• Medientyp: E-Artikel
  • Titel: Immunoadsorption for Pregnancy‐Associated Severe Acquired Hemophilia
  • Beteiligte: Zeitler, Heike; Ulrich‐Merzenich, Gudrun; Marquardt, Natascha; Oldenburg, Johannes; Goldmann, Georg
  • Erschienen: Wiley, 2014
  • Erschienen in: Therapeutic Apheresis and Dialysis
  • Sprache: Englisch
  • DOI: 10.1111/1744-9987.12061
  • ISSN: 1744-9979; 1744-9987
  • Schlagwörter: Nephrology ; Hematology
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <jats:title>Abstract</jats:title><jats:p>Postpartum hemorrhage is a common cause of maternal mortality. Acquired hemophilia (<jats:styled-content style="fixed-case">AH</jats:styled-content>) is a rare, life‐threatening bleeding disorder induced by autoantibodies against coagulation factors (inhibitors). We report about eight patients with postpartum <jats:styled-content style="fixed-case">AH</jats:styled-content> (out of 82). Seven <jats:styled-content style="fixed-case">AH</jats:styled-content> patients with severe bleeding complications were treated by the “Modified Bonn‐Malmö Protocol (<jats:styled-content style="fixed-case">MBMP</jats:styled-content>)” which consists of inhibitor elimination via immunoadsorption (<jats:styled-content style="fixed-case">IA</jats:styled-content>) in combination with immunosuppression and high‐dose Factor <jats:styled-content style="fixed-case">VIII</jats:styled-content> substitution. One patient was treated only by immunosuppression. Seven out of eight patients with severe <jats:styled-content style="fixed-case">AH</jats:styled-content> and mean inhibitor titers (<jats:styled-content style="fixed-case">IT</jats:styled-content>) of 118 <jats:styled-content style="fixed-case">BU</jats:styled-content>/mL were referred to our center. They were severe cases with a median delay of diagnosis of 30.5 days (range 7–278 days). After a median of 3 <jats:styled-content style="fixed-case">IA</jats:styled-content> sessions (range 3–5 days), no inhibitor was detectable. The factor substitution was discontinued after a median of 13 <jats:styled-content style="fixed-case">IA</jats:styled-content> sessions (range 8–24 days) and <jats:styled-content style="fixed-case">IA</jats:styled-content> was terminated after a median of 15 sessions (range 9–27 days). One less severe affected patient (<jats:styled-content style="fixed-case">IT</jats:styled-content>: 2.1 <jats:styled-content style="fixed-case">BU</jats:styled-content>/mL) received prednisolone (1.5 mg/kg <jats:styled-content style="fixed-case">BW</jats:styled-content>) for 120 days. Complete remission was achieved in all patients with a median follow‐up of 100 months (range 56–126 m). The delayed diagnosis of pregnancy‐associated <jats:styled-content style="fixed-case">AH</jats:styled-content> leads to a high bleeding risk with bleeding associated complications. Immunoadsorption offers an important treatment option in severe <jats:styled-content style="fixed-case">AH</jats:styled-content>, enabling a fast reconstitution of the blood coagulation with a reduced time for the Factor <jats:styled-content style="fixed-case">VIII</jats:styled-content> substitution and for immunosuppressive treatment. In cases of postpartum bleeding the diagnosis of <jats:styled-content style="fixed-case">AH</jats:styled-content> should be routinely considered.</jats:p>