Journal
ACTA HAEMATOLOGICA
Volume 128, Issue 2, Pages 69-72Publisher
KARGER
DOI: 10.1159/000338179
Keywords
Macrophage activation syndrome; Hemophagocytic syndrome; TNF-alpha; Soluble TNF receptor 1; Etanercept
Categories
Ask authors/readers for more resources
Macrophage activation syndrome (MAS) is a life-threatening disease with sustained activation of inflammatory cells and release of proinflannmatory TNF-alpha. Under physiological conditions, TNF-a 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 (C) 2012 S. Karger AG, Basel
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available