Abstract
Acquired autoantibodies targeting coagulation factors are fortunately rare. However, unfamiliarity with them can lead to misinterpretation of clinical presentations and screening laboratory tests, erroneous or delayed diagnoses, and inappropriate management decisions which may result in severe morbidity or mortality. Using acquired neutralizing antibodies to factor VIII (acquired hemophilia A) as a model for clinical presentation, laboratory diagnosis, management of bleeding, and immunosuppression treatment, this chapter reviews our current knowledge of acquired inhibitors of the coagulation system.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Abbreviations
- aPTT:
-
Activated partial thromboplastin time
- FEIBA:
-
Factor eight inhibitor bypass activity
- INR:
-
International normalized ratio
- LA:
-
Lupus anticoagulant
- NPP:
-
Normal pooled plasma
- PT:
-
Prothrombin time
- rFVIIa:
-
Recombinant factor VIIa
- TT:
-
Thrombin time
References
Adcock DM, Favaloro EJ. Pearls and pitfalls in factor inhibitor assays. Int J Lab Hematol. 2015;37(Suppl 1):52–60.
Clyne LP, White PF. Time dependency of lupuslike anticoagulants. Arch Intern Med. 1988;148(5):1060–3.
Collins PW, et al. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood. 2007;109(5):1870–7.
Knoebl P, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012;10(4):622–31.
Tiede A, et al. Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study. Blood. 2015;125(7):1091–7.
Borg JY, et al. Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l’Hemophilie Acquise) registry. Haemophilia. 2013;19(4):564–70.
Franchini M, et al. Drug-induced anti-factor VIII antibodies: a systematic review. Med Sci Monit. 2007;13(4):RA55–61.
Tengborn L, et al. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. BJOG. 2012;119(12):1529–37.
Ames PR, et al. Prolonged activated partial thromboplastin time: difficulties in discriminating coexistent factor VIII inhibitor and lupus anticoagulant. Clin Appl Thromb Hemost. 2015;21(2):149–54.
Baudo F, et al. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood. 2012;120(1):39–46.
Morrison AE, Ludlam CA, Kessler C. Use of porcine factor VIII in the treatment of patients with acquired hemophilia. Blood. 1993;81(6):1513–20.
Kruse-Jarres R, et al. Efficacy and safety of OBI-1, an antihaemophilic factor VIII (recombinant), porcine sequence, in subjects with acquired haemophilia A. Haemophilia. 2015;21(2):162–70.
Jansen M, et al. Treatment of coagulation inhibitors with extracorporeal immunoadsorption (Ig-Therasorb). Br J Haematol. 2001;112(1):91–7.
Losos M, et al. The tipping point: the critical role of therapeutic apheresis in a case of refractory acquired hemophilia. J Clin Apher. 2017;32(6):564–6.
Mahlangu J, et al. Emicizumab prophylaxis in patients who have hemophilia A without inhibitors. N Engl J Med. 2018;379(9):811–22.
Collins P, et al. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Blood. 2012;120(1):47–55.
Collins PW. Therapeutic challenges in acquired factor VIII deficiency. Hematology Am Soc of Hematol Educ Program. 2012;2012:369–74.
Eisert S, et al. Successful use of mycophenolate mofetil and prednisone in a 14-year-old girl with acquired hemophilia A. Thromb Haemost. 2005;93(4):792–3.
Franchini M, et al. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management. Blood Transfusion Trasfusione del sangue. 2015. p. 1–16.
Hay CR, editor. Hemostasis and thrombosis basic principles and clinical practice. In: Acquired disorders of coagulation: the immune coagulopathies. 6th ed. Philadelphia: Wolters Kluwer/Lippincott Wiliams & Wilkins: 2013. p. 723–37.
Franchini M, Lippi G, Favaloro EJ. Acquired inhibitors of coagulation factors: part II. Semin Thromb Hemost. 2012;38(5):447–53.
Krishnamurthy P, et al. A rare case of an acquired inhibitor to factor IX. Haemophilia. 2011;17(4):712–3.
Bortoli R, et al. Acquired factor XI inhibitor in systemic lupus erythematosus--case report and literature review. Semin Arthritis Rheum. 2009;39(1):61–5.
Girolami A, et al. Acquired isolated FVII deficiency: an underestimated and potentially important laboratory finding. Clin Appl Thromb Hemost. 2016;22(8):705–11.
Mulliez SM, Devreese KM. Isolated acquired factor VII deficiency: review of the literature. Acta Clin Belg. 2015.
Kamikubo Y, et al. Purification and characterization of factor VII inhibitor found in a patient with life threatening bleeding. Thromb Haemost. 2000;83(1):60–4.
Delmer A, et al. Life-threatening intracranial bleeding associated with the presence of an antifactor VII autoantibody. Blood. 1989;74(1):229–32.
Lippi G, et al. Inherited and acquired factor V deficiency. Blood Coagul Fibrinolysis. 2011;22(3):160–6.
Franchini M, Lippi G. Acquired factor V inhibitors: a systematic review. J Thromb Thrombolysis. 2011;31(4):449–57.
Siekanska-Cholewa A, et al. Acquired factor V inhibitor in a woman following aortic aneurysm surgery. Blood Coagul Fibrinolysis. 2014;25(5):515–7.
Mumford AD, et al. Bleeding symptoms and coagulation abnormalities in 337 patients with AL-amyloidosis. Br J Haematol. 2000;110(2):454–60.
Veneri D, et al. Use of prothrombin complex concentrate for prophylaxis of bleeding in acquired factor X deficiency associated with light-chain amyloidosis. Blood Transfus. 2016;14(6):585–6.
Mahmood S, et al. Utility of factor X concentrate for the treatment of acquired factor X deficiency in systemic light-chain amyloidosis. Blood. 2014;123(18):2899–900.
Broze GJ Jr. An acquired, calcium-dependent, factor X inhibitor. Blood Cells Mol Dis. 2014;52(2–3):116–20.
Lee G, Duan-Porter W, Metjian AD. Acquired, non-amyloid related factor X deficiency: review of the literature. Haemophilia. 2012;18(5):655–63.
Karapetian H. Reptilase time (RT). Methods Mol Biol. 2013;992:273–7.
Kohler HP, et al. Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost. 2011;9(7):1404–6.
Yan MTS, et al. Acquired factor XIII deficiency: a review. Transfus Apher Sci. 2018;57(6):724–30.
Franchini M, et al. Acquired FXIII inhibitors: a systematic review. J Thromb Thrombolysis. 2013;36(1):109–14.
Godaert L, et al. Acquired hemophilia A in aged people: a systematic review of case reports and case series. Semin Hematol. 2018;55(4):197–201.
Kottke-Marchant K, editor. An algorithmic approach to hemostasis testing. Northfield, IL: College of American Pathologists; 2008.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Eby, C. (2021). Bleeding Associated with Coagulation Factor Inhibitors. In: Teruya, J. (eds) Management of Bleeding Patients. Springer, Cham. https://doi.org/10.1007/978-3-030-56338-7_10
Download citation
DOI: https://doi.org/10.1007/978-3-030-56338-7_10
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-56337-0
Online ISBN: 978-3-030-56338-7
eBook Packages: MedicineMedicine (R0)