Treatment of acquired hemophilia A, a balancing act:results from a 27-year Dutch cohort study

Acquired hemophilia A (AHA) is a severe auto-immune bleeding disorder. Treatment of AHA is burdensome and optimal management is still unresolved. Therefore a retrospective nationwide multi-center cohort study (1992-2018) was performed to evaluate clinical presentation and treatment efficacy and safety of AHA in the Netherlands. Multivariate logistic and Cox regression analysis was used to study independent associations between patient characteristics and clinical outcomes. A total of 143 patients (median age 73 years; 52.4% male) were included with a median follow-up of 16.8 months (IQR 3.6-41... Mehr ...

Verfasser: Schep, Sarah J.
van Dijk, Wobke E. M.
Beckers, Erik A. M.
Meijer, Karina
Coppens, Michiel
Eikenboom, Jeroen
Leebeek, Frank W. G.
van Vulpen, Lize F. D.
Fischer, Kathelijn F.
Schutgens, Roger E. G.
Dokumenttyp: Artikel
Erscheinungsdatum: 2021
Reihe/Periodikum: Dutch Soc Haemophilia Treaters Ne , Schep , S J , van Dijk , W E M , Beckers , E A M , Meijer , K , Coppens , M , Eikenboom , J , Leebeek , F W G , van Vulpen , L F D , Fischer , K F & Schutgens , R E G 2021 , ' Treatment of acquired hemophilia A, a balancing act : results from a 27-year Dutch cohort study ' , American Journal of Hematology , vol. 96 , no. 1 , pp. 51-59 . https://doi.org/10.1002/ajh.26009
Schlagwörter: SURVEILLANCE / EXPERIENCE / INHIBITORS
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-26671063
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://hdl.handle.net/11370/6cf7b942-aa55-4895-8026-19d485ddac6c

Acquired hemophilia A (AHA) is a severe auto-immune bleeding disorder. Treatment of AHA is burdensome and optimal management is still unresolved. Therefore a retrospective nationwide multi-center cohort study (1992-2018) was performed to evaluate clinical presentation and treatment efficacy and safety of AHA in the Netherlands. Multivariate logistic and Cox regression analysis was used to study independent associations between patient characteristics and clinical outcomes. A total of 143 patients (median age 73 years; 52.4% male) were included with a median follow-up of 16.8 months (IQR 3.6-41.5 months). First-line immunosuppressive treatment was mostly steroid monotherapy (67.6%), steroids/cyclophosphamide (11.9%) and steroids/rituximab (11.9%), with success rates of 35.2%, 80.0% and 66.7% respectively, P