Immunoadsorption for pregnancy-associated severe acquired hemophilia.
Postpartum hemorrhage is a common cause of maternal mortality. Acquired hemophilia (AH) is a rare, life-threatening bleeding disorder induced by autoantibodies against coagulation factors (inhibitors). We report about eight patients with postpartum AH (out of 82). Seven AH patients with severe bleeding complications were treated by the "Modified Bonn-Malmö Protocol (MBMP)" which consists of inhibitor elimination via immunoadsorption (IA) in combination with immunosuppression and high-dose Factor VIII substitution. One patient was treated only by immunosuppression. Seven out of eight patients with severe AH and mean inhibitor titers (IT) of 118 BU/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 IA sessions (range 3-5 days), no inhibitor was detectable. The factor substitution was discontinued after a median of 13 IA sessions (range 8-24 days) and IA was terminated after a median of 15 sessions (range 9-27 days). One less severe affected patient (IT: 2.1 BU/mL) received prednisolone (1.5 mg/kg BW) 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 AH leads to a high bleeding risk with bleeding associated complications. Immunoadsorption offers an important treatment option in severe AH, enabling a fast reconstitution of the blood coagulation with a reduced time for the Factor VIII substitution and for immunosuppressive treatment. In cases of postpartum bleeding the diagnosis of AH should be routinely considered.
Medical Clinic I, Centre of Extracorporeal Therapy and Autoimmunity (CETA), University Clinic Centre Bonn, Bonn, Germany.
Heike
H
Zeitler
Gudrun
G
Ulrich-Merzenich
Natascha
N
Marquardt
Johannes
J
Oldenburg
Georg
G
Goldmann
D000328:Adult; D005169:Factor VIII / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006467:Hemophilia A / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D006473:Postpartum Hemorrhage / Q000209:etiology / Q000628:therapy*; D011239:Prednisolone / Q000627:therapeutic use; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000473:pathology / Q000628:therapy*; D012074:Remission Induction; D012720:Severity of Illness Index; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D005169:Factor VIII; D011239:Prednisolone
Acquired hemophilia; Immunmodulation; Immunoadsorption; Modified Bonn-Malmö protocol; Postpartum; Pregnancy