Acquired hemophilia A

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Last updated on 2024-07-23:5 regimens on this page
8 variants on this page
This page is meant to cover regimens that are used in the context of acquired factor VIII inhibitors. See the inherited coagulopathy page for regimens relevant to inherited factor deficiency. In the future, we may add other acquired coagulopathy pages such as acquired vWD.
Incidence: 1.48/million/yearCollins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA, Hay CR; UK Haemophilia Centre Doctors' Organisation. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood. 2007 Mar 1;109(5):1870-7. link to original article EuroPMC
=Guidelines=
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
==International recommendations==
*2009: Huth-Kühne et al. International Recommendations On The Diagnosis And Treatment Of Patients With Acquired Hemophilia A link to PMC article EuroPMC
=All lines of therapy=
==Cyclophosphamide & Prednisolone==
===Regimen===
Study 	Evidence
Collins et al. 2007	Review
====Immunosuppressive therapy====
*Cyclophosphamide (Cytoxan) 1 to 2 mg/kg PO once per day
*Prednisolone (Millipred) 1 mg/kg PO once per day
One course
===References===
# Review: Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA, Hay CR; UK Haemophilia Centre Doctors' Organisation. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood. 2007 Mar 1;109(5):1870-7. link to original article EuroPMC
==Cyclophosphamide & Prednisone==
===Regimen variant #1===
Study 	Evidence
Shaffer et al. 1997	Case report
Note: Per Shaffer et al. 1997, "the lower ends of these ranges became the standard therapy to minimize side effects"
====Immunosuppressive therapy====
*Cyclophosphamide (Cytoxan) 100 to 200 mg PO once per day
*Prednisone (Sterapred) 50 to 80 mg PO once per day
Continued until therapeutic response "Cyclophosphamide and prednisone were continued at full doses until the factor VIII inhibitor titer decreased to zero; after this, the doses of prednisone and cyclophosphamide were slowly decreased."
===Regimen variant #2===
Study 	Evidence
Bayer et al. 1999	Case report
====Immunosuppressive therapy====
*Cyclophosphamide (Cytoxan) 100 mg PO once per day
*Prednisone (Sterapred) 1 mg/kg PO once per day
Continued until therapeutic response, then prednisone and cyclophosphamide were tapered slowly
===Regimen variant #3===
Study 	Dates of enrollment	Evidence
Green et al. 1993	Not reported 	Randomized, fewer than 20 pts (E-esc)
====Preceding treatment====
*Prednisone x 21 d, with no response
====Immunosuppressive therapy====
*Cyclophosphamide (Cytoxan) 2 mg/kg (route not specified) once per day
*Prednisone (Sterapred) 1 mg/kg PO once per day
One course
===References===
# Case series: Herbst KD, Rapaport SI, Kenoyer DG, Stanton W, Feinstein DI. Syndrome of an acquired inhibitor of factor VIII responsive to cyclophosphamide and prednisone. Ann Intern Med. 1981 Nov;95(5):575-8. link to original article EuroPMC
# 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):753-7. link to original article EuroPMC
# Case report: Shaffer LG, Phillips MD. Successful treatment of acquired hemophilia with oral immunosuppressive therapy. Ann Intern Med. 1997 Aug 1;127(3):206-9. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
# Case report: Bayer RL, Lichtman SM, Allen SL, Budman DR, Buchbinder A, Fetten J, Kolitz J, Loscalzo J. Acquired factor VIII inhibitors--successful treatment with an oral outpatient regimen. Am J Hematol. 1999 Jan;60(1):70-1. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
# Case series: Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA, Hay CR; UK Haemophilia Centre Doctors' Organisation. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood. 2007 Mar 1;109(5):1870-7. link to original article EuroPMC
==Prednisolone monotherapy==
===Regimen===
Study 	Evidence
Collins et al. 2007	Review
Note: Duration of therapy was not explicitly described.
====Immunosuppressive therapy====
*Prednisolone (Millipred) 1 mg/kg PO once per day
One course
===References===
# Review: Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA, Hay CR; UK Haemophilia Centre Doctors' Organisation. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood. 2007 Mar 1;109(5):1870-7. link to original article EuroPMC
==Prednisone monotherapy==
===Regimen===
Study 	Evidence
Green et al. 1993	Non-randomized part of RCT
====Immunosuppressive therapy====
*Prednisone (Sterapred) 1 mg/kg PO once per day
21-day course
====Subsequent treatment====
*Green et al. 1993, if no response after 21 days: Cyclophosphamide versus Cyclophosphamide & Prednisone
===References===
# Case series: Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost. 1981 Jun 30;45(3):200-3. EuroPMC
# Case series: Spero JA, Lewis JH, Hasiba U. Corticosteroid therapy for acquired F VIII:C inhibitors. Br J Haematol. 1981 Aug;48(4):635-42. link to original article EuroPMC content property of HemOnc.org
# 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):753-7. link to original article EuroPMC
# Review: 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 Apr;121(1):21-35. link to original article
==Rituximab monotherapy==
===Regimen variant #1, low-dose===
Study 	Evidence
Yao et al. 2014	Case report
====Immunosuppressive therapy====
*Rituximab (Rituxan) 100 mg/m2 IV once per day on days 1, 8, 15, 22
28-day course
===Regimen variant #2, standard-dose===
Study 	Evidence
Field et al. 2006	Case series
====Immunosuppressive therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once per day on days 1, 8, 15, 22
28-day course
===References===
# Case series: Field JJ, Fenske TS, Blinder MA. Rituximab for the treatment of patients with very high-titre acquired factor VIII inhibitors refractory to conventional chemotherapy. Haemophilia. 2007 Jan;13(1):46-50. link to original article dosing details in abstract have been reviewed by our editors EuroPMC
# Review: Franchini M. Rituximab in the treatment of adult acquired hemophilia A: a systematic review. Crit Rev Oncol Hematol. 2007 Jul;63(1):47-52. link to original article EuroPMC
# Case report: Yao Q, Zhu X, Liu Y, Zhang F, Yuan T, Xu J, Wang X. Low-dose rituximab in the treatment of acquired haemophilia. Hematology. 2014 Dec;19(8):483-6. link to original article EuroPMC
=References=
Category:Acquired hemophilia A regimens
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Category:Autoimmune hematologic conditions
Category:Bleeding disorders