Main Article Content

Maria Gabriella Mazzucconi
Erminia Baldacci
Antonietta Ferretti
Cristina Santoro


: autoantibodies against FVIII, bleeding symptoms, bleeding treatment, eradication therapy.


Acquired Haemophilia A is a rare acquired bleeding disorder caused by autoantibodies directed against Factor VIII, which neutralize FVIII activity. These inhibitors differ from alloantibodies against FVIII which can occur in congenital Haemophilia A after repeated exposures to plasma-derived or recombinant FVIII products. In most cases the disease occurs suddenly in subjects without personal or familiar history of bleedings, with symptoms that may be mild, moderate or severe. However, only laboratory alterations are present in   ? 30% of patients. The incidence varies from 1 to 4 cases per million/year; more than 80% of patients are elderly, males and females are similarly affected. There is a small peak of incidence related to pregnancy in young women aged 20–40 years. The disease may be underdiagnosed in the elderly. The diagnostic algorithm is based on an isolated prolonged activated partial thromboplastin time, normal thrombin time, absence of Lupus Anticoagulant and a mixing test that reveals the presence of an inhibitor: the finding of reduced FVIII activity and the detection of neutralising autoantibodies against FVIII lead to diagnosis. The disease is idiopathic in   44%-63% of cases, while in the others etiological factors are present. Bleeding prevention and treatment are based on therapeutic tools as bypassing agents, recombinant porcine FVIII concentrate or, in a limited number of cases, FVIII concentrates and desmopressin. As soon as the diagnosis has been made, immunosuppressive therapy must be started to eradicate the inhibitor. Better knowledge of the disease, optimal management of bleeding and eradication of the inhibitor have significantly reduced morbidity and mortality in most patients. 


Keywords: autoantibodies against FVIII, bleeding symptoms, bleeding treatment, eradication therapy. 


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1) Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A.Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol 2003; 121: 21-35

2) Oldenburg J, Zeitler H, Pavlova A. Genetic markers in acquired haemophilia. Haemophilia. 2010;16 (Suppl 3):41–45

3) Tiede A, Eisert R, Czwalinna A, Miesbach W, Scharrer I, Ganser A.
Acquired haemophilia caused by non-haemophilic factor VIII gene variants. Ann Hematol. 2010; 89:607–612.

4) Pavlova A, Zeitler H, Scharrer I, Brackmann HH, Oldenburg J. HLA
genotype in patients with acquired haemophilia A. Haemophilia. 2010; 16:107–112.

5) Collins PW: Treatment of acquired hemophilia A. J Thromb Haemost 2007; 5 (5):893-900.

6) Hay CR, Brown S, Collins PW, Keeling DM, Liesner R: The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation. Br J Haematol 2006; 133 (6):591-605.

7) Knoebl P, Marco P, Baudo F, Collins P, Huth-Ku¨ hne A, Nemes L, Pellegrini F, Tengborn L, Le´ vesque H, on behalf of the EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).
J Thromb Haemost 2012; 10: 622–31.

8) Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA, Hay CR. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors Organisation. Blood 2007; 109: 1870–1877.

9) P Collins, F Baudo, Angela Huth-Kühne, J Ingerslev, C M Kessler, M E Mingot Castellano, M Shima, J St-Louis and H Lévesque. Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Research Notes 2010; 3:161

10) M Franchini and G Lippi. Acquired factor VIII inhibitors. Blood; 2008;112 :250-255

11) J Charlebois, G -É Rivarda, J n St-Louis Management of acquired hemophilia A: Review of current evidence. Transfusion and Apheresis Science 57; 2018 717–720

12) Franchini M, Targher G, Montagnana M, Lippi G. Laboratory, clinical and therapeutic aspects of acquired haemophilia A. Clin Chim Acta 2008; 395: 14-18

13) Collins PW, Percy CL. Advances in the understanding of acquired haemophilia A: implications for clinical practice. Br J Haematol 2010; 148: 183-194.

14) Verbruggen B, et al. The Nijmegen modification of the Bethesda assay for factor VIII:C inhibitors: improved specificity and reliability. Thromb Haemost 1995; 73: 247-251.

15) Sahud MA, Pratt KP, Zhukov O, Qu K, Thompson AR. ELISA system for detection of immune responses to FVIII: a study of 246 samples and correlation with the Bethesda assay. Haemophilia. 2007 May;13 (3):317-22.

