Management of Acquired Hemophilia A in Elderly Patients

Table 1

Clinical features of 6 elderly cases with AHA.

	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6
Age/gender	91/F	93/M	92/F	90/M	80/M	62/F
Body weight (kg)	29.1	48.1	43.0	41.1	53.0	50.9
Symptoms	Subcutaneous, intramuscular hemorrhages at extremities	Soft tissue hemorrhages, hematuria	Generalized subcutaneous hemorrhages	Intragluteal hemorrhage	Soft tissue hemorrhages at forearm and legs	Hemorrhagic shock after tooth extraction
Comorbidities	LAC positive	After influenza B	After surgery	Heart failure	CNL	Liver cirrhosis
Hb (g/dL)	4.6	7.5	7.3	6.4	8.0	4.6
APTT (sec)	93.3	73.1	55.2	69.2	69.0	>100
FVIII activity (%)	<1.0	<1.0	3.0	<1.0	<1.0	<1.0
Anti-FVIII inhibitor (BU/mL)	110.6	8.8	10.0	240	190	11.9
Time to Dx and hemostasis (days)	10	5	7	0^†	3	8
Agents for hemostasis (total doses)	rFVIIa (36)	rFVIIa (6)	rFVIIa (2)	aPCC (3)	aPCC/rFVIIa (16)/(4)	FFP/PE, PSL
Time from hemostasis to IS agents (days)	23	6	0	0	11	20
Agents for IS	PSL/CPA	PSL	PSL	PSL	PSL/Ritux	CSA/PSL
Time (weeks) to restoration of FVIII activity (>50%)	7	2	7	Not done	26	10
Undetectable inhibitor	7	2	7	Not done	26	7
Outcome
CR/PR	CR	CR	CR	NR	CR	CR
Follow-up (years)	<1	<1	>2	<1	>3	1
Relapse/reactivation	No	No	Yes	NA	Yes	No

Abbreviations: Dx = diagnosis; CNL = chronic neutrophilic leukemia; LAC = lupus anticoagulant; aPCC = activated prothrombin complex concentrate; rFVII = recombinant factor VII; IS = immunosuppression; PSL = prednisolone; CPA = cyclophosphamide; Ritux = rituximab; CSA = cyclosporine A; FFP = fresh frozen plasma; PE = plasma exchange; CR = complete remission; NR = no remission; NA = not applicable; relapse occurred 2 years after CR; reactivation at the 7^th week of treatment; died of cirrhosis-related hepatic failure; ^†AHA previously diagnosed.