Difference between revisions of "Acquired hemophilia A"

From HemOnc.org - A Hematology Oncology Wiki
Jump to navigation Jump to search
m (Text replacement - "doi.org/full/10.1111" to "doi.org/10.1111")
m
 
(20 intermediate revisions by 3 users not shown)
Line 3: Line 3:
 
[[#top|Back to Top]]
 
[[#top|Back to Top]]
 
</div>
 
</div>
−
{{#lst:Section editor transclusions|heme}}
+
{{#lst:Editorial board transclusions|heme}}
 
{| class="wikitable" style="float:right; margin-right: 5px;"
 
{| class="wikitable" style="float:right; margin-right: 5px;"
 
|-
 
|-
−
|<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Regimen |limit=10000|format=sum}} [[Tutorial#Regimens|regimens]] on this page</b></font></div>
+
|<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Regimen |limit=10000|format=sum}} [[Tutorial#Regimens|regimens]] on this page</b></font></div>
−
<div style="background-color: #deebf6; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Variant |limit=10000|format=sum}} [[Tutorial#Variants|variants]] on this page</b></font></div>
+
<div style="background-color: #deebf6; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Variant |limit=10000|format=sum}} [[Tutorial#Variants|variants]] on this page</b></font></div>
 
|}
 
|}
 
{{TOC limit|limit=3}}
 
{{TOC limit|limit=3}}
−
 
+
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.<br>
−
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.
+
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. [https://doi.org/10.1182/blood-2006-06-029850 link to original article] [https://pubmed.ncbi.nlm.nih.gov/17047148/ PubMed]</ref>
 
=Guidelines=
 
=Guidelines=
−
*'''2009:''' [http://www.haematologica.org/content/94/4/566.short International Recommendations On The Diagnosis And Treatment Of Patients With Acquired Hemophilia A]
+
'''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. [https://doi.org/10.3324/haematol.2008.001743 International Recommendations On The Diagnosis And Treatment Of Patients With Acquired Hemophilia A] [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc2663620/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/19336751/ PubMed]
  
−
=Acquired hemophilia A, all lines of therapy=
+
=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}}==
 
==Cyclophosphamide & Prednisolone {{#subobject:2fd67|Regimen=1}}==
−
 
+
<div class="toccolours" style="background-color:#eeeeee">
 
===Regimen {{#subobject:84e0fd|Variant=1}}===
 
===Regimen {{#subobject:84e0fd|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 25: Line 26:
 
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
|-
 
|-
−
|[http://www.bloodjournal.org/content/109/5/1870.long Collins et al. 2007]
+
|[https://doi.org/10.1182/blood-2006-06-029850 Collins et al. 2007]
 
|style="background-color:#ffffbe"|Review
 
|style="background-color:#ffffbe"|Review
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Immunosuppressive therapy====
 
====Immunosuppressive therapy====
 
*[[Cyclophosphamide (Cytoxan)]] 1 to 2 mg/kg PO once per day
 
*[[Cyclophosphamide (Cytoxan)]] 1 to 2 mg/kg PO once per day
 
*[[Prednisolone (Millipred)]] 1 mg/kg PO once per day
 
*[[Prednisolone (Millipred)]] 1 mg/kg PO once per day
−
 
+
</div></div>
 
===References===
 
===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]
+
# '''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. [https://doi.org/10.1182/blood-2006-06-029850 link to original article] [https://pubmed.ncbi.nlm.nih.gov/17047148/ PubMed]
−
 
 
 
==Cyclophosphamide & Prednisone {{#subobject:e5cfe6|Regimen=1}}==
 
==Cyclophosphamide & Prednisone {{#subobject:e5cfe6|Regimen=1}}==
−
 
+
<div class="toccolours" style="background-color:#eeeeee">
 
===Regimen variant #1 {{#subobject:852fed|Variant=1}}===
 
===Regimen variant #1 {{#subobject:852fed|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 48: Line 49:
 
|}
 
|}
 
''Note: Per Shaffer et al. 1997, "the lower ends of these ranges became the standard therapy to minimize side effects"
 
''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====
 
====Immunosuppressive therapy====
 
*[[Cyclophosphamide (Cytoxan)]] 100 to 200 mg PO once per day
 
*[[Cyclophosphamide (Cytoxan)]] 100 to 200 mg PO once per day
 
*[[Prednisone (Sterapred)]] 50 to 80 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."
 
