title: Immunosuppressive treatment for acquired haemophilia: current practice and future directions in Germany, Austria and Switzerland.
abstract: Acquired haemophilia is an autoimmune disorder characterised by autoantibody formation against coagulation factor VIII. Immunosuppressive treatments including steroids, cytotoxic drugs, rituximab or combinations thereof have been used to eradicate autoantibodies. Very few prospective studies exist evaluating the use of these treatments. Here, we performed a survey among 73 physicians from 57 haemophilia treatment centres in order to describe current practice patterns and critical issues for future research in acquired haemophilia. The results demonstrate a high diversity of first- and second-line treatments. Factors influencing treatment decision were underlying disorder, severity of bleeding and inhibitor titre. Frequently used first-line treatments were steroids plus cyclophosphamide (44%) and steroids alone (11%). Second-line treatment was most often rituximab (30%), with or without steroids and/or cyclophosphamide. Most participants indicated to change from first- to second-line treatment after 4 weeks in case of failure to obtain partial remission (31%), continued bleeding (40%) or continued severe bleeding requiring bypass treatment (59%). Immunoadsorption was preferred for first- and second-line treatment by 10% and 9% of participants, respectively. These results highlight critical issues in the field. Open questions and directions for future research are discussed.
authors.affiliation: Department of Haematology, Haemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany. tiede.andreas@mh-hannover.de
authors.forename: Andreas
authors.initials: A
authors.lastname: Tiede
authors.forename: Angela
authors.initials: A
authors.lastname: Huth-Kühne
authors.forename: Johannes
authors.initials: J
authors.lastname: Oldenburg
authors.forename: Ralf
authors.initials: R
authors.lastname: Grossmann
authors.forename: Ulrich
authors.initials: U
authors.lastname: Geisen
authors.forename: Manuela
authors.initials: M
authors.lastname: Krause
authors.forename: Brigit
authors.initials: B
authors.lastname: Brand
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Alberio
authors.forename: Robert
authors.initials: R
authors.lastname: Klamroth
authors.forename: Michael
authors.initials: M
authors.lastname: Spannagl
authors.forename: Paul
authors.initials: P
authors.lastname: Knöbl
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001317:Austria; D001323:Autoantibodies / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D003625:Data Collection; D005858:Germany; D006467:Hemophilia A / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D010818:Practice Patterns, Physicians'; D012074:Remission Induction; D000069283:Rituximab; D013256:Steroids / Q000627:therapeutic use; D013557:Switzerland; D017211:Treatment Failure
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D013256:Steroids; D000069283:Rituximab; D003520:Cyclophosphamide