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


ABSTRACT: 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

SUBMITTER: Schep SJ 

PROVIDER: S-EPMC7756759 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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

Schep Sarah J SJ   van Dijk Wobke E M WEM   Beckers Erik A M EAM   Meijer Karina K   Coppens Michiel M   Eikenboom Jeroen J   Leebeek Frank W G FWG   van Vulpen Lize F D LFD   Fischer Kathelijn F KF   Schutgens Roger E G REG  

American journal of hematology 20201010 1


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 patient  ...[more]

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