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Assessment of Factor XIII

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Hemostasis and Thrombosis

Part of the book series: Methods in Molecular Biology ((MIMB,volume 1646))

Abstract

Blood coagulation factor XIII (FXIII) is essential for maintaining hemostasis. The absence of FXIII results in severe bleeding diathesis, which without prophylaxis frequently leads to fatal bleeding. As the usual hemostasis screening tests remain normal, the diagnosis of FXIII deficiency needs specific tests. Here, we describe FXIII activity determination by the ammonia release assay, which is the first-line test in the diagnostic algorithm for FXIII deficiency. The method for another activity test, the undeservedly rarely used fibrin cross-linking assay, is also presented. Further tests used for the classification of FXIII deficiencies, measurement of FXIII activity in platelets, ELISAs for the measurement of complex plasma FXIII (FXIII-A2B2) antigen and for FXIII-A2 in plasma and platelets are also included. Detailed description of the methods for the detection and measurement of neutralizing auto- and alloantibodies is also provided.

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References

  1. Muszbek L, Bereczky Z, Bagoly Z, Komaromi I, Katona E (2011) Factor XIII: a coagulation factor with multiple plasmatic and cellular functions. Physiol Rev 91:931–972

    Article  CAS  PubMed  Google Scholar 

  2. Komaromi I, Bagoly Z, Muszbek L (2011) Factor XIII: novel structural and functional aspects. J Thromb Haemost 9:9–20

    Article  CAS  PubMed  Google Scholar 

  3. Schroeder V, Kohler HP (2016) Factor XIII: structure and function. Semin Thromb Hemost 42:422–428

    Article  CAS  PubMed  Google Scholar 

  4. Muszbek L, Bagoly Z, Cairo A, Peyvandi F (2011) Novel aspects of factor XIII deficiency. Curr Opin Hematol 18:366–372

    Article  CAS  PubMed  Google Scholar 

  5. Muszbek L, Katona E (2016) Diagnosis and management of congenital and acquired FXIII deficiencies. Semin Thromb Hemost 42:429–439

    Article  PubMed  Google Scholar 

  6. Schroeder V, Kohler HP (2013) Factor XIII deficiency: an update. Semin Thromb Hemost 39:632–641

    Article  CAS  PubMed  Google Scholar 

  7. Dorgalaleh A, Naderi M, Hosseini MS, Alizadeh S, Hosseini S, Tabibian S, Eshghi P (2015) Factor XIII deficiency in Iran: a comprehensive review of the literature. Semin Thromb Hemost 41:323–329

    Article  CAS  PubMed  Google Scholar 

  8. Eshghi P, Cohan N, Lak M, Naderi M, Peyvandi F, Menegatti M, Karimi M (2012) Arg77His and Trp187Arg are the most common mutations causing FXIII deficiency in Iran. Clin Appl Thromb Hemost 18:100–103

    Article  CAS  PubMed  Google Scholar 

  9. Duncan EM, Dale BJ, Lloyd JV (2014) Investigation of a link between raised levels of pepsinogen in blood as a mediator of in-vitro clot lysis in acid and a cause of abnormal factor XIII screening tests. Blood Coagul Fibrinolysis 25:340–348

    Article  CAS  PubMed  Google Scholar 

  10. Köhler HP, Ichinose A, Seitz R, Ariens RA, Muszbek L (2011) Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost 9:1404–1406

    Article  PubMed  Google Scholar 

  11. Muszbek L, Polgar J, Fesus L (1985) Kinetic determination of blood coagulation factor XIII in plasma. Clin Chem 31:35–40

    CAS  PubMed  Google Scholar 

  12. Karpati L, Penke B, Katona E, Balogh I, Vamosi G, Muszbek L (2000) A modified, optimized kinetic photometric assay for the determination of blood coagulation factor XIII activity in plasma. Clin Chem 46:1946–1955

    CAS  PubMed  Google Scholar 

  13. Fickenscher K, Aab A, Stuber W (1991) A photometric assay for blood coagulation factor XIII. Thromb Haemost 65:535–540

    CAS  PubMed  Google Scholar 

  14. Ajzner E, Schlammadinger A, Kerenyi A, Bereczky Z, Katona E, Haramura G, Boda Z, Muszbek L (2009) Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency. Blood 113:723–725

