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- Title
Critical Imbalance of TNF-α and Soluble TNF Receptor 1 in a Patient with Macrophage Activation Syndrome: Potential Implications for Diagnostics and Treatment.
- Authors
Flammiger, Anna; Fiedler, Walter; Bacher, Ulrike; Bokemeyer, Carsten; Schneider, Marion; Binder, Mascha
- Abstract
Macrophage activation syndrome (MAS) is a life-threatening disease with sustained activation of inflammatory cells and release of proinflammatory TNF-α. Under physiological conditions, TNF-α initiates a negative feedback mechanism, mediated by shedded, soluble TNF receptor ectodomains, eventually limiting the inflammatory reaction. Here, we report on a 27-year-old critically ill patient with refractory MAS and an insufficient negative feedback regulation, resulting in an overwhelming inflammatory response. A personalized treatment with soluble TNF receptor etanercept resulted in a durable remission. Further studies are warranted to establish whether the TNF cytokine profile may help to successfully guide patient selection for biological therapies. Copyright © 2012 S. Karger AG, Basel
- Subjects
CASE studies; MACROPHAGE activation syndrome; TUMOR necrosis factors; ETANERCEPT; INFLAMMATION; CRITICALLY ill; DISEASE remission
- Publication
Acta Haematologica, 2012, Vol 128, Issue 2, p69
- ISSN
0001-5792
- Publication type
Article
- DOI
10.1159/000338179