PDF herunterladen
Semin Thromb Hemost 2012; 38(05): 447-453
DOI: 10.1055/s-0032-1305779
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Acquired Inhibitors of Coagulation Factors: Part II

Massimo Franchini
1   Department of Transfusion Medicine and Hematology, “C. Poma” Hospital, Mantova, Italy
,
Giuseppe Lippi
2   U.O. di Diagnostica Ematochimica, Dipartimento di Patologia e Medicina di Laboratorio, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy
,
Emmanuel J. Favaloro
3   Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead, Australia
› Institutsangaben
Weitere Informationen

Publikationsverlauf

Publikationsdatum:
27. Juni 2012 (online)

    Lizenzen und Reprints

Abstract

Acquired coagulation inhibitors are antibodies that bind to coagulation factors and neutralize their activity or accelerate their clearance. Inhibitors occurring in patients with inherited deficiencies of coagulation factors are referred to as “alloantibodies,” while those developing spontaneously in individuals with previously normal coagulation factor function are designated as “autoantibodies.” The latter category includes inhibitors against coagulation factors I, II, V, VII, VIII (acquired hemophilia A), IX (acquired hemophilia B), X, XI, and XIII. This review will discuss the most important pathogenic, clinical, laboratory, and therapeutic aspects of the inhibitors of coagulation factors other than acquired hemophilia A, as this is reviewed separately within this issue of the journal.

