单中心22例获得性血友病的回顾性临床研究

OBJECTIVE: To investigate the feature of underlying disorders, clinical symptoms, diagnosis and treatment strategies of patients with acquired hemophilia(AH). METHODS: The clinical data and laboratory tests results of 22 patients with AH from March 2010 to June 2014 were retrospectively analyzed. RESULTS: A total of 22 patients with AH were enrolled in our study, including 20 patients diagnosed as acquired hemophilia A (AHA) and 2 as acquired hemophilia B (AHB). Among the AHA patients, there were 10 males and 10 females with the median age of 37.5 (range, 2–95) years old. The median activity of FⅧ (FⅧ∶C) of the 20 AHA patients was 1.9% (0.5%–39.0%). Soft tissue hematoma (80.0%) and subcutaneous bleeding (75.0%) were the most common clinical symptoms. Two male children were diagnosed as AHB (age 1 and 3 years old, respectively) with mild bleeding symptoms, and the activities of FⅨ (FⅨ∶C) were 5.0% and 16.0%, respectively. In addition, an underlying disorder was found in 7 patients (31.8%). In laboratory testing, all patients had prolonged APTT, normal PT, decreased F Ⅷ∶C or F Ⅸ∶C, positive antibody screening test or antibody titer (2–32 BU), and negative for lupus anticoagulant and anticardiolipin antibody. Nineteen out of 20 patients were treated with blood products to stop acute bleeding episodes. Corticosteroid alone was applied to 7 patients, corticosteroid combined with other immunosuppressive agents to 11 patients, rituximab to 3 patients. Nineteen patients responded well to hemostatic treatment, except 1 patient who died of fatal bleeding. The FⅧ∶C of 8 patients increased to a normal level with the median time of 42.5 (21–145) days. After treatment, the activity of FⅨ∶C of the 2 AHB patients achieved 35% and 24% in 48 and 60 days, respectively. CONCLUSION: Acquired hemophilia is not an uncommon disease in clinical practices, which can occur in people of all ages. AH is a bleeding disorder with heterogeneous characteristics. Compared with adult, the clinical symptoms of children patients were mild, which lead to underdiagnosis.