Immunosuppressive treatment for acquired haemophilia: current practice and future directions in Germany, Austria and Switzerland.
Détails
ID Serval
serval:BIB_198F33B94FED
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Immunosuppressive treatment for acquired haemophilia: current practice and future directions in Germany, Austria and Switzerland.
Périodique
Annals of Hematology
ISSN
1432-0584 (Electronic)
ISSN-L
0939-5555
Statut éditorial
Publié
Date de publication
2009
Peer-reviewed
Oui
Volume
88
Numéro
4
Pages
365-370
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't Publication Status: ppublish
Résumé
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.
Mots-clé
Antibodies, Monoclonal/therapeutic use, Antibodies, Monoclonal, Murine-Derived, hic" UI="D001317">Austria, Autoantibodies/blood, Cyclophosphamide/therapeutic use, Data Collection, hic" UI="D005858">Germany, Hemophilia A/drug therapy, Hemophilia A/etiology, Humans, Immunosorbent Techniques, Immunosuppressive Agents/therapeutic use, Physician's Practice Patterns, Remission Induction, Steroids/therapeutic use, hic" UI="D013557">Switzerland, Treatment Failure
Pubmed
Web of science
Création de la notice
10/02/2015 10:11
Dernière modification de la notice
20/08/2019 12:50