Keywords

acquired hemophilia - inhibitors - autoantibodies - bleeding
 

  • References

  • 1 Watson HG, Chee YL, Greaves M. Rare acquired bleeding disorders. Rev Clin Exp Hematol 2001; 5 (4) 405-429 , quiz 431
    CrossrefPubMedSuche in Google Scholar
  • 2 Bouvry P, Recloux P. Acquired hemophilia. Haematologica 1994; 79 (6) 550-556
    PubMedSuche in Google Scholar
  • 3 Cohen AJ, Kessler CM. Acquired inhibitors. Baillieres Clin Haematol 1996; 9 (2) 331-354
    CrossrefPubMedSuche in Google Scholar
  • 4 Franchini M, Veneri D. Acquired coagulation inhibitor-associated bleeding disorders: an update. Hematology 2005; 10 (6) 443-449
    CrossrefPubMedSuche in Google Scholar
  • 5 Franchini M, Targher G, Montagnana M, Lippi G. Laboratory, clinical and therapeutic aspects of acquired hemophilia A. Clin Chim Acta 2008; 395 (1–2) 14-18
    CrossrefPubMedSuche in Google Scholar
  • 6 Franchini M, Lippi G. Acquired factor V inhibitors: a systematic review. J Thromb Thrombolysis 2011; 31 (4) 449-457
    CrossrefPubMedSuche in Google Scholar
  • 7 Lippi G, Favaloro EJ, Montagnana M, Manzato F, Guidi GC, Franchini M. Inherited and acquired factor V deficiency. Blood Coagul Fibrinolysis 2011; 22 (3) 160-166
    CrossrefPubMedSuche in Google Scholar
  • 8 Knöbl P, Lechner K. Acquired factor V inhibitors. Baillieres Clin Haematol 1998; 11 (2) 305-318
    CrossrefPubMedSuche in Google Scholar
  • 9 Favaloro EJ, Posen J, Ramakrishna R , et al. Factor V inhibitors: rare or not so uncommon? A multi-laboratory investigation. Blood Coagul Fibrinolysis 2004; 15 (8) 637-647
    CrossrefPubMedSuche in Google Scholar
  • 10 Miesbach W, Voigt J, Peetz D, Scharrer I. [Massive bleeding symptoms in two patients with factor V inhibitor and antiphospholipid antibodies after treatment with ciprofloxacin]. Med Klin (Munich) 2003; 98 (6) 339-343
    CrossrefPubMedSuche in Google Scholar
  • 11 Lucia JF, Aguilar C. A case of an asymptomatic idiopathic inhibitor to coagulation factor V. Haemophilia 2005; 11 (2) 178-180
    CrossrefPubMedSuche in Google Scholar
  • 12 Takahashi H, Fuse I, Abe T, Yoshino N, Aizawa Y. Acquired factor V inhibitor complicated by Hashimoto's thyroditis, primary biliary cirrhosis and membranous nephropathy. Blood Coagul Fibrinolysis 2003; 14 (1) 87-93
    CrossrefPubMedSuche in Google Scholar
  • 13 Emori Y, Sakugawa M, Niiya K , et al. Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis. Blood Coagul Fibrinolysis 2002; 13 (6) 555-559
    CrossrefPubMedSuche in Google Scholar
  • 14 Koyama T, Saito T, Kusano T, Hirosawa S. Factor V inhibitor associated with Sjögren's syndrome. Br J Haematol 1995; 89 (4) 893-896
    PubMedSuche in Google Scholar
  • 15 Streiff MB, Ness PM. Acquired FV inhibitors: a needless iatrogenic complication of bovine thrombin exposure. Transfusion 2002; 42 (1) 18-26
    CrossrefPubMedSuche in Google Scholar
  • 16 Bänninger H, Hardegger T, Tobler A , et al. Fibrin glue in surgery: frequent development of inhibitors of bovine thrombin and human factor V. Br J Haematol 1993; 85 (3) 528-532
    PubMedSuche in Google Scholar
  • 17 Ortel TL, Mercer MC, Thames EH, Moore KD, Lawson JH. Immunologic impact and clinical outcomes after surgical exposure to bovine thrombin. Ann Surg 2001; 233 (1) 88-96
    CrossrefPubMedSuche in Google Scholar
  • 18 Lu L, Liu Y, Wei J, Zhang L, Zhang L, Yang R. Acquired inhibitor of factor V: first report in China and literature review. Haemophilia 2004; 10 (5) 661-664
    CrossrefPubMedSuche in Google Scholar
  • 19 Leus B, Devreese K, van den Bossche J, Malfait R. Factor V inhibitor: case report. Blood Coagul Fibrinolysis 2006; 17 (7) 585-587
    CrossrefPubMedSuche in Google Scholar
  • 20 Caers J, Reekmans A, Jochmans K , et al. Factor V inhibitor after injection of human thrombin (tissucol) into a bleeding peptic ulcer. Endoscopy 2003; 35 (6) 542-544
    Thieme ConnectPubMedSuche in Google Scholar
  • 21 Bowman LJ, Anderson CD, Chapman WC. Topical recombinant human thrombin in surgical hemostasis. Semin Thromb Hemost 2010; 36 (5) 477-484
    Thieme ConnectPubMedSuche in Google Scholar
  • 22 Wiwanitkit V. Spectrum of bleeding in acquired factor V inhibitor: a summary of 33 cases. Clin Appl Thromb Hemost 2006; 12 (4) 485-488
    CrossrefPubMedSuche in Google Scholar
