Revision as of 00:17, 1 March 2023

5 regimens on this page 7 variants on this page

This page is meant to cover regimens that are used in the context of acquired factor inhibitors. See the inherited coagulopathy page for regimens relevant to inherited factor deficiency.

Guidelines

2009: International Recommendations On The Diagnosis And Treatment Of Patients With Acquired Hemophilia A

Acquired hemophilia A, all lines of therapy

Incidence: 1.48/million/year^[1]

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

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 PubMed

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	Evidence
Green et al. 1993	Randomized, <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

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 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. link to original article PubMed
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 contains dosing details in manuscript PubMed
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 contains dosing details in manuscript PubMed
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 PubMed

Prednisolone monotherapy

Regimen

Study	Evidence
Collins et al. 2007	Review

Immunosuppressive therapy

Prednisolone (Millipred) 1 mg/kg PO once per day

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 PubMed

Prednisone monotherapy

Regimen

Study	Evidence
Green et al. 1981	Case report, review
Green et al. 1993	Non-randomized portion of RCT

Immunosuppressive therapy

Prednisone (Sterapred) 1 mg/kg PO once per day

Continued for varying durations (see individual papers)

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. PubMed
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 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. link to original article PubMed
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 contains dosing details in abstract PubMed
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
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 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

[1] 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

[1]

@@ Line 10: / Line 10: @@
 |}
 {{TOC limit|limit=3}}
 This page is meant to cover regimens that are used in the context of acquired factor inhibitors. See the '''[[inherited coagulopathy]]''' page for regimens relevant to inherited factor deficiency.
 =Guidelines=
 *'''2009:''' [http://www.haematologica.org/content/94/4/566.short International Recommendations On The Diagnosis And Treatment Of Patients With Acquired Hemophilia A]
 =Acquired hemophilia A, all lines of therapy=
 Incidence: 1.48/million/year<ref>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. [http://www.bloodjournal.org/content/109/5/1870.long link to original article]  [https://pubmed.ncbi.nlm.nih.gov/17047148 PubMed]</ref>
 ==Cyclophosphamide & Prednisolone {{#subobject:2fd67|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:84e0fd|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 29: / Line 26: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Immunosuppressive therapy====
 *[[Cyclophosphamide (Cytoxan)]] 1 to 2 mg/kg PO once per day
 *[[Prednisolone (Millipred)]] 1 mg/kg PO once per day
+</div></div>
 ===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. [http://www.bloodjournal.org/content/109/5/1870.long link to original article] [https://pubmed.ncbi.nlm.nih.gov/17047148 PubMed]
 ==Cyclophosphamide & Prednisone {{#subobject:e5cfe6|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #1 {{#subobject:852fed|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 48: / Line 45: @@
 |}
 ''Note: Per Shaffer et al. 1997, "the lower ends of these ranges became the standard therapy to minimize side effects"
+<div class="toccolours" style="background-color:#b3e2cd">
 ====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."
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #2 {{#subobject:852fed|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 63: / Line 61: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====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'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #3 {{#subobject:404cf3|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 78: / Line 77: @@
 |-
 |}
+<div class="toccolours" style="background-color:#cbd5e8">
 ====Preceding treatment====
 *[[#Prednisone_monotherapy|Prednisone]] x 21 d, with no response
+</div>
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Immunosuppressive therapy====
 *[[Cyclophosphamide (Cytoxan)]] 2 mg/kg (route not specified) once per day
 *[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
+</div></div>
 ===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. [http://annals.org/article.aspx?articleid=695187 link to original article] [https://pubmed.ncbi.nlm.nih.gov/6794408 PubMed]
@@ Line 90: / Line 92: @@
 # '''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. [https://doi.org/10.1002/%28SICI)1096-8652%28199901)60:1%3C70::AID-AJH12%3E3.0.CO;2-D link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/9883809 PubMed]
 # '''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. [http://www.bloodjournal.org/content/109/5/1870.long link to original article] [https://pubmed.ncbi.nlm.nih.gov/17047148 PubMed]
 ==Prednisolone monotherapy {{#subobject:78729f|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:6e2d2d|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 102: / Line 103: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Immunosuppressive therapy====
 *[[Prednisolone (Millipred)]] 1 mg/kg PO once per day
+</div></div>
 ===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. [http://www.bloodjournal.org/content/109/5/1870.long link to original article] [https://pubmed.ncbi.nlm.nih.gov/17047148 PubMed]
 ==Prednisone monotherapy {{#subobject:dcf925|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:c5ce2e|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 122: / Line 123: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Immunosuppressive therapy====
 *[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
 '''Continued for varying durations (see individual papers)'''
+</div>
+<div class="toccolours" style="background-color:#cbd5e7">
 ====Subsequent treatment====
 *Green et al. 1993, if no response after 21 days: [[#Cyclophosphamide_monotherapy_88|Cyclophosphamide]] versus [[#Cyclophosphamide_.26_Prednisone|Cyclophosphamide & Prednisone]]
+</div></div>
 ===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. [https://pubmed.ncbi.nlm.nih.gov/6792737 PubMed]
@@ Line 133: / Line 137: @@
 # 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. [https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0038-1649664 link to original article] [https://pubmed.ncbi.nlm.nih.gov/8128430 PubMed]
 # '''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. [https://doi.org/10.1046/j.1365-2141.2003.04162.x link to original article]
 ==Rituximab monotherapy {{#subobject:6d79c2|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #1, low-dose {{#subobject:3f2f14|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 145: / Line 148: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Immunosuppressive therapy====
 *[[Rituximab (Rituxan)]] 100 mg/m<sup>2</sup> IV once per day on days 1, 8, 15, 22
 '''28-day course'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #2, standard-dose {{#subobject:3f9925|Variant=1}}===
 {| class="wikitable" style="width: 40%; text-align:center;"
@@ Line 159: / Line 163: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Immunosuppressive therapy====
 *[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once per day on days 1, 8, 15, 22
 '''28-day course'''
+</div></div>
 ===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. [https://doi.org/10.1111/j.1365-2516.2006.01342.x link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/17212724 PubMed]
 # '''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. [http://www.croh-online.com/article/S1040-8428%2806)00225-3 link to original article] [https://pubmed.ncbi.nlm.nih.gov/17236786/ PubMed]
 # '''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. [http://www.maneyonline.com/doi/full/10.1179/1607845414Y.0000000152 link to original article] [https://pubmed.ncbi.nlm.nih.gov/24611711 PubMed]
 =References=
 <references/>
 [[Category:Acquired coagulopathy regimens]]
 [[Category:Disease-specific pages]]
 [[Category:Autoimmune hematologic conditions]]
 [[Category:Bleeding disorders]]

Difference between revisions of "Acquired hemophilia A"

Revision as of 00:17, 1 March 2023

Contents

Guidelines

Acquired hemophilia A, all lines of therapy

Cyclophosphamide & Prednisolone

Regimen

Immunosuppressive therapy

References

Cyclophosphamide & Prednisone

Regimen variant #1

Immunosuppressive therapy

Regimen variant #2

Immunosuppressive therapy

Regimen variant #3

Preceding treatment

Immunosuppressive therapy

References

Prednisolone monotherapy

Regimen

Immunosuppressive therapy

References

Prednisone monotherapy

Regimen

Immunosuppressive therapy

Subsequent treatment

References

Rituximab monotherapy

Regimen variant #1, low-dose

Immunosuppressive therapy

Regimen variant #2, standard-dose

Immunosuppressive therapy

References

References

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