Critical imbalance of TNF-α and soluble TNF receptor 1 in a patient with macrophage activation syndrome: potential implications for diagnostics and treatment.
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.
Departments of Oncology and Hematology, University Medical Center Hamburg-Eppendorf, Hubertus Wald Cancer Center, Hamburg, Germany.
Anna
A
Flammiger
Walter
W
Fiedler
Ulrike
U
Bacher
Carsten
C
Bokemeyer
Marion
M
Schneider
Mascha
M
Binder
D000328:Adult; D005260:Female; D006801:Humans; D008262:Macrophage Activation; D018124:Receptors, Tumor Necrosis Factor / Q000097:blood*; D014409:Tumor Necrosis Factor-alpha / Q000097:blood*
D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha