[A single-center clinical study of 22 patients with acquired hemophilia]

Abstract

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 FVIII:C or FIX: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.