A single-center retrospective clinical study of 7 cases of acquired hemophilia A

Objective This study aims to explore the etiology, clinical manifestations, diagnosis and treatment, and prognosis of acquired hemophilia A (AHA), and to improve the clinical understanding, diagnosis, and treatment of AHA. Methods A retrospective analysis was conducted on 7 cases of AHA diagnosed at our hospital from January 2013 to December 2023. Relevant literature was searched in PubMed, CNKI, and Wanfang databases using the terms “Acquired hemophilia A”. A comparative analysis was performed on general data, etiology, diagnosis time, clinical manifestations, treatment, and prognosis. Results All 7 AHA patients were female, with a median age of 44.1 years (range 26-68 years). One case was secondary to hepatitis B virus infection; one was secondary to pregnancy; one developed after gastrointestinal surgery; and one was secondary to Sjögren’s syndrome. Skin ecchymosis was the most common clinical manifestation of AHA（7/7）, with increased activated partial thromboplastin time (APTT), decreased coagulation factor Ⅷ(FⅧ) activity (FⅧ:C), and increased FⅧ antibody (FⅧ: Ab). Six patients received hemostatic therapy, and all patients received antibody clearance therapy. The median follow-up time was 391 days (28-777 days), with one patient lost to follow-up. Four patients achieved complete response (CR), with a median time to CR of 40 days (27-48 days). One patient achieved partial response (PR), and no recurrence occurred. One patient had no response (NR) and no new clinical bleeding during the follow-up period. The literature review found that AHA can occur in both men and women, with a predominance of women of childbearing age. 56.8% of the patients had idiopathic AHA, while 11.4%, 11.1%, and 6.7% patients of AHA associated with tumors, autoimmune diseases, and pregnancy, respectively. The male to female ratio is 1.18:1, with median ages of 65.6 years and 55.8 years, respectively. Bypass pathway drugs were the first choice for hemostatic treatment, and antibody clearance therapy was mostly based on glucocorticoids, cyclophosphamide, and rituximab. Conclusions AHA can be secondary to tumors, autoimmune diseases, infections, pregnancy, etc. Patients may have skin bruising; The use of bypass routes for hemostasis and immunosuppressive therapy to clear antibodies results in a good prognosis for most patients.