Difference between revisions of "Acquired hemophilia A"
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Revision as of 02:25, 24 July 2017
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Is there a regimen missing from this list? Would you like to share a different dosage/schedule or an additional reference for a regimen? Have you noticed an error? Do you have an idea that will help the site grow to better meet your needs and the needs of many others? You are invited to contribute to the site.
5 regimens on this page
7 variants on this page
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This page is meant to cover regimens that are used above and beyond replacement products. At this time replacement products are out of scope but could be added by an interested party.
Acquired factor VIII inhibitor, hemophilia A (factor 8 inhibitor)
Incidence: 1.48/million/year[1]
Cyclophosphamide & Prednisolone
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Regimen
Study | Evidence |
Collins et al. 2007 | Review |
Chemotherapy
- Cyclophosphamide (Cytoxan) 1 to 2 mg/kg PO once per day
- Prednisolone (Millipred) 1 mg/kg PO once per day
References
- 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 PubMed
Cyclophosphamide & Prednisone
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Regimen #1
Study | Evidence |
Shaffer et al. 1997 | Case report |
Chemotherapy
- Cyclophosphamide (Cytoxan) 100 to 200 mg PO once per day
- Prednisone (Sterapred) 50 to 80 mg PO once per day
- Per Shaffer et al. 1997: "the lower ends of these ranges became the standard therapy to minimize side effects"
Used 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 #2
Study | Evidence |
Bayer et al. 1999 | Case report |
Chemotherapy
- Cyclophosphamide (Cytoxan) 100 PO once per day
- Prednisone (Sterapred) 1 mg/kg PO once per day
Used until therapeutic response. "Prednisone and cyclophosphamide were tapered slowly."
Regimen #3
Study | Evidence |
Green et al. 1993 | Case series, >20 pts |
Chemotherapy
- Prednisone (Sterapred) 1 mg/kg PO once per day
If no response to just prednisone after 21 days, change treatment to:
- Cyclophosphamide (Cytoxan) 2 mg/kg (route not specified) once per day
- Prednisone (Sterapred) 1 mg/kg PO once per day
References
- For historic purposes only; case reports of varying doses & schedule: 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 PubMed
- 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. PubMed
- 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 contains verified protocol PubMed
- 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 contains verified protocol PubMed
- 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 PubMed
- Baudo F, Caimi T, de Cataldo F. Diagnosis and treatment of acquired haemophilia. Haemophilia. 2010 May;16(102):102-6.
Prednisolone (Millipred)
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Regimen
Study | Evidence |
Collins et al. 2007 | Review |
Therapy
- Prednisolone (Millipred) 1 mg/kg PO once per day
References
- 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 PubMed
Prednisone (Sterapred)
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Regimen
Study | Evidence |
Green et al. 1981 | Case report, review |
Therapy
- Prednisone (Sterapred) 1 mg/kg PO once per day
References
- 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. PubMed
- Spero JA, Lewis JH, Hasiba U. Corticosteroid therapy for acquired F VIII:C inhibitors. Br J Haematol. 1981 Aug;48(4):635-42. PubMed 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. PubMed
- 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 (Rituxan)
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Regimen #1
Study | Evidence |
Leissinger et al. 2014 | Phase II |
Chemotherapy
- Rituximab (Rituxan) 375 mg/m2 IV once per day on days 1, 8, 15, 22
28-day course
Regimen #2
Study | Evidence |
Yao et al. 2014 | Case report |
Chemotherapy
- Rituximab (Rituxan) 100 mg/m2 IV once per day on days 1, 8, 15, 22
28-day course
References
- 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 PubMed
- Kempton CL, Allen G, Hord J, Kruse-Jarres R, Pruthi RK, Walsh C, Young G, Soucie JM. Eradication of factor VIII inhibitors in patients with mild and moderate hemophilia A. Am J Hematol. 2012 Sep;87(9):933-6. link to original article PubMed
- Leissinger C, Josephson CD, Granger S, Konkle BA, Kruse-Jarres R, Ragni MV, Journeycake JM, Valentino L, Key NS, Gill JC, McCrae KR, Neufeld EJ, Manno C, Raffini L, Saxena K, Torres M, Marder V, Bennett CM, Assmann SF. Rituximab for treatment of inhibitors in haemophilia A. A Phase II study. Thromb Haemost. 2014 Sep 2;112(3):445-58. link to original article PubMed
- 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 PubMed
References
- ↑ 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 PubMed