Factor VIII inhibitor - Revision history

Danbot: Strip excess bold

2026-03-22T09:34:45Z

Strip excess bold

Danbot: Expand: acquired hemophilia context, mixing study pearl, bypassing agents for acute bleeding

2026-03-21T01:41:01Z

Expand: acquired hemophilia context, mixing study pearl, bypassing agents for acute bleeding

← Older revision		Revision as of 01:41, 21 March 2026
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	==Background==		==Background==
			*Autoantibodies (IgG) directed against Factor VIII, causing an acquired coagulopathy
			*Also called '''acquired hemophilia A''' — distinct from congenital [[hemophilia]]
			*Rare but potentially life-threatening; mortality 8-22%
			*'''Associations:''' autoimmune diseases, malignancy, pregnancy/postpartum, medications (penicillin, sulfonamides), idiopathic (~50%)
			*Most common in elderly patients (median age 60-70)

	==Clinical Features==		==Clinical Features==
			*Spontaneous soft tissue bleeding, ecchymoses, hematomas (often extensive)
			*Mucosal bleeding, GI bleeding, [[hematuria]]
			*'''Unlike congenital hemophilia:''' hemarthrosis is uncommon
			*May present with life-threatening hemorrhage without prior bleeding history

	==Differential Diagnosis==		==Differential Diagnosis==
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	==Evaluation==		==Evaluation==
	*PTT does ~~not~~ correct ~~after mixing~~		*'''Isolated prolonged PTT''' with normal PT and platelet count
			*'''Mixing study:''' PTT does NOT correct (distinguishes inhibitor from factor deficiency)
			*Factor VIII activity level markedly reduced
			*'''Bethesda assay:''' Quantifies inhibitor titer (Bethesda Units)
			*CBC, fibrinogen, DIC panel to assess for concurrent coagulopathy

	==Management==		==Management==
	*~~Bleeding~~		*'''Acute bleeding:'''
	**~~Give high~~ dose Factor ~~VII, prothrombin, or recombinant factor VIIa~~		**'''Bypassing agents''' (first-line for significant hemorrhage):
			***Recombinant Factor VIIa (NovoSeven) 90 mcg/kg IV q2-3h
			***Activated prothrombin complex concentrate (FEIBA) 50-100 units/kg IV q8-12h
			**High-dose Factor VIII concentrate may be tried but often ineffective if high-titer inhibitor
			**[[Desmopressin]] (DDAVP) for low-titer inhibitors only
			*'''Inhibitor eradication''' (in consultation with hematology):
			**Immunosuppression: corticosteroids ± cyclophosphamide
			**Rituximab for refractory cases
			*Avoid procedures and IM injections; hold anticoagulants

	==Disposition==		==Disposition==
			*Admit all patients — hematology consult urgently
			*ICU for significant hemorrhage

	==See Also==		==See Also==
			*[[Hemophilia]]
	*[[Coagulopathy (Main)]]		*[[Coagulopathy (Main)]]
			*[[DIC]]

	==References==		==References==
	<references/>		<references/>

	[[Category:Heme/Onc]]		[[Category:Heme/Onc]]

Mholtz: Text replacement - "==References== " to "==References== "

2017-07-24T01:13:04Z

Text replacement - "==References== " to "==References== <references/> "

← Older revision		Revision as of 01:13, 24 July 2017
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	==References==		==References==
			<references/>
	[[Category:Heme/Onc]]		[[Category:Heme/Onc]]

Neil.m.young: Text replacement - "==Diagnosis==" to "==Evaluation=="

2016-07-23T01:57:37Z

Text replacement - "==Diagnosis==" to "==Evaluation=="

← Older revision		Revision as of 01:57, 23 July 2016
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	{{Increased bleeding DDX}}		{{Increased bleeding DDX}}

	==~~Diagnosis~~==		==Evaluation==
	*PTT does not correct after mixing		*PTT does not correct after mixing

Rossdonaldson1: Rossdonaldson1 moved page Factor VIII Inhibitor to Factor VIII inhibitor

2015-06-10T12:59:55Z

Rossdonaldson1 moved page Factor VIII Inhibitor to Factor VIII inhibitor

← Older revision	Revision as of 12:59, 10 June 2015
(No difference)

Rossdonaldson1: /* Management */

2015-06-10T12:59:39Z

Management

← Older revision		Revision as of 12:59, 10 June 2015
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	==Management==		==Management==
	*Bleeding		*Bleeding
	**~~High~~ dose Factor VII, prothrombin, or recombinant factor VIIa		**Give high dose Factor VII, prothrombin, or recombinant factor VIIa

	==Disposition==		==Disposition==

Rossdonaldson1 at 12:59, 10 June 2015

2015-06-10T12:59:27Z

← Older revision		Revision as of 12:59, 10 June 2015
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	==Background==		==Background==
	~~#(PTT does not correct after mixing)~~

	==Management ~~with Bleeding~~==		==Clinical Features==
	~~#high~~ dose Factor VII, prothrombin, or recombinant factor VIIa
			==Differential Diagnosis==
			{{Increased bleeding DDX}}

			==Diagnosis==
			*PTT does not correct after mixing

			==Management==
			*Bleeding
			**High dose Factor VII, prothrombin, or recombinant factor VIIa

			==Disposition==

	==See Also==		==See Also==
	*[[Coagulopathy (Main)]]		*[[Coagulopathy (Main)]]

	==~~Source~~==		==References==
	*Tintinalli
	*UpToDate

	[[Category:Heme/Onc]]		[[Category:Heme/Onc]]

Rossdonaldson1: Created page with "==Background== #(PTT does not correct after mixing) ==Management with Bleeding== #high dose Factor VII, prothrombin, or recombinant factor VIIa ==See Also== *Coagulopathy ..."

