title: Acquired hemophilia A that developed during the induction of hemodialysis: the use of double-filtration plasmapheresis .
abstract: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII). AHA onset during the induction of dialysis is extremely rare, and the management of blood access is difficult. We present a case of AHA that developed during induction of dialysis and treatment with double filtration plasmapheresis (DFPP). An 86-year-old man with chronic kidney disease was admitted to our hospital with multiple subcutaneous hemorrhages. Because of his prolonged activated partial thromboplastin time (aPTT) and high titer of inhibitors to FVIII, he was diagnosed with AHA, and prednisolone treatment was started. After 3 weeks of steroid therapy, his renal function deteriorated, and dialysis was needed. We performed femoral catheter placement under administration of recombinant activated factor VII (rFVIIa) to prevent bleeding. The patient developed catheter-related bloodstream infection and needed arteriovenous fistula (AVF) immediately. After 4 DFPP sessions, his hemostasis recovered to normal. AVF placement did not cause any complication, and he could safely undergo maintenance hemodialysis. Clinicians should suspect AHA in end-stage renal disease patients with acute onset of bleeding and an unexplained prolonged aPTT. DFPP is useful in patients with AHA that develops during induction of dialysis and requires surgical treatment. .
authors.forename: Aya
authors.initials: A
authors.lastname: Imafuku
authors.forename: Naoki
authors.initials: N
authors.lastname: Sawa
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
mesh_terms: D000369:Aged, 80 and over; D005169:Factor VIII / Q000627:therapeutic use*; D005938:Glucocorticoids / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy*; D008297:Male; D010956:Plasmapheresis / Q000379:methods*; D011239:Prednisolone / Q000627:therapeutic use; D006435:Renal Dialysis / Q000009:adverse effects*; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D005169:Factor VIII; D011239:Prednisolone