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Tiede, A; Klamroth, R; Scharf, RE; Trappe, RU; Holstein, K; Huth-Kühne, A; Gottstein, S; Geisen, U; Schenk, J; Scholz, U; Schilling, K; Neumeister, P; Miesbach, W; Manner, D; Greil, R; von Auer, C; Krause, M; Leimkühler, K; Kalus, U; Blumtritt, JM; Werwitzke, S; Budde, E; Koch, A; Knöbl, P.
Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study.
BLOOD. 2015; 125(7): 1091-1097. Doi: 10.1182/blood-2014-07-587089 [OPEN ACCESS]
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Co-authors Med Uni Graz: Neumeister Peter
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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.
Find related publications in this database (using NLM MeSH Indexing): Adult -; Aged -; Aged, 80 and over -; Autoantibodies - adverse effects; Factor VIII - analysis Factor VIII - immunology; Female -; Hemophilia A - diagnosis Hemophilia A - mortality Hemophilia A - therapy; Humans -; Immunosuppressive Agents - therapeutic use; Male -; Middle Aged -; Prognosis -; Remission Induction -; Survival Analysis -; Treatment Outcome -