Combined prednisolone and intravenous immunoglobulin treatment for acquired factor VIII inhibitors: a 2-year review

Acquired inhibitors to factor VIII (FVIII) are rare, but life-threatening i n up to 22% of cases. The optimal therapy for suppression of these inhibito rs remains unclear. Prednisolone is the mainstay of therapy, producing resp onses in approximately 30% of cases. Intravenous immunoglobulin (IVIg) has a similar response rate, but a more rapid effect. We report the results of prednisolone 1 mg kg(-1) combined with IVIg 2 g kg(-1) in divided doses as first-line therapy in seven consecutive patients with acquired FVIII inhibi tors. All patients were bleeding at the time of diagnosis with prolonged ac tivated partial thromboplastin time. There were four complete responses, on e partial response, one nonresponse and one with an inadequate follow-up fo r assessment of response, giving an overall response rate of 71%. In all co mplete responders the inhibitor declined rapidly and was undetectable by da y 21 from start of treatment. Therapy was well tolerated and responses have been maintained off treatment for 2-8 months. This is a safe, well-tolerat ed rapidly acting regimen with good response rates.