  • 23 Morris CJ, Curry N. Acquired factor V inhibitor in a critically ill patient. Anaesthesia 2009; 64 (9) 1014-1017
    CrossrefPubMedSuche in Google Scholar
  • 24 Ang AL, Kuperan P, Ng CH, Ng HJ. Acquired factor V inhibitor. A problem-based systematic review. Thromb Haemost 2009; 101 (5) 852-859
    PubMedSuche in Google Scholar
  • 25 Kershaw G, Favaloro EJ. Laboratory identification of factor inhibitors: an update. Pathology 2012; 44 (4) 293-302
    CrossrefPubMedSuche in Google Scholar
  • 26 Ortel TL. Clinical and laboratory manifestations of anti-factor V antibodies. J Lab Clin Med 1999; 133 (4) 326-334
    CrossrefPubMedSuche in Google Scholar
  • 27 Favaloro EJ, Bonar R, Duncan E , et al; RCPA QAP in Haematology Haemostasis Committee. Identification of factor inhibitors by diagnostic haemostasis laboratories: a large multi-centre evaluation. Thromb Haemost 2006; 96 (1) 73-78
    Thieme ConnectPubMedSuche in Google Scholar
  • 28 Favaloro EJ, Bonar R, Duncan E , et al. Mis-identification of factor inhibitors by diagnostic haemostasis laboratories: recognition of pitfalls and elucidation of strategies. A follow up to a large multicentre evaluation. Pathology 2007; 39 (5) 504-511
    CrossrefPubMedSuche in Google Scholar
  • 29 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 (1 Pt 2) 132-138
    CrossrefPubMedSuche in Google Scholar
  • 30 Fu YX, Kaufman R, Rudolph AE, Collum SE, Blinder MA. Multimodality therapy of an acquired factor V inhibitor. Am J Hematol 1996; 51 (4) 315-318
    CrossrefPubMedSuche in Google Scholar
  • 31 Franchini M, Lippi G. Recombinant activated factor VII: mechanisms of action and current indications. Semin Thromb Hemost 2010; 36 (5) 485-492
    Thieme ConnectPubMedSuche in Google Scholar
  • 32 Jansen M, Schmaldienst S, Banyai S , et al. Treatment of coagulation inhibitors with extracorporeal immunoadsorption (Ig-Therasorb). Br J Haematol 2001; 112 (1) 91-97
    CrossrefPubMedSuche in Google Scholar
  • 33 de Raucourt E, Barbier C, Sinda P, Dib M, Peltier JY, Ternisien C. High-dose intravenous immunoglobulin treatment in two patients with acquired factor V inhibitors. Am J Hematol 2003; 74 (3) 187-190
    CrossrefPubMedSuche in Google Scholar
  • 34 Bayani N, Rugina M, Haddad-Vergnes L, Lelong F. High-titer acquired factor V inhibitor responsive to corticosteroids and cyclophosphamide in a patient with two malignant tumors. Am J Hematol 2002; 71 (1) 33-36
    CrossrefPubMedSuche in Google Scholar
  • 35 Lebrun A, Leroy-Matheron C, Arlet JB, Bartolucci P, Michel M. Successful treatment with rituximab in a patient with an acquired factor V inhibitor. Am J Hematol 2008; 83 (2) 163-164
    CrossrefPubMedSuche in Google Scholar
  • 36 Lian EC, Tzakis AG, Andrews D. Response of factor V inhibitor to rituximab in a patient who received liver transplantation for primary biliary cirrhosis. Am J Hematol 2004; 77 (4) 363-365
    CrossrefPubMedSuche in Google Scholar
  • 37 Galanakis DK, Ginzler EM, Fikrig SM. Monoclonal IgG anticoagulants delaying fibrin aggregation in two patients with systemic lupus erythematosus (SLE). Blood 1978; 52 (5) 1037-1046
    PubMedSuche in Google Scholar
  • 38 Marciniak E, Greenwood MF. Acquired coagulation inhibitor delaying fibrinopeptide release. Blood 1979; 53 (1) 81-92
    PubMedSuche in Google Scholar
  • 39 Hoots WK, Carrell NA, Wagner RH, Cooper HA, McDonagh J. A naturally occurring antibody that inhibits fibrin polymerization. N Engl J Med 1981; 304 (15) 857-861
    CrossrefPubMedSuche in Google Scholar
  • 40 Plow EF, Edgington TS. Immune responses to the cleavage-associated neoantigens of fibrinogen in man. Identification and characterization of humoral antibodies specific for cleavage fragments. J Clin Invest 1975; 56 (6) 1509-1518
    CrossrefPubMedSuche in Google Scholar
  • 41 Whitaker AN, McFarlane JR, Rowe EA, Lee K, Masci PP. Measurement of autoantibodies against fibrinogen and fibrin degradation products by enzyme-linked immunoassay. Thromb Haemost 1985; 53 (1) 80-85
    PubMedSuche in Google Scholar
  • 42 Michalski A, Kondera-Anasz Z, Gonciarz Z. Autoantibodies against fibrinogen during interferon therapy in patients with chronic viral hepatitis. Ital J Gastroenterol Hepatol 1995; 2 (Suppl) 226-227
