Critical imbalance of TNF-α and soluble TNF receptor 1 in a patient with macrophage activation syndrome: potential implications for diagnostics and treatment.
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Critical imbalance of TNF-α and soluble TNF receptor 1 in a patient with macrophage activation syndrome: potential implications for diagnostics and treatment. / Flammiger, Anna; Fiedler, Walter; Bacher, Ulrike; Bokemeyer, Carsten; Schneider, Marion; Binder, Mascha.
In: ACTA HAEMATOL-BASEL, Vol. 128, No. 2, 2, 2012, p. 69-72.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Critical imbalance of TNF-α and soluble TNF receptor 1 in a patient with macrophage activation syndrome: potential implications for diagnostics and treatment.
AU - Flammiger, Anna
AU - Fiedler, Walter
AU - Bacher, Ulrike
AU - Bokemeyer, Carsten
AU - Schneider, Marion
AU - Binder, Mascha
PY - 2012
Y1 - 2012
N2 - Macrophage activation syndrome (MAS) is a life-threatening disease with sustained activation of inflammatory cells and release of proinflammatory TNF-?. Under physiological conditions, TNF-? 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.
AB - Macrophage activation syndrome (MAS) is a life-threatening disease with sustained activation of inflammatory cells and release of proinflammatory TNF-?. Under physiological conditions, TNF-? 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.
KW - Adult
KW - Humans
KW - Female
KW - Macrophage Activation
KW - Receptors, Tumor Necrosis Factor/blood
KW - Tumor Necrosis Factor-alpha/blood
KW - Adult
KW - Humans
KW - Female
KW - Macrophage Activation
KW - Receptors, Tumor Necrosis Factor/blood
KW - Tumor Necrosis Factor-alpha/blood
M3 - SCORING: Journal article
VL - 128
SP - 69
EP - 72
JO - ACTA HAEMATOL-BASEL
JF - ACTA HAEMATOL-BