Systemic lupus erythematosus with acquired hemophilia A: A case report and literature review / 中南大学学报(医学版)
Journal of Central South University(Medical Sciences)
;
(12): 789-794, 2023.
Artículo
en Inglés
| WPRIM
| ID: wpr-982349
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.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Factor VIII
/
Rituximab
/
Glucocorticoides
/
Hemofilia A
/
Hemorragia
/
Lupus Eritematoso Sistémico
Límite:
Adulto
/
Femenino
/
Humanos
Idioma:
Inglés
Revista:
Journal of Central South University(Medical Sciences)
Año:
2023
Tipo del documento:
Artículo
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