    PubMedSuche in Google Scholar
  • 43 MacLean MA, Wilson R, Thomson JA, Krishnamurthy S, Walker JJ. Changes in immunologic parameters in normal pregnancy and spontaneous abortion. Am J Obstet Gynecol 1991; 165 (4 Pt 1) 890-895
    CrossrefPubMedSuche in Google Scholar
  • 44 Mammen EF, Schmidt KP, Barnhart MI. Thrombophlebitis migrans associated with circulating antibodies against fibrinogen. A case report. Thromb Diath Haemorrh 1967; 18 (3–4) 605-611
    PubMedSuche in Google Scholar
  • 45 Mertas A, Kondera-Anasz Z. Antifibrinogen antibodies in serum of blood recipients with post-transfusion reactions. Clin Biochem Rev 1993; 14: 199
    PubMedSuche in Google Scholar
  • 46 Ghosh S, McEvoy P, McVerry BA. Idiopathic autoantibody that inhibits fibrin monomer polymerization. Br J Haematol 1983; 53 (1) 65-72
    CrossrefPubMedSuche in Google Scholar
  • 47 Ruiz-Arguelles A. Spontaneous reversal of acquired autoimmune dysfibrinogenemia probably due to an antiidiotypic antibody directed to an interspecies cross-reactive idiotype expressed on antifibrinogen antibodies. J Clin Invest 1988; 82 (3) 958-963
    CrossrefPubMedSuche in Google Scholar
  • 48 Kondera-Anasz Z. Antibodies against fibrinogen in pregnant women, in post delivery women and in the newborns. Thromb Haemost 1998; 79 (5) 963-968
    PubMedSuche in Google Scholar
  • 49 La Spada AR, Skålhegg BS, Henderson R, Schmer G, Pierce R, Chandler W. Brief report: fatal hemorrhage in a patient with an acquired inhibitor of human thrombin. N Engl J Med 1995; 333 (8) 494-497
    CrossrefPubMedSuche in Google Scholar
  • 50 Sié P, Bezeaud A, Dupouy D , et al. An acquired antithrombin autoantibody directed toward the catalytic center of the enzyme. J Clin Invest 1991; 88 (1) 290-296
    CrossrefPubMedSuche in Google Scholar
  • 51 Fleck RA, Rapaport SI, Rao LV. Anti-prothrombin antibodies and the lupus anticoagulant. Blood 1988; 72 (2) 512-519
    PubMedSuche in Google Scholar
  • 52 Galli M, Barbui T. Antithrombin antibodies: detection and its significance in the antiphospholipid syndrome. Blood 1999; 93: 2149-2157
    PubMedSuche in Google Scholar
  • 53 Bajaj SP, Rapaport SI, Barclay S, Herbst KD. Acquired hypoprothrombinemia due to non-neutralizing antibodies to prothrombin: mechanism and management. Blood 1985; 65 (6) 1538-1543
    PubMedSuche in Google Scholar
  • 54 Holm M, Andreasen R, Ingerslev J. Management of bleeding using recombinant factor VIIa in a patient suffering from bleeding tendency due to a lupus anticoagulant-hypoprothrombinemia syndrome. Thromb Haemost 1999; 82 (6) 1776-1778
    PubMedSuche in Google Scholar
  • 55 Castro O, Farber LR, Clyne LP. Circulating anticoagulants against factors IX and XI in systemic lupus erythematosus. Ann Intern Med 1972; 77 (4) 543-548
    CrossrefPubMedSuche in Google Scholar
  • 56 Largo R, Sigg P, von Felten A, Straub PW. Acquired factor-IX inhibitor in a nonhaemophilic patient with autoimmune disease. Br J Haematol 1974; 26 (1) 129-140
    CrossrefPubMedSuche in Google Scholar
  • 57 Ozsoylu S, Ozer FL. Acquired factor IX deficiency. A report of two cases. Acta Haematol 1973; 50 (5) 305-314
    CrossrefPubMedSuche in Google Scholar
  • 58 Abshire T, Kenet G. Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors. J Thromb Haemost 2004; 2 (6) 899-909
    CrossrefPubMedSuche in Google Scholar
  • 59 Campbell E, Sanal S, Mattson J , et al. Factor VII inhibitor. Am J Med 1980; 68 (6) 962-964
    CrossrefPubMedSuche in Google Scholar
  • 60 Delmer A, Horellou MH, Andreu G , et al. Life-threatening intracranial bleeding associated with the presence of an antifactor VII autoantibody. Blood 1989; 74 (1) 229-232
    PubMedSuche in Google Scholar
  • 61 Rao LV, Zivelin A, Iturbe I, Rapaport SI. Antibody-induced acute factor X deficiency: clinical manifestations and properties of the antibody. Thromb Haemost 1994; 72 (3) 363-371
    PubMedSuche in Google Scholar
  • 62 Smith SV, Liles DK, White II GC, Brecher ME. Successful treatment of transient acquired factor X deficiency by plasmapheresis with concomitant intravenous immunoglobulin and steroid therapy. Am J Hematol 1998; 57 (3) 245-252
    CrossrefPubMedSuche in Google Scholar
