title: Extracorporeal Treatment for the Acute und Long-Term Outcome of Patients with Life-Threatening Acquired Hemophilia.
abstract: OBJECTIVES: In acquired hemophilia (AH), autoantibodies (inhibitors) impede blood coagulation factors leading to severe bleedings. Cornerstones of a successful treatment are the control of bleeding and an eradication of autoantibodies. The present study is an update of our previous documentation of the treatment of high-titer AH patients with severe life-threatening bleeding undergoing the modified Bonn-Malmö-Protocol (MBMP).  METHODS: 64 AH patients were treated by a standard combination protocol (MBMP) consisting of antibody depletion through immunoadsorption, i.v. immunoglobulin, immunosuppression, and high-dose FVIII substitution. They underwent a long-term follow-up.  RESULTS: Primary study endpoints loss of detection of the activity of the inhibitor and FVIII recovery ? 5% were reached in a median time of 3 days (95% CI: 2.6-3.4 days), the median time of FVIII substitution was 13 days (95% CI 10.6-15.3 days), and the median time of immunoadsorption was 16 days (95% CI 13-18.9 days). In 5 patients the AH occurred as paraneoplastic syndrome, and partial remission was achieved. Relapses without bleeding event occurred only in second-line MBMP. Those responded excellently to short time treatment. Overall patients remained in remission over a median follow-up time of 8 years. Conclusion: Except for paraneoplastic AH, MBMP-treated patients have a remarkable prognosis which is confirmed by long-term follow-up with a complete response rate of 93% (53/57) in the first year post MBMP and 100% during long-term follow-up. These outcome in life-threatening AH is unique and until now not achievable via other treatment schedules. In life-threatening bleedings physicians should take into account MBMP as a first line treatment.
authors.affiliation: Internal Medical Clinic I, Center of Extracorporeal Therapy and Autoimmunity (CETA), Germany.
authors.forename: Heike
authors.initials: H
authors.lastname: Zeitler
authors.forename: Gudrun
authors.initials: G
authors.lastname: Ulrich-Merzenich
authors.forename: Darius
authors.initials: D
authors.lastname: Panek
authors.forename: Georg
authors.initials: G
authors.lastname: Goldmann
authors.forename: Natascha
authors.initials: N
authors.lastname: Vidovic
authors.forename: Hans-Hermann
authors.initials: HH
authors.lastname: Brackmann
authors.forename: Johannes
authors.initials: J
authors.lastname: Oldenburg