    Article  CAS  PubMed  Google Scholar 

  15. Penzes K, Vezina C, Bereczky Z, Katona E, Kun M, Muszbek L, Rivard GE (2016) Alloantibody developed in a factor XIII a subunit deficient patient during substitution therapy; characterization of the antibody. Haemophilia 22:268–275

    Article  CAS  Google Scholar 

  16. Ichinose A, Osaki T, Souri M, Japanese Collaborative Research Group on AH (2015) Clinical features of 32 new Japanese cases with autoimmune haemorrha-philia due to anti-factor XIII antibodies. Haemophilia 21:653–658

    Article  CAS  PubMed  Google Scholar 

  17. Ichinose A, Japanese Collaborative Research Group on AHHAC (2014) Inhibitors of factor XIII/13 in older patients. Semin Thromb Hemost 40:704–711

    Article  CAS  PubMed  Google Scholar 

  18. Katona E, Penzes K, Molnar E, Muszbek L (2012) Measurement of factor XIII activity in plasma. Clin Chem Lab Med 50:1191–1202

    Article  CAS  PubMed  Google Scholar 

  19. Peyvandi F, Tagliabue L, Menegatti M, Karimi M, Komaromi I, Katona E, Muszbek L, Mannucci PM (2004) Phenotype-genotype characterization of 10 families with severe a subunit factor XIII deficiency. Hum Mutat 23:98

    Article  PubMed  Google Scholar 

  20. Katona E, Haramura G, Karpati L, Fachet J, Muszbek L (2000) A simple, quick one-step ELISA assay for the determination of complex plasma factor XIII (A2B2). Thromb Haemost 83:268–273

    CAS  PubMed  Google Scholar 

  21. Verbruggen B, Novakova I, Wessels H, Boezeman J, van den Berg M, Mauser-Bunschoten E (1995) The Nijmegen modification of the Bethesda assay for factor VIII:C inhibitors: improved specificity and reliability. Thromb Haemost 73:247–251

    CAS  PubMed  Google Scholar 

  22. Cini M, Legnani C, Frascaro M, Pancani C, Cappelli C, Rodorigo G, Valdre L, Cosmi B (2016) Measurement of factor XIII (FXIII) activity by an automatic ammonia release assay using iodoacetamide blank-procedure: no more overestimation in the low activity range and better detection of severe FXIII deficiencies. Clin Chem Lab Med 54:805–809

    Article  CAS  PubMed  Google Scholar 

  23. Ajzner E, Muszbek L (2004) Kinetic spectrophotometric factor XIII activity assays: the subtraction of plasma blank is not omissible [corrected]. J Thromb Haemost 2:2075–2077

    Article  CAS  PubMed  Google Scholar 

  24. Lawrie AS, Green L, Mackie IJ, Liesner R, Machin SJ, Peyvandi F (2010) Factor XIII–an under diagnosed deficiency–are we using the right assays? J Thromb Haemost 8:2478–2482

    Article  CAS  PubMed  Google Scholar 

  25. Katona E, Ajzner E, Toth K, Karpati L, Muszbek L (2001) Enzyme-linked immunosorbent assay for the determination of blood coagulation factor XIII A-subunit in plasma and in cell lysates. J Immunol Methods 258:127–135

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Division of Clinical Laboratory Science, Faculty of Medicine, University of Debrecen, Nagyerdei krt. 98, Debrecen, Hungary

    László Muszbek & Éva Katona

  2. Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary

    Adrienne Kerényi

Authors
  1. László Muszbek
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  2. Éva Katona
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  3. Adrienne Kerényi
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Corresponding author

Correspondence to László Muszbek .

Editor information

Editors and Affiliations

  1. Department of Haematology, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, New South Wales, Australia

    Emmanuel J. Favaloro

  2. Section of Clinical Biochemistry, University of Verona, Verona, Italy

    Giuseppe Lippi

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Muszbek, L., Katona, É., Kerényi, A. (2017). Assessment of Factor XIII. In: Favaloro, E., Lippi, G. (eds) Hemostasis and Thrombosis. Methods in Molecular Biology, vol 1646. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7196-1_22

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  • DOI: https://doi.org/10.1007/978-1-4939-7196-1_22

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  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4939-7194-7

  • Online ISBN: 978-1-4939-7196-1

  • eBook Packages: Springer Protocols

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