  • 63 Kamikubo Y, Miyamoto S, Iwasa A, Ishii M, Okajima K. Purification and characterization of factor VII inhibitor found in a patient with life threatening bleeding. Thromb Haemost 2000; 83 (1) 60-64
    PubMedSuche in Google Scholar
  • 64 Goodrick MJ, Prentice AG, Copplestone JA, Pamphilon DH, Boon RJ. Acquired factor XI inhibitor in chronic lymphocytic leukaemia. J Clin Pathol 1992; 45 (4) 352-353
    CrossrefPubMedSuche in Google Scholar
  • 65 Reece EA, Clyne LP, Romero R, Hobbins JC. Spontaneous factor XI inhibitors. Seven additional cases and a review of the literature. Arch Intern Med 1984; 144 (3) 525-529
    CrossrefPubMedSuche in Google Scholar
  • 66 Bortoli R, Monticielo OA, Chakr RM , et al. Acquired factor XI inhibitor in systemic lupus erythematosus—case report and literature review. Semin Arthritis Rheum 2009; 39 (1) 61-65
    CrossrefPubMedSuche in Google Scholar
  • 67 Fukue H, Anderson K, McPhedran P, Clyne L, McDonagh J. A unique factor XIII inhibitor to a fibrin-binding site on factor XIIIA. Blood 1992; 79 (1) 65-74
    PubMedSuche in Google Scholar
  • 68 Lorand L, Maldonado N, Fradera J, Atencio AC, Robertson B, Urayama T. Haemorrhagic syndrome of autoimmune origin with a specific inhibitor against fibrin stabilizing factor (factor XIII). Br J Haematol 1972; 23 (1) 17-27
    PubMedSuche in Google Scholar
  • 69 Lorand L, Velasco PT, Murthy SN, Lefebvre P, Green D. Autoimmune antibody in a hemorrhagic patient interacts with thrombin-activated factor XIII in a unique manner. Blood 1999; 93 (3) 909-917
    PubMedSuche in Google Scholar
  • 70 Daly HM, Carson PJ, Smith JK. Intracerebral haemorrhage due to acquired factor XIII inhibitor—successful response to factor XIII concentrate. Blood Coagul Fibrinolysis 1991; 2 (4) 507-514
    CrossrefPubMedSuche in Google Scholar
  • 71 Luo YY, Zhang GS. Acquired FXIII inhibitor: clinical features, treatment, fibrin structure and epitope determination. Haemophilia 2011; 17: 393-398
    CrossrefPubMedSuche in Google Scholar
  • 72 Ajzner E, Schlammadinger A, Kerényi A , et al. Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency. Blood 2009; 113 (3) 723-725
    CrossrefPubMedSuche in Google Scholar
  • 73 Karimi M, Bereczky Z, Cohan N, Muszbek L. Factor XIII deficiency. Semin Thromb Hemost 2009; 35 (4) 426-438
    Thieme ConnectPubMedSuche in Google Scholar
  • 74 Favaloro EJ. Laboratory testing in disseminated intravascular coagulation. Semin Thromb Hemost 2010; 36 (4) 458-467
    Thieme ConnectPubMedSuche in Google Scholar
  • 75 Favaloro EJ, Lippi G. Laboratory testing and/or monitoring of the new oral anticoagulants/antithrombotics: for and against?. Clin Chem Lab Med 2011; 49 (5) 755-757
    CrossrefPubMedSuche in Google Scholar
  • 76 Favaloro EJ, Lippi G, Koutts J. Laboratory testing of anticoagulants: the present and the future. Pathology 2011; 43 (7) 682-692
    CrossrefPubMedSuche in Google Scholar
  • 77 Peyvandi F, Favaloro EJ. Rare bleeding disorders. Semin Thromb Hemost 2009; 35 (4) 345-347
    Thieme ConnectPubMedSuche in Google Scholar
  • 78 Peyvandi F, Palla R, Menegatti M, Mannucci PM. Introduction. Rare bleeding disorders: general aspects of clinical features, diagnosis, and management. Semin Thromb Hemost 2009; 35 (4) 349-355
    Thieme ConnectPubMedSuche in Google Scholar
  • 79 de Moerloose P, Neerman-Arbez M. Congenital fibrinogen disorders. Semin Thromb Hemost 2009; 35 (4) 356-366
    Thieme ConnectPubMedSuche in Google Scholar
  • 80 Lancellotti S, De Cristofaro R. Congenital prothrombin deficiency. Semin Thromb Hemost 2009; 35 (4) 367-381
    Thieme ConnectPubMedSuche in Google Scholar
  • 81 Asselta R, Peyvandi F. Factor V deficiency. Semin Thromb Hemost 2009; 35 (4) 382-389
    Thieme ConnectPubMedSuche in Google Scholar
  • 82 Mariani G, Bernardi F. Factor VII deficiency. Semin Thromb Hemost 2009; 35 (4) 400-406
    Thieme ConnectPubMedSuche in Google Scholar
  • 83 Menegatti M, Peyvandi F. Factor X deficiency. Semin Thromb Hemost 2009; 35 (4) 407-415
    Thieme ConnectPubMedSuche in Google Scholar
  • 84 Duga S, Salomon O. Factor XI deficiency. Semin Thromb Hemost 2009; 35 (4) 416-425
    Thieme ConnectPubMedSuche in Google Scholar
  • 85 Karimi M, Bereczky Z, Cohan N, Muszbek L. Factor XIII deficiency. Semin Thromb Hemost 2009; 35 (4) 426-438
    Thieme ConnectPubMedSuche in Google Scholar