Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study
Andreas Tiede, Robert Klamroth, Rüdiger E Scharf, Ralf U Trappe, Katharina Holstein, Angela Huth-Kühne, Saskia Gottstein, Ulrich Geisen, Joachim Schenk, Ute Scholz, Kristina Schilling, Peter Neumeister, Wolfgang Miesbach, Daniela Manner, Richard Greil, Charis von Auer, Manuela Krause, Klaus Leimkühler, Ulrich Kalus, Jan-Malte Blumtritt, Sonja Werwitzke, Eva Budde, Armin Koch, Paul Knöbl, Andreas Tiede, Robert Klamroth, Rüdiger E Scharf, Ralf U Trappe, Katharina Holstein, Angela Huth-Kühne, Saskia Gottstein, Ulrich Geisen, Joachim Schenk, Ute Scholz, Kristina Schilling, Peter Neumeister, Wolfgang Miesbach, Daniela Manner, Richard Greil, Charis von Auer, Manuela Krause, Klaus Leimkühler, Ulrich Kalus, Jan-Malte Blumtritt, Sonja Werwitzke, Eva Budde, Armin Koch, Paul Knöbl
Abstract
Acquired hemophilia A (AHA) is caused by autoantibodies against factor VIII (FVIII). Immunosuppressive treatment (IST) results in remission of disease in 60% to 80% of patients over a period of days to months. IST is associated with frequent adverse events, including infections as a leading cause of death. Predictors of time to remission could help guide IST intensity but have not been established. We analyzed prognostic factors in 102 prospectively enrolled patients treated with a uniform IST protocol. Partial remission (PR; defined as no active bleeding, FVIII restored >50 IU/dL, hemostatic treatment stopped >24 hours) was achieved by 83% of patients after a median of 31 days (range 7-362). Patients with baseline FVIII <1 IU/dL achieved PR less often and later (77%, 43 days) than patients with ≥1 IU/dL (89%, 24 days). After adjustment for other baseline characteristics, low FVIII remained associated with a lower rate of PR (hazard ratio 0.52, 95% confidence interval 0.33-0.81, P < .01). In contrast, PR achieved on steroids alone within ≤21 days was more common in patients with FVIII ≥1 IU/dL and inhibitor concentration <20 BU/mL (odds ratio 11.2, P < .0001). Low FVIII was also associated with a lower rate of complete remission and decreased survival. In conclusion, presenting FVIII and inhibitor concentration are potentially useful to tailor IST in AHA.
© 2015 by The American Society of Hematology.
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Source: PubMed