2014-01-07T13:24:12Z

Created page with "==Background== #(PTT does not correct after mixing) ==Management with Bleeding== #high dose Factor VII, prothrombin, or recombinant factor VIIa ==See Also== *Coagulopathy ..."

New page

==Background==
#(PTT does not correct after mixing)

==Management with Bleeding==
#high dose Factor VII, prothrombin, or recombinant factor VIIa

==See Also==
*[[Coagulopathy (Main)]]

==Source==
*Tintinalli
*UpToDate

[[Category:Heme/Onc]]

← Older revision		Revision as of 09:34, 22 March 2026
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	==Background==		==Background==
	*Autoantibodies (IgG) directed against Factor VIII, causing an acquired coagulopathy		*Autoantibodies (IgG) directed against Factor VIII, causing an acquired coagulopathy
	*Also called ~~'''~~acquired hemophilia A~~'''~~ — distinct from congenital [[hemophilia]]		*Also called acquired hemophilia A — distinct from congenital [[hemophilia]]
	*Rare but potentially life-threatening; mortality 8-22%		*Rare but potentially life-threatening; mortality 8-22%
	*~~'''~~Associations:~~'''~~ autoimmune diseases, malignancy, pregnancy/postpartum, medications (penicillin, sulfonamides), idiopathic (~50%)		*Associations: autoimmune diseases, malignancy, pregnancy/postpartum, medications (penicillin, sulfonamides), idiopathic (~50%)
	*Most common in elderly patients (median age 60-70)		*Most common in elderly patients (median age 60-70)

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	*Spontaneous soft tissue bleeding, ecchymoses, hematomas (often extensive)		*Spontaneous soft tissue bleeding, ecchymoses, hematomas (often extensive)
	*Mucosal bleeding, GI bleeding, [[hematuria]]		*Mucosal bleeding, GI bleeding, [[hematuria]]
	*~~'''~~Unlike congenital hemophilia:~~'''~~ hemarthrosis is uncommon		*Unlike congenital hemophilia: hemarthrosis is uncommon
	*May present with life-threatening hemorrhage without prior bleeding history		*May present with life-threatening hemorrhage without prior bleeding history

Line 16:		Line 16:

	==Evaluation==		==Evaluation==
	*~~'''~~Isolated prolonged PTT~~'''~~ with normal PT and platelet count		*Isolated prolonged PTT with normal PT and platelet count
	*~~'''~~Mixing study:~~'''~~ PTT does NOT correct (distinguishes inhibitor from factor deficiency)		*Mixing study: PTT does NOT correct (distinguishes inhibitor from factor deficiency)
	*Factor VIII activity level markedly reduced		*Factor VIII activity level markedly reduced
	*~~'''~~Bethesda assay:~~'''~~ Quantifies inhibitor titer (Bethesda Units)		*Bethesda assay: Quantifies inhibitor titer (Bethesda Units)
	*CBC, fibrinogen, DIC panel to assess for concurrent coagulopathy		*CBC, fibrinogen, DIC panel to assess for concurrent coagulopathy

	==Management==		==Management==
	*~~'''~~Acute bleeding:~~'''~~		*Acute bleeding:
	**~~'''~~Bypassing agents~~'''~~ (first-line for significant hemorrhage):		**Bypassing agents (first-line for significant hemorrhage):
	***Recombinant Factor VIIa (NovoSeven) 90 mcg/kg IV q2-3h		***Recombinant Factor VIIa (NovoSeven) 90 mcg/kg IV q2-3h
	***Activated prothrombin complex concentrate (FEIBA) 50-100 units/kg IV q8-12h		***Activated prothrombin complex concentrate (FEIBA) 50-100 units/kg IV q8-12h
	**High-dose Factor VIII concentrate may be tried but often ineffective if high-titer inhibitor		**High-dose Factor VIII concentrate may be tried but often ineffective if high-titer inhibitor
	**[[Desmopressin]] (DDAVP) for low-titer inhibitors only		**[[Desmopressin]] (DDAVP) for low-titer inhibitors only
	*~~'''~~Inhibitor eradication~~'''~~ (in consultation with hematology):		*Inhibitor eradication (in consultation with hematology):
	**Immunosuppression: corticosteroids ± cyclophosphamide		**Immunosuppression: corticosteroids ± cyclophosphamide
	**Rituximab for refractory cases		**Rituximab for refractory cases