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Blood. 2015 Feb 12;125(7):1091-7. doi: 10.1182/blood-2014-07-587089. Epub 2014 Dec 18.

Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study.

Author information

1
Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany;
2
Internal Medicine, Vivantes Clinic Friedrichshain, Berlin, Germany;
3
Hemostasis, Hemotherapy, and Transfusion Medicine, Heinrich Heine University Center, Düsseldorf, Germany;
4
Hematology and Oncology, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany; Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Evangelisches Diakonie-Krankenhaus Bremen, Bremen, Germany;
5
Hematology and Oncology, University Hospital Eppendorf, Hamburg, Germany;
6
Hemophilia Care Center, SRH Kurpfalzkrankenhaus Heidelberg, Heidelberg, Germany;
7
Institute for Clinical Chemistry and Laboratory Medicine, Freiburg University Hospital, Freiburg, Germany;
8
Clinical Hemostaseology and Transfusion Medicine, Saarland University Hospital, Homburg/Saar, Germany;
9
Center of Coagulation Disorders, Leipzig, Germany;
10
Hematology and Oncology, Jena University Hospital, Jena, Germany;
11
Hematology, Graz Medical University, Graz, Austria;
12
Hematology and Oncology, Johann Wolfgang Goethe University Hospital, Frankfurt/Main, Germany;
13
Clinical Chemistry, University Medical Center Schleswig-Holstein, Campus Lübeck, Lübeck, Germany;
14
Hematology, Hemostasis, and Oncology, Paracelsus Medical University, Salzburg, Austria;
15
Hematology and Oncology, Mainz University Hospital, Mainz, Germany;
16
Internal Medicine, German Diagnostic Clinic, Wiesbaden, Germany;
17
Anesthesiology, Evangelical Hospital, Bielefeld, Germany;
18
Transfusion Medicine, Charité University Hospital, Berlin, Germany;
19
Biometry, Hannover Medical School, Hannover, Germany; and.
20
Hematology and Hemostasis, Vienna Medical University, Vienna, Austria.

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.

PMID:
25525118
PMCID:
PMC4326770
DOI:
10.1182/blood-2014-07-587089
[Indexed for MEDLINE]
Free PMC Article

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