. Blood. 1947;2:542-544.
Moschkowitz E. Hyaline thrombosis of the terminal tarterioles and capillaires: a hitherto undescribed disease. Proc NY Pathol Soc. 1924;24:21-24.
Moake JL, Rudy CK, Troll JH, et al. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombycytopenic purpura. N Engl J Med. 1982;307(23):1432-1435.
Furlan M, Robels 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(10):4223-4234.
Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996;87(10):4235-4244.
Upshaw JD. Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N Engl J Med. 1978;298(24):1350-1352.
Jamme M, Rondeau E. The PLASMIC score for thrombotic thrombocytopenic purpura. Lancet Haematol. 2017;4(4):e148-e149.
Benhamou Y, Assié C, Boelle PY, et al. Thrombotic Microangiopathies reference center. Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA reference center experience. Haematologica. 2012;97(8):1181-1186.
Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2486-2495.
Favaloro EJ, Mohammed S, Chapman K, et al. A multicentre laboratory assessment of a new automated chemiluminescent assay for ADAMTS13 activity. J Thromb Haemost. 2021;19(2):417-428.
Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian apheresis study group. N Engl J Med. 1991;325:393-397.
Scully M, Knöbl P, Kentouche K, et al. Recombinant ADAMTS-13: first-in-human pharmacokinetics and safety in congenital thrombotic thrombocytopenic purpura. Blood. 2017;130(19):2055-2063.
Chapman K, Seldon M, Richards R. Thrombotic microangiopathies, thrombotic thrombocytopenic purpura, and ADAMTS-13. Semin Thromb Hemost. 2012;38(1):47-54.
Favaloro EJ, Henry BM, Lippi G. Increased VWF and decreased ADAMTS13 in COVID-19: creating a milieu for (micro)thrombosis. Semin Thromb Hemost. 2021;47(4):400-418. https://doi.org/10.1055/s-0041-1727282.
Moore GW, Meijer D, Griffiths M, et al. A multi-center evaluation of TECHNOSCREEN® ADAMTS-13 activity assay as a screening tool for detecting deficiency of ADAMTS-13. J Thromb Haemost. 2020;18(7):1686-1694.
Lippi G, Sanchis-Gomar F, Favaloro EJ, Lavie CJ, Henry BM. Coronavirus disease 2019-associated coagulopathy. Mayo Clin Proc. 2021;96(1):203-217.
Thachil J, Srivastava A. SARS-2 coronavirus-associated hemostatic lung abnormality in COVID-19: is it pulmonary thrombosis or pulmonary embolism? Semin Thromb Hemost. 2020;46(7):777-780.
Schulman S. Coronavirus disease 2019, Prothrombotic factors, and venous thromboembolism. Semin Thromb Hemost. 2020;46(7):772-776.
Di Minno A, Ambrosino P, Calcaterra I, Di Minno MND. COVID-19 and venous thromboembolism: a meta-analysis of literature studies. Semin Thromb Hemost. 2020;46(7):763-771.
Jenner WJ, Kanji R, Mirsadraee S, et al. Thrombotic complications in 2928 patients with COVID-19 treated in intensive care: a systematic review. J Thromb Thrombolysis. 2021;14:1-13.
Uaprasert N, Moonla C, Sosothikul D, Rojnuckarin P, Chiasakul T. Systemic coagulopathy in hospitalized patients with coronavirus disease 2019: a systematic review and meta-analysis. Clin Appl Thromb Hemost. 2021;27. https://doi.org/10.1177/1076029620987629.
Carsana L, Sonzogni A, Nasr A, et al. Pulmonary post-mortem findings in a series of COVID-19 cases from northern Italy: a two-Centre descriptive study. Lancet Infect Dis. 2020;20(10):1135-1140.
Wichmann D, Sperhake J, Lutgehetmann M, Steurer S, Edler C. Autopsy findings and venous thromboembolism in patients with COVID-19. Ann Clin Med. 2020;173(4):268-277.
Bradley BT, Maioli H, Johnston R, et al. Histopathology and ultrastructural findings of fatal COVID-19 infections in Washington state: a case series. Lancet. 2020;396(10247):320-332.
Varga Z, Flammer AJ, Steiger P, et al. Endothelial cell infection and endotheliitis in COVID-19. Lancet. 2020;395(10234):1417-1418.
Levi M, Thachil J. Coronavirus disease 2019 coagulopathy: disseminated intravascular coagulation and thrombotic Microangiopathy-either, neither, or both. Semin Thromb Hemost. 2020;46(7):781-784.
Kwaan HC. Coronavirus disease 2019: the role of the fibrinolytic system from transmission to organ injury and sequelae. Semin Thromb Hemost. 2020;46(7):841-844.
Larsen JB, Pasalic L, Hvas AM. Platelets in coronavirus disease 2019. Semin Thromb Hemost. 2020;46(7):823-825.
Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre doctors organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014;167(4):453-465.
O'Donnell J, Laffan MA. The relationship between ABO histo-blood group, factor VIII and von Willebrand factor. Transfus Med. 2001;11(4):343-351.
Ward S, O'Sullivan JM, O'Donnell JS. The biological significance of von willebrand factor O-linked glycosylation. Semin Thromb Hemost. 2021. https://doi.org/10.1055/s-0041-1726373. [Epub ahead of print].
Stepanian A, Cohen-Moatti M, Sanglier T, et al. Von Willebrand factor and ADAMTS13: a candidate couple for preeclampsia pathophysiology. Arterioscler Thromb Vasc Biol. 2011;31(7):1703-1709.
Khanal N, Dahal S, Upadhyay S, Bhatt VR, Bierman PJ. Differentiating malignant hypertension-induced thrombotic microangiopathy from thrombotic thrombocytopenic purpura. Ther Adv Hematol. 2015;6(3):97-102.
van den Born BJ, van der Hoeven NV, Groot E, et al. Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension. Hypertension. 2008;51(4):862-866.
Schwameis M, Schorgenhofer C, Assinger A, Steiner MM, Jilma B. VWF excess and ADAMTS-13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP. Thromb Haemost. 2015;113:708-718.
Habe K, Wada H, Ito-Habe N, et al. Plasma ADAMTS-13, von Willebrand factor (VWF) and VWF propeptide profiles in patients with DIC and related diseases. Thromb Res. 2012;129:598-602.
Kremer Hovinga JA, Zeerleder S, Kessler P, et al. ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock. J Thromb Haemost. 2007;5(11):2284-2290.
Taylor A, Vendramin C, Singh D, Brown MM, Scully M. Von willebrand factor/adamts13 ratio at presentation of acute ischemic brain injury is predictive of outcome. Blood Adv. 2020;4(2):398-407.
Uemura M, Fujimura Y, Matsumoto M, et al. Comprehensive analysis of ADAMTS13 in patients with liver cirrhosis. Thromb Haemost. 2008;99(6):1019-1029.
Kobayashi S, Yokoyama Y, Matsushita T, et al. Increased von Willebrand factor to ADAMTS13 ratio as a predictor of thrombotic complications following a major hepatectomy. Arch Surg. 2012;147(10):909-917.
Matsukawa M, Kaikita K, Soejima K, et al. Serial changes in von Willebrand factor-cleaving protease (Adamts13) and prognosis after acute myocardial infarction. Am J Cardiol. 2007;100(5):758-763.
Oh J, Oh D, Lee SJ, et al. Korean TTP registry investigators. Prognostic utility of ADAMTS13 activity for the atypical hemolytic uremic syndrome (aHUS) and comparison of complement serology between aHUS and thrombotic thrombocytopenic purpura. Blood Res. 2019;54(3):218-228.
Mackie I, Mancini I, Muia J, et al. International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13. Int J Lab Hematol. 2020;42(6):685-696.