J Rheum Dis.  2017 Oct;24(5):309-312. 10.4078/jrd.2017.24.5.309.

Acquired Hemophilia A Combined with Systemic Lupus Erythematosus: A Case Report and Literature Review

Affiliations
  • 1Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
  • 2Department of Internal Medicine, National Health Insurance Service Ilsan Hospital, Goyang, Korea. chanheell@daum.net

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%).

Keyword

Hemophilia A; Acquired; Systemic lupus erythematosus

MeSH Terms

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
Autoantibodies
Factor VIII
Lupus Coagulation Inhibitor
Prednisolone
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