Difference between revisions of "Acquired hemophilia A"
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− | + | {{#lst:Editorial board transclusions|heme}} | |
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{| class="wikitable" style="float:right; margin-right: 5px;" | {| class="wikitable" style="float:right; margin-right: 5px;" | ||
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− | |<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] | + | |<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}}]] | + | <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> |
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{{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:''' [ | + | '''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] | ||
− | = | + | =All lines of therapy= |
− | |||
==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: | + | {| class="wikitable" style="width: 40%; text-align:center;" |
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[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. [ | + | # '''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}}=== | |
− | + | {| class="wikitable" style="width: 40%; text-align:center;" | |
− | |||
− | === | ||
− | {| class="wikitable" style="width: | ||
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.7326/0003-4819-127-3-199708010-00005 Shaffer et al. 1997] |
|style="background-color:#ffffbe"|Case report | |style="background-color:#ffffbe"|Case report | ||
|- | |- | ||
|} | |} | ||
+ | ''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." | |
− | + | </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;" |
− | {| class="wikitable" style="width: | ||
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[https:// | + | |[https://doi.org/10.1002/%28SICI)1096-8652%28199901)60:1%3C70::AID-AJH12%3E3.0.CO;2-D Bayer et al. 1999] |
|style="background-color:#ffffbe"|Case report | |style="background-color:#ffffbe"|Case report | ||
|- | |- | ||
|} | |} | ||
+ | <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''' | |
− | ''' | + | </div></div><br> |
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
− | === | + | ===Regimen variant #3 {{#subobject:404cf3|Variant=1}}=== |
− | {| class="wikitable" style="width: | + | {| class="wikitable" style="width: 40%; text-align:center;" |
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
|[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, | + | |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. [ | + | # '''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:// | + | # 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. [ | + | # '''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:// | + | # '''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. [ | + | # '''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: | + | {| class="wikitable" style="width: 40%; text-align:center;" |
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[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. [ | + | # '''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: | + | {| class="wikitable" style="width: 40%; text-align:center;" |
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[https:// | + | |[https://pubmed.ncbi.nlm.nih.gov/6792737 Green et al. 1981] |
|style="background-color:#ffffbe"|Case report, review | |style="background-color:#ffffbe"|Case report, review | ||
|- | |- | ||
|[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 | + | | 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)''' | |
− | ''' | + | </div> |
+ | <div class="toccolours" style="background-color:#cbd5e7"> | ||
====Subsequent treatment==== | ====Subsequent treatment==== | ||
− | *Green et al. 1993, if no response after 21 days: 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:// | + | # '''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:// | + | # '''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:// | + | # 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:// | + | # '''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}}=== | |
− | + | {| class="wikitable" style="width: 40%; text-align:center;" | |
− | |||
− | === | ||
− | {| class="wikitable" style="width: | ||
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[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"> |
− | {| class="wikitable" style="width: | + | ===Regimen variant #2, standard-dose {{#subobject:3f9925|Variant=1}}=== |
+ | {| class="wikitable" style="width: 40%; text-align:center;" | ||
!style="width: 25%"|Study | !style="width: 25%"|Study | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[https:// | + | |[https://doi.org/10.1111/j.1365-2516.2006.01342.x Field et al. 2006] |
|style="background-color:#ffffbe"|Case series | |style="background-color:#ffffbe"|Case series | ||
|- | |- | ||
|} | |} | ||
+ | <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:// | + | # '''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. [ | + | # '''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. [ | + | # '''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 | ||
[[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
Section editor | |
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Benjamin Tillman, MD Vanderbilt University Nashville, TN, USA |
5 regimens on this page
7 variants on this page
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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
- 2009: Huth-Kühne et al. International Recommendations On The Diagnosis And Treatment Of Patients With Acquired Hemophilia A link to PMC article PubMed
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
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, 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
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 part 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