Acquired hemophilia A in patients with systemic lupus erythematosus:report of a case and review of literature / 中华风湿病学杂志

ABSTRACT

Objective To summarize the manifestation and treatment of acquired hemophilia A in pa- tients with systemic lupus erythematosus(SLE).Method A case was investigated retrospectively and the lit- erature was reviewed.Results A 25-year-old woman with a 5 year history of SLE was admitted to hospital due to abdominal pain.She was diagnosed with acquired factorⅧinhibitor deficiency based on a prolonged activated partial-thromboplastin time(APTT,135.3 s),reduced factorⅧactivity(0.9%)and factorⅧin- hibitor(26.1 BU/ml).Sonography and magnetic nuclear resonance of the abdomen confirmed the presence of a retro-uterine hematoma.The patient was initially treated with intravenous pulse and oral corticosteroids,factorⅧplasma concentrated and intravenous immunoglobulin.Clinical and biological improvement was promptly obtained.Conclusions Attention should be paid to the association between SLE and acquired hemophilia A. Combination therapy may be recommended as initial therapy for the management of acquired hemophilia A in patients with SLE.But no standardized treatment can be recommended at present.