Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature.
Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE) is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced factor VIII activity and a high titer of FVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, factor VIII inhibitor assay was negative.
Rheumatic Disease Research Center, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
Zahra
Z
Rezaieyazdi
Davood
D
Sharifi-Doloui
Kamila
K
Hashemzadeh
Abbas
A
Shirdel
Hassan
H
Mansouritorghabeh
D000328:Adult; D000368:Aged; D001780:Blood Coagulation Tests; D005169:Factor VIII / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000150:complications*; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D055815:Young Adult
D005169:Factor VIII