Critical imbalance of TNF-α and soluble TNF receptor 1 in a patient with macrophage activation syndrome: potential implications for diagnostics and treatment

Acta Haematol. 2012;128(2):69-72. doi: 10.1159/000338179. Epub 2012 Jun 6.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Humans
  • Macrophage Activation*
  • Receptors, Tumor Necrosis Factor / blood*
  • Tumor Necrosis Factor-alpha / blood*


  • Receptors, Tumor Necrosis Factor
  • Tumor Necrosis Factor-alpha