Systemic lupus erythematosus with acquired hemophilia A: A case report and literature review.
10.11817/j.issn.1672-7347.2023.220440
- Author:
Mei YE
1
;
Ruiying DENG
2
;
Fengcai SHEN
2
;
Zhiduo HOU
3
;
Ling LIN
2
Author Information
1. Department of Rheumatology and Immunology, First Affiliated Hospital of Shantou University Medical College, Shantou Guangdong 515041. yzaleaf@163.com.
2. Department of Rheumatology and Immunology, First Affiliated Hospital of Shantou University Medical College, Shantou Guangdong 515041.
3. Department of Rheumatology and Immunology, First Affiliated Hospital of Shantou University Medical College, Shantou Guangdong 515041. houzhiduo@foxmail.com.
- Publication Type:Journal Article
- Keywords:
acquired hemophilia;
rituximab;
systemic lupus erythematosus
- MeSH:
Female;
Humans;
Adult;
Hemophilia A/therapy*;
Rituximab;
Glucocorticoids;
Factor VIII;
Lupus Erythematosus, Systemic/complications*;
Hemorrhage/complications*
- From:
Journal of Central South University(Medical Sciences)
2023;48(5):789-794
- CountryChina
- Language:English
-
Abstract:
Systemic lupus erythematosus (SLE) complicated with acquired hemophilia A (AHA) is a rare condition with frequently delayed diagnosis and a high mortality rate, so it is necessary to strengthen the understanding of this disease. In this study, the characteristics and treatment in 1 case of SLE complicated by AHA is reported and analyzed, and a literature review is conducted. The patient was a 29-year-old young female with a 10-year history of SLE, the main clinical manifestation was severe abdominal bleeding. Laboratory tests revealed that the activated partial thromboplastin time (APTT) was notably prolonged (118.20 s), and the coagulation factor VIII activity (FVIII꞉C) was extremely decreased (0.20%) with high-titer of factor VIII (FVIII) inhibitor (31.2 BU/mL). After treating with high-dose glucocorticoid, immunoglobulin, cyclophosphamide, rituximab, blood transfusion, and intravenous infusion of human coagulation FVIII, the coagulation function and coagulation FVIII꞉C were improved, and FVIII inhibitor was negative without serious adverse reactions. During the next 5-year follow-up, the patient's condition was stable and no bleeding occurred. In the case of coagulation dysfunction in SLE, especially with isolated APTT prolongation, AHA should be screened. When the therapeutic effects of glucocorticoid combined with immunosuppressants are not desirable, rituximab could be introduced.