16) Batty P, Moore GW, Platton S, Maloney JC, Palmer B, Bowles L, Pasi KJ, Rangarajan S, Hart DP. Diagnostic accuracy study of a factor VIII ELISA for detection of factor VIII antibodies in congenital and acquired haemophilia A. Thromb Haemost. 2015;114:804–811.

17) Tiede A, Werwitzke S, Scharf RE. Laboratory diagnosis of acquired hemophilia A: limitations, consequences, and challenges. Semin Thromb Hemost. 2014;40:803–811

18) Kruse-Jarres R, Kempton Christine L, Baudo F,. Collins PW, Knoebl P,. Leissinger CA, Tiede A,. Kessler CM. Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017;92:695–705.

19) lgiman M, Dietrich G, Nydegger UE, Boieldieu D, Sultan Y, Kazatchkine MD. Natural antibodies to factor VIII (anti-hemophilicfactor) in healthy individuals. Proc Natl Acad Sci USA 1992; 89: 3795–3799.

20) Lollar P. Pathogenic antibodies to coagulation factors. Part one: Factor VIII and factor IX. J Thromb Haemost 2004; 2; 1082–1095.

21) Reding MT. Immunological aspects of inhibitor development. Haemophilia 2006; 12 (Suppl 6): 30–35.

22) Franchini M, Mannucci PM. Acquired haemophilia A: A 2013 update. Thromb Haemost 2013; 110: 1114–1120.

23) Reding MT, et al. Distribution of Th1- and Th2-induced anti-factor VIII IgG subclasses in congenital and acquired haemophilia patients. Thromb Haemost 2002; 88: 568–575.

24) Tiede A, Hofbauer CJ, Werwitzke S, Knöbl P, Gottstein S, Scharf RE, Heinz J, Groß J, Holstein K, Dobbelstein C, Scheiflinger F, Koch A, Reipert BM. Anti-factor VIII IgA as a potential marker of poor prognosis in acquired hemophilia A: results from the GTH-AH 01/2010 study. Blood. 2016 May 12;127(19):2289-2297.

25) Kahle J, Orlowski A, Stichel D ,. Healey J F,. Parker ET, Jacquemin M , Krause M, Tiede A, Schwabe D , Lollar P, and Konigs C. Frequency and epitope specificity of anti–factor VIII C1 domain antibodies in acquired and congenital hemophilia A. Blood. 2017;130(6):808-816.

26) Matsumoto T, Nogami K, Ogiwara K , Midori S. A putative inhibitory mechanism in the tenase complex responsible for loss of coagulation function in acquired haemophilia A patients with anti-C2 autoantibodies. Thromb Haemost 2012; 107: 288–301

27) Lapalud P, Ali T, Cayzac C, Mathieu-Dupas E, Levesque H, Pfeiffer C, Balicchi J, Gruel Y, Borg JY, Schved JF, Granier C, Lavigne-Lissalde G. The IgG autoimmune response in postpartum acquired hemophilia A targets mainly the A1a1 domain of FVIII. J Thromb Haemost 2012;10: 1814–1822.

28) Collins PW, Chalmers E, Hart DP, Liesner R, Rangarajan S, Talks K, Williams M, Hay CR; UK Haemophilia Centre Doctors. Diagnosis and treatment of factor VIII and IX inhibitors in congenital haemophilia: (4th edition). UK Haemophilia Centre Doctors Organization.
Br J Haematol. 2013; 162: 758–773

29) Baudo F, Mostarda G, de Cataldo F. Acquired factor ? and factor ? inhibitors: survey of the Italian haemophila centers (AICE). Haematologica 2003; 88 Suppl 12: S93-99 Available from: URL:

30) Green D, Lechner K: A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost 1981, 45(3):200-203.

31) Lottenberg R, Kentro TB, Kitchens CS. Acquired hemophilia. A natural history study of 16 patients with factor VIII inhibitors receiving little or no therapy. Arch Intern Med 1987;147:

32) Charlebois J, Rivard GÉ, St-Louis J. Management of acquired hemophilia A: Review of current evidence. Transfus Apher Sci. 2018 Dec;57(6):717-720.

33) Vautier M, de Boysson H, Creveuil C, Repesse Y, Borel-Derlon A, Troussard X, Damaj GL, Bienvenu B, Gautier P, Aouba A.Influence of factor VIII level and its inhibitor titer on the therapeutic response to corticosteroids alone in the management of acquired hemophilia: a retrospective single-center study. Medicine (Baltimore) 2016;95 (November (48): e5232.

34) Reeves BN, Key NS. Acquired hemophilia in malignancy. Thromb Res. 2012 Apr;129 Suppl 1:S66-8

35) Sallah S, Wan JY. Inhibitors against factor VIII in patients with cancer. Analysis of 41 patients. Cancer 2001; 91: 1067-1074

36) Michiels JJ. Acquired haemophilia A in women post-partum: clinical manifestations, diagnosis and treatment. Clin Appl Thromb Haemost 2000; 6: 82-86.

37) Solymoss, S. Postpartum acquired factor VIII inhibitors: results of a survey. Am J Hematol 1998; 59: 1–4.

38) Franchini M, Zaffanello M, Lippi G. Acquired hemophilia in pediatrics: a systematic
review. Pediatr Blood Cancer 2010;55 (October (4):606–11.

39) Baudo F, de Cataldo F. Acquired factor VIII inhibitors in pregnancy: data from the Italian Haemophilia Registry relevant to clinical practice. Br J Obstetr Gynecol 2003; 110: 311-314.

40) Tengborn L, et al.; EACH2 registry contributors. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. Br J Obstet Gynecol 2012; 119: 1529-1537.

41) Hauser, I., Schneider, B. & Lechner, K. Post-partum factor VIII inhibitors. A review of the literature with special reference to the value of steroid and immunosuppressive treatment. Thrombosis and Haemostasis, 1995; 73: 1–5.

42) Borg JY, Guillet B, Le Cam-Duchez V, Goudemand J, Lévesque H; SACHA Study Group.
Haemophilia. 2013. Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l'Hémophilie Acquise) registry. Haemophilia. 2013 Jul;19(4):564-570.

43) Tiede A, Klamroth R, Scharf RE, Trappe RU, Holstein K, Huth-Kühne A, Gottstein S, Geisen U, Schenk J, Scholz U, Schilling K, Neumeister P, Miesbach W, Manner D, Greil R, von Auer C, Krause M, Leimkühler K, Kalus U, Blumtritt JM, Werwitzke S, Budde E, Koch A, Knöbl P.
Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study. Blood 2015 Feb 12;125(7):1091-1097.

44) Huang SY, Tsay W, Lin SY, Hsu SC, Hung MH, Shen MC. A study of 65 patients with acquired hemophilia A in Taiwan. J Formos Med Assoc. 2015;114:321–327.

45) Jayakar JP, O'Neill N, Yan M, Nisenbaum R, Garvey MB, Teitel J, Sholzberg M.
Retrospective review of Acquired Haemophilia A from the largest Canadian Haemophilia treatment centre. Haemophilia. 2018 Sep;24(5):e383-e387

46 ) Wermes C, Niekrens C, Sykora KW. Successful long-time treatment with mycophenolate-mofetil in a child with acquired factor VIII inhibitor. Hamostaseologie. 2012;32 Suppl 1: S75-8.

47) Somaratne PD, Jansan J, Senanayake HM, Ratnamalala V, Jayathilake MM, Thirumavalavan K. A child with acquired haemophilia. Ceylon Med J. 2014 Jun;59 (2):66-67.

48) Fletcher M, Crombet O, Morales-Arias J. Successful treatment of acquired hemophilia a with rituximab and steroids in a 5-year-old girl. J Pediatr Hematol Oncol. 2014 Mar;36(2):e103-104.

49) Todo K, Ohmae T, Osamura T, Kiyosawa N, Sugimoto M, Shima M, Imamura T, Imashuku S. Exsanguinating bleeding following tooth extraction in a 12-year-old girl: a rare case of acquired haemophilia A. Blood Coagul Fibrinolysis. 2015 Dec;26(8):964-966.

50) Jones L, Dandoy C, Jodele S, Myers KC, Luchtman-Jones L, Quinn CT, Mullins E, El-Bietar J.Successful management of concurrent acquired hemophilia A and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient. Bone Marrow Transplant. 2018 Apr;53(4):487-489.

51) Takeyama M, Nogami K, Kajimoto T, Ogiwara K, Matsumoto T, Shima M. First report of real-time monitoring of coagulation function potential and IgG subtype of anti-FVIII autoantibodies in a child with acquired hemophilia A associated with streptococcal infection and amoxicillin. Int J Hematol. 2018 Jan;107(1):112-116

52) Gamage M, Weerasinghe S, Nasoor M, Karunarathne AMPW, Abeyrathne SP.
Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A.
Case Rep Hematol. 2018 Oct 24; 2018:6208597

53) Godaert L, Bartholet S, Colas S, Kanagaratnam L, Fanon JL, Dramé M. Acquired Hemophilia A in Aged People: A Systematic Review of Case Reports and Case Series.
Semin Hematol. 2018 Oct;55(4):197-201

54) Yamaguchi T, Kudo N, Endo S, Usui T, Imashuku S. Management of Acquired Hemophilia A in Elderly Patients. Case Rep Hematol. 2018 Nov 13; 2018:6757345

55) Mudad R, Kane WH. DDAVP in acquired haemophilia A: case report and review
of the literature. Am J Hematol 1993; 43: 295-299.

56) Franchini M, Lippi G. The use of desmopressin in acquired haemophilia A: a systematic review. Blood Transfus 2011; 9: 377-382

57) Baudo F, et al.; EACH2 registry contributors. Management of bleeding in acquired
haemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood 2012; 120: 39-46

58) Morrison AE, et al. Use of porcine factor VIII in the treatment of patients with
acquired haemophilia. Blood 1993; 81: 1513-1520.

59) Giangrande PL. Porcine factor VIII. Haemophilia 2012 May;18(3):305-309

60) Doering CB, Healey JF, Parker ET, Barrow RT, Lollar P. High level expression of
recombinant porcine coagulation factor VIII. J Biol Chem 2002; 277: 38345–9.

61) Kempton CL, Abshire TC, Deveras RA et al. Pharmacokinetics and safety of OBI-
1, a recombinant B domain-deleted porcine factor VIII, in subjects with haemophilia A.
Haemophilia 2012; 18: 798–804.

62) Lillicrap D., Schiviz A. , Apostol C., Wojciechowski F., Horling F. , Lai C. K. , Piskernik C., Hoellriegl W., and Lollar P. Porcine recombinant factor VIII (Obizur; OBI-1; BAX801):product characteristics and preclinical profile. Haemophilia. 2016 Mar; 22(2):308-317.

63) Kruse-Jarres R, St-Louis J, Greist A, et al. Efficacy and safety of OBI-1, an antihaemophilic factor VIII (recombinant), porcine sequence, in subjects with acquired haemophilia A. Haemophilia 2015; 21:162–170.

64) Martin K, Kasthuri R, Mooberry MJ, Chen SL, Key NS, Ma AD. Lower doses of recombinant porcine factor VIII maintain excellent haemostatic efficacy. Haemophilia. 2016 Nov; 22(6):e549-e551.

65) Tarantino MD, Cuker A, Hardesty B, Roberts JC, Sholzberg M. Recombinant porcine sequence factor VIII (rpFVIII) for acquired haemophilia A: practical clinical experience of its use in seven patients. Haemophilia. 2017 Jan; 23(1):25-32.

66) Hay CRM, Negrier C, Ludlam CA. The treatment of bleeding in acquired haemophilia with recombinant factor VIIa: a multicenter study. Thromb Haemost 1997; 78: 3-7.

67) Baudo F, de Cataldo F, Gaidano G; Italian registry of acquired hemophilia. Treatment of acquired factor VIII inhibitor with recombinant activated factor VIIa: data from the Italian registry of acquired hemophilia. Haematologica. 2004 Jun;89(6):759-761.

68) Sumner MJ, Geldziler BD, Pedersen M, Seremetis S. Treatment of acquired haemophilia with recombinant activated FVII: a critical appraisal. Haemophilia 2007; 13: 451-461.

69) Amano K, Seita I, Higasa S, Sawada A, Kuwahara M, Shima M. Treatment of acute bleeding in acquired haemophilia A with recombinant activated factor VII: analysis of 10-year Japanese postmarketing surveillance data. Haemophilia. 2017 Jan;23(1):50-58.

70) Franchini M, Lippi G. Recombinant activated factor VII: Mechanisms of action and current indications. Semin Thromb Haemost 2010; 36: 485-492.

71) Sallah S. Treatment of acquired haemophilia with factor eight inhibitor bypassing activity. Haemophilia 2004; 10: 169-173.

72) Borg JY, Négrier C, Durieu I, Dolimier E, Masquelier AM, Lévesque H; FEIBHAC Study Group.FEIBA in the treatment of acquired haemophilia A: results from the prospective multicentre French 'FEIBA dans l'hémophilie A acquise' (FEIBHAC) registry.Haemophilia. 2015 May;21(3):330-337

73) Zanon E, Pasca S, Santoro C, Gamba G, Siragusa SM, Rocino A, Cantori I, Federici AB, Mameli L, Giuffrida G, Falanga A, Lodigiani C, Santoro RC, Milan M, Ambaglio C, Napolitano M, Mazzucconi MG. Activated prothrombin complex concentrate (FEIBA®) in acquired haemophilia A: a large multicentre Italian study - the FAIR Registry. Br J Haematol. 2019 Mar;184(5):853-855.

74) Ingerslev J, Sorensen B. Parallel use of by-passing agents in haemophilia with inhibitors: a critical review. Br J Haematol. 2011;155: 256–262.

75) Pasca S, Ambaglio C, Rocino A, Santoro C, Cantori I, Zanon E; FAIR Study Group.
Combined use of antifibrinolytics and activated prothrombin complex concentrate (aPCC) is not related to thromboembolic events in patients with acquired haemophilia A: data from FAIR Registry. J Thromb Thrombolysis. 2019 Jan;47(1):129-133.

76) Kim CH, Simmons SC, Bui CM, Jiang N, Pham HP. aPCC vs. rFVIIa for the treatment of bleeding in patients with acquired haemophilia - a cost-effectiveness model. Vox Sang. 2019 Jan;114 (1):63-72.

77) Franchini M, et al. Extracorporeal immunoadsorption for the treatment of coagulation
inhibitors. Semin Thromb Haemost 2009; 35: 76-80.

78) Oldenburg J, Mahlangu JN, Kim B, Schmitt C, Callaghan MU, Young G, Santagostino E, Kruse-Jarres R, Negrier C, Kessler C, Valente N, Asikanius E, Levy GG, Windyga J, Shima M. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med 2017; 377: 809–818.

79) Young G, Liesner R, Chang T, Sidonio R, Oldenburg J, Jiménez-Yuste V, Mahlangu J, Kruse-Jarres R, Wang M, Uguen M, Doral MY, Wright LY, Schmitt C, Levy GG, Shima M, Mancuso ME. A multicenter, open-label phase 3 study of emicizumab prophylaxis in children with hemophilia A with inhibitors. Blood. 2019 Dec 12;134(24):2127-2138.

80) Mahlangu J, Oldenburg J, Paz-Priel I, Negrier C, Niggli M, Mancuso ME, Schmitt C, Jiménez-Yuste V, Kempton C, Dhalluin C, Callaghan MU, Bujan W, Shima M, Adamkewicz JI, Asikanius E, Levy GG, Kruse-Jarres R. Emicizumab Prophylaxis in Patients Who Have Hemophilia A without Inhibitors. N Engl J Med. 2018 Aug 30;379(9):811-822.

81) Pipe SW, Shima M, Lehle M, Shapiro A, Chebon S, Fukutake K, Key NS, Portron A, Schmitt C, Podolak-Dawidziak M, Selak Bienz N, Hermans C, Campinha-Bacote A, Kiialainen A, Peerlinck K, Levy GG, Jiménez-Yuste V. Efficacy, safety, and pharmacokinetics of emicizumab prophylaxis given every 4 weeks in people with haemophilia A (HAVEN 4): a multicentre, open-label, non-randomised phase 3 study. Lancet Haematol. 2019 Jun;6(6):e295-e305.

82) Knoebl P, Sperr W, Schellongowski P, Staudinger T, Jilma?Stohlawetz P, Quehenberger P, et al. Emicizumab for the treatment of acquired hemophilia A: lessons learned from 4 very different cases [abstract] Blood. 2018;132(Suppl 1):2476.

83) Dane KE, Lindsley JP, Streiff MB, Moliterno AR, Khalid MK, Shanbhag S. Successful use of emicizumab in a patient with refractory acquired hemophilia A and acute coronary syndrome requiring percutaneous coronary intervention. Res Pract Thromb Haemost. 2019 Apr 9;3(3):420-423.

84) Möhnle P, Pekrul I, Spannagl M, Sturm A, Singh D, Dechant C. Emicizumab in the Treatment of Acquired Haemophilia: A Case Report. Transfus Med Hemother. 2019 Apr;46(2):121-123.

85) Al-Banaa K, Alhillan A, Hawa F, Mahmood R, Zaki A, El Abdallah M, Zimmerman J, Musa F. Emicizumab Use in Treatment of Acquired Hemophilia A: A Case Report. Am J Case Rep. 2019 Jul 18;20:1046-1048.

86) Takeyama M, Nogami K, Matsumoto T, Noguchi-Sasaki M, Kitazawa T, Shima M. An anti-factor IXa/factor X bispecific antibody, emicizumab, improves ex vivo coagulant potentials in plasma from patients with acquired hemophilia A. J Thromb Haemost. 2020 Jan 26. doi: 10.1111/jth.14746. [Epub ahead of print]

87) Green D, Rademaker AW, Briët E. A prospective, randomized trial of prednisone and cyclophosphamide in the treatment of patients with factor VIII autoantibodies. Thromb Haemost. 1993 Nov 15;70(5):753-757.

88) Bitting RL, Bent S, Li Y, Kohlwes J. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis. 2009 Oct;20(7):517-523

89) Collins P, Baudo F, Knoebl P, Lévesque H, Nemes L, Pellegrini F, Marco P, Tengborn L, Huth-Kühne A; EACH2 registry collaborators. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Blood. 2012 Jul 5;120(1):47-55.

90) Nemes L, Pitlik E. New protocol for immune tolerance induction in acquired haemophilia. Haematologica 2000; 85: 64-68.

91) Zeitler H, Ulrich-Merzenich G, Hess L, Konsek E, Unkrig C, Walger P, Vetter H, Brackmann HH. Treatment of acquired haemophilia by the Bonn-Malmo Protocol:documentation of an in vivo immunomodulating concept. Blood 2005; 105:2287-2293.

92) Franchini M, Mannucci PM. Inhibitor eradication with rituximab in haemophilia: where do we stand? Br J Haematol. 2014 Jun;165(5):600-608.

93) D'Arena G, Grandone E, Di Minno MN, Musto P, Di Minno G. The anti-CD20 monoclonal antibody rituximab to treat acquired haemophilia A. Blood Transfus. 2016 May;14(2):255-261

94) Maillard H, Launay D, Hachulla E, Goudemand J, Lambert M, Morell-Dubois S, Queyrel V, Hatron PY. Rituximab in postpartum-related acquired hemophilia. Am J Med. 2006 Jan;119(1):86-88.

95) Santoro C, Rago A, Biondo F, De Propris MS, De Vellis A, Guarini A, Pignoloni P, Mazzucconi MG. Efficacy of rituximab treatment in postpartum acquired haemophilia A. Haemophilia. 2008 Jan;14(1):147-149.

96) Dedeken L, St-Louis J, Demers C, Meilleur C, Rivard GE. Postpartum acquired haemophilia: a single centre experience with rituximab. Haemophilia. 2009;15:1166–1168

97) Kyrle PA. High factor VIII and the risk of venous thromboembolism. Hamostaseologie 2003;23:41-44

98) Mizrahi T, Doyon K, Dubé E, Bonnefoy A, Warner M, Cloutier S, Demers C, Castilloux JF, Rivard GE, St-Louis J. Relapse pattern and long-term outcomes in subjects with acquired haemophilia A. Haemophilia. 2019 Mar;25(2):252-257.

99) Huth-Kühne A, Baudo F, Collins P, Ingerslev J, Kessler CM, Lévesque H, Mingot Castellano ME, Shima M, and St-Louis J. International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica 2009; 94:566-575

100) Franchini M, Castaman G, Coppola A, Santoro C, Zanon E, Di Minno G, Morfini M, Santagostino E, Rocino A, on behalf of the AICE Working Group. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management. Blood Transfus 2015; 13; 498-513

101) Guyatt GH, Oxman AD, Kunz R, Vist GE, Falck-Ytter Y, Schünemann HJ; GRADE Working Group. What is "quality of evidence" and why is it important to clinicians? BMJ 2008; 336: 995-998.