'''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}}===
 
===Regimen variant #2 {{#subobject:852fed|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 63: Line 65:
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Immunosuppressive therapy====
 
====Immunosuppressive therapy====
 
*[[Cyclophosphamide (Cytoxan)]] 100 mg PO once per day
 
*[[Cyclophosphamide (Cytoxan)]] 100 mg PO once per day
 
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
 
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
−
 
 
'''Continued until therapeutic response, then prednisone and cyclophosphamide were tapered slowly'''
 
'''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}}===
 
===Regimen variant #3 {{#subobject:404cf3|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 75: Line 78:
 
|-
 
|-
 
|[https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0038-1649664 Green et al. 1993]
 
|[https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0038-1649664 Green et al. 1993]
−
|style="background-color:#91cf61"|Randomized, <20 pts (E-esc)
+
|style="background-color:#91cf61"|Randomized, fewer than 20 pts (E-esc)
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#cbd5e8">
 
====Preceding treatment====
 
====Preceding treatment====
 
*[[#Prednisone_monotherapy|Prednisone]] x 21 d, with no response
 
*[[#Prednisone_monotherapy|Prednisone]] x 21 d, with no response
 +
</div>
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Immunosuppressive therapy====
 
====Immunosuppressive therapy====
 
*[[Cyclophosphamide (Cytoxan)]] 2 mg/kg (route not specified) once per day
 
*[[Cyclophosphamide (Cytoxan)]] 2 mg/kg (route not specified) once per day
 
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
 
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
−
 
+
</div></div>
 
===References===
 
===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]
+
# '''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. [https://doi.org/10.7326/0003-4819-95-5-575 link to original article] [https://pubmed.ncbi.nlm.nih.gov/6794408/ 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. [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]
+
# 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://doi.org/10.1055/s-0038-1649664 link to original article] [https://pubmed.ncbi.nlm.nih.gov/8128430/ 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. [https://doi.org/10.7326/0003-4819-127-3-199708010-00005 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/9245226 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. [https://doi.org/10.7326/0003-4819-127-3-199708010-00005 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/9245226/ 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. [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 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]
+
# '''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. [https://doi.org/10.1182/blood-2006-06-029850 link to original article] [https://pubmed.ncbi.nlm.nih.gov/17047148/ PubMed]
−
 
 
 
==Prednisolone monotherapy {{#subobject:78729f|Regimen=1}}==
 
==Prednisolone monotherapy {{#subobject:78729f|Regimen=1}}==
−
 
+
<div class="toccolours" style="background-color:#eeeeee">
 
===Regimen {{#subobject:6e2d2d|Variant=1}}===
 
===Regimen {{#subobject:6e2d2d|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 98: Line 103:
 
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
|-
 
|-
−
|[http://www.bloodjournal.org/content/109/5/1870.long Collins et al. 2007]
+
|[https://doi.org/10.1182/blood-2006-06-029850 Collins et al. 2007]
 
|style="background-color:#ffffbe"|Review
 
|style="background-color:#ffffbe"|Review
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Immunosuppressive therapy====
 
====Immunosuppressive therapy====
 
*[[Prednisolone (Millipred)]] 1 mg/kg PO once per day
 
*[[Prednisolone (Millipred)]] 1 mg/kg PO once per day
−
 
+
</div></div>
 
===References===
 
===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]
+
# '''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. [https://doi.org/10.1182/blood-2006-06-029850 link to original article] [https://pubmed.ncbi.nlm.nih.gov/17047148/ PubMed]
−
 
 
 
==Prednisone monotherapy {{#subobject:dcf925|Regimen=1}}==
 
==Prednisone monotherapy {{#subobject:dcf925|Regimen=1}}==
−
 
+
<div class="toccolours" style="background-color:#eeeeee">
 
===Regimen {{#subobject:c5ce2e|Variant=1}}===
 
===Regimen {{#subobject:c5ce2e|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 119: Line 124:
 
|-
 
|-
 
|[https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0038-1649664 Green et al. 1993]
 
|[https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0038-1649664 Green et al. 1993]
−
| style="background-color:#91cf61" |Non-randomized portion of RCT
+
| style="background-color:#91cf61" |Non-randomized part of RCT
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Immunosuppressive therapy====
 
====Immunosuppressive therapy====
 
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
 
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day
−
 
 
'''Continued for varying durations (see individual papers)'''
 
'''Continued for varying durations (see individual papers)'''
 +
</div>
 +
<div class="toccolours" style="background-color:#cbd5e7">
 
====Subsequent treatment====
 
====Subsequent treatment====
−
*Green et al. 1993, if no response after 21 days: [[#Cyclophosphamide_monotherapy_88|Cyclophosphamide]] versus [[#Cyclophosphamide_.26_Prednisone|Cyclophosphamide & Prednisone]]
+
*Green et al. 1993, if no response after 21 days: [[#Cyclophosphamide_monotherapy_888|Cyclophosphamide]] versus [[#Cyclophosphamide_.26_Prednisone|Cyclophosphamide & Prednisone]]
 +
</div></div>
 
