Acquired Hemophilia A Combined with Systemic Lupus Erythematosus: A Case Report and Literature Review.
10.4078/jrd.2017.24.5.309
- Author:
Juyoung YOU
1
;
Hojae KIM
;
Jin Su PARK
;
Myung Hee CHANG
;
Chan Hee LEE
Author Information
1. Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Hemophilia A;
Acquired;
Systemic lupus erythematosus
- MeSH:
Aged, 80 and over;
Anemia;
Autoantibodies;
Buttocks;
Contusions;
Ecchymosis;
Factor VIII;
Female;
Hematuria;
Hemophilia A*;
Hemorrhage;
Hemorrhagic Disorders;
Humans;
Lower Extremity;
Lupus Coagulation Inhibitor;
Lupus Erythematosus, Systemic*;
Partial Thromboplastin Time;
Platelet Count;
Prednisolone;
Prothrombin Time;
Ulcer
- From:Journal of Rheumatic Diseases
2017;24(5):309-312
- CountryRepublic of Korea
- Language:English
-
Abstract:
Acquired hemophilia A (AHA) is a rare hemorrhagic disorder caused by autoantibodies against factor VIII (FVIII). An 80-year-old woman presented multiple bruises on her upper and lower extremities, along with gross hematuria. Extensive ecchymosis and swelling were observed on the buttocks. She had anemia and normal platelet count. The initial coagulation results showed prolonged activated partial thromboplastin time (aPTT, 68.5 seconds) and normal prothrombin time. According to the mixing test, we observed a decreased FVIII activity (2%), increased factor VIII inhibitor (FVIII-I) titer (74.4 BU), and negative lupus anticoagulant. AHA was diagnosed based on late onset bleeding and increased FVIII-I titer. Additionally, she met the criteria for systemic lupus erythematosus (oral ulcer, photosensitivity, renal disorder, and positivity for antinuclear and anti-β2-glycoprotein-I antibodies). She was started on oral prednisolone for FVIII-I eradication. Post-treatment, her bleeding tendency, aPTT (47.3 seconds), and FVIII-I titer decreased (1.24 BU), and FVIII activity increased (10%).
