4.6 Article

Reduced production of IFN-γ and LT-α is associated with successful prednisone therapy in patients with acquired hemophilia A: A pilot study

Journal

THROMBOSIS RESEARCH
Volume 128, Issue 5, Pages E86-E90

Publisher

PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.thromres.2011.06.032

Keywords

Prednisone; Acquired hemophilia A; Interferon-gamma; Lymphotoxin-alpha

Funding

  1. Louisiana Comprehensive Hemophilia Care Center

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Glucocorticoids (GC) are a standard treatment for acquired hemophilia A (AH). Although the optimal treatment regimen and duration of GC's is unknown, measurement of sub-clinical immune responses may help direct therapeutic decision making. To study the helpfulness of this approach, three male patients diagnosed with AH were treated with prednisone. The therapy resulted in inhibitor elimination in two out of the three individuals. During the treatment, peripheral mononuclear cells were isolated at different time points and stimulated in vitro. The expression of IFN-gamma and LT-alpha were monitored at both the protein and the mRNA levels. The amount of IFN-gamma and LT-alpha were markedly reduced by the time of inhibitor disappearance in the patients responding to GC therapy but remained high in the non-responder until cyclophosphamide was added. This study suggests that the secretion level of IFN-gamma and/or LT-alpha could be a predictive marker of prednisone responsiveness. (C) 2011 Elsevier Ltd. All rights reserved.

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