Acquired Hemophilia A: Clinical Features, Surgery and Treatment of 34 Cases, and Experience of Using Recombinant Factor VIIa

Acquired hemophilia A is rare, but life-threatening disorder caused by autoantibody against factor VIII. As it is useful to gather more data on epidemiology, clinical pictures and therapy of it, we evaluated relevant medical findings in 34 acquired hemophiliacs from Dec 1999 to Dec 2007. Eight patients (23.5%) had low titers (<10 Bethesda Unit BU) and 26 patients (76.5%) had high titers of inhibitors (>10 BU). The mean of inhibitors was 548.38 +/- 359.27 SD BU. The most common hemorrhagic symptoms were hematoma 21 (33.33%), ecchymosis 16 (25.39%), hemarthrosis 8 (12.69%), hematuria 6 (9.52%), menorrhagia 4 (6.34%), compartment syndrome 3 episodes (4.76%). The eliminator therapies were recruited according to titers of inhibitor and types of bleeding and it's results were 27 efficient treatments (79.4%), 5 partial efficient treatment (14.7%) and two treatments inefficient (5.9%). Elimination therapy using steroid alone or with combination can terminate complete remission in most cases.