===References===
 
===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]
+
# '''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]
−
# '''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. [https://doi.org/10.1111/j.1365-2141.1981.00635.x link to original article] [https://pubmed.ncbi.nlm.nih.gov/6791677 PubMed] content property of [http://hemonc.org HemOnc.org]
+
# '''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. [https://doi.org/10.1111/j.1365-2141.1981.00635.x link to original article] [https://pubmed.ncbi.nlm.nih.gov/6791677/ PubMed] content property of [https://hemonc.org 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. [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]
+
# 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]
 
# '''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}}==
 
==Rituximab monotherapy {{#subobject:6d79c2|Regimen=1}}==
−
 
+
<div class="toccolours" style="background-color:#eeeeee">
 
===Regimen variant #1, low-dose {{#subobject:3f2f14|Variant=1}}===
 
===Regimen variant #1, low-dose {{#subobject:3f2f14|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 141: Line 148:
 
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
|-
 
|-
−
|[http://www.maneyonline.com/doi/full/10.1179/1607845414Y.0000000152 Yao et al. 2014]
+
|[https://doi.org/10.1179/1607845414y.0000000152 Yao et al. 2014]
 
|style="background-color:#ffffbe"|Case report
 
|style="background-color:#ffffbe"|Case report
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Immunosuppressive therapy====
 
====Immunosuppressive therapy====
 
*[[Rituximab (Rituxan)]] 100 mg/m<sup>2</sup> IV once per day on days 1, 8, 15, 22
 
*[[Rituximab (Rituxan)]] 100 mg/m<sup>2</sup> IV once per day on days 1, 8, 15, 22
−
 
 
'''28-day course'''
 
'''28-day course'''
−
 
+
</div></div><br>
 +
<div class="toccolours" style="background-color:#eeeeee">
 
===Regimen variant #2, standard-dose {{#subobject:3f9925|Variant=1}}===
 
===Regimen variant #2, standard-dose {{#subobject:3f9925|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
Line 159: Line 167:
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Immunosuppressive therapy====
 
====Immunosuppressive therapy====
 
*[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once per day on days 1, 8, 15, 22
 
*[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once per day on days 1, 8, 15, 22
−
 
 
'''28-day course'''
 
'''28-day course'''
−
 
+
</div></div>
 
===References===
 
===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]
+
# '''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]
+
# '''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. [https://doi.org/10.1016/j.critrevonc.2006.11.004 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]
+
# '''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. [https://doi.org/10.1179/1607845414y.0000000152 link to original article] [https://pubmed.ncbi.nlm.nih.gov/24611711/ PubMed]
−
 
 
 
=References=
 
=References=
 
<references/>
 
<references/>
−
 
+
[[Category:Acquired hemophilia A regimens]]
−
[[Category:Acquired coagulopathy regimens]]
 
 
[[Category:Disease-specific pages]]
 
[[Category:Disease-specific pages]]
 
[[Category:Autoimmune hematologic conditions]]
 
[[Category:Autoimmune hematologic conditions]]
 
[[Category:Bleeding disorders]]
 
[[Category:Bleeding disorders]]

Latest revision as of 20:09, 27 June 2024

Back to Top

Section editor
Tillman Benjamin-2.jpg
 Benjamin Tillman, MD
Vanderbilt University
Nashville, TN, USA
LinkedIn
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 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/year[1]

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

All lines of therapy

Cyclophosphamide & Prednisolone

Regimen

Study Evidence
Collins et al. 2007 Review

Immunosuppressive therapy

References

  1. 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

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

Continued until therapeutic response, then prednisone and cyclophosphamide were tapered slowly


Regimen variant #3

Study Evidence
Green et al. 1993 Randomized, fewer than 20 pts (E-esc)

Preceding treatment

Immunosuppressive therapy

References

  1. 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
  2. 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
  3. 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
  4. 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
  5. 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

References

  1. 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 part of RCT

Immunosuppressive therapy

Continued for varying durations (see individual papers)

Subsequent treatment

References

  1. 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
  2. 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
  3. 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
  4. 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

28-day course


Regimen variant #2, standard-dose

Study Evidence
Field et al. 2006 Case series

Immunosuppressive therapy

28-day course

References

  1. 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
  2. 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
  3. 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

  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
Retrieved from "https://hemonc.org/w/index.php?title=Acquired_hemophilia_A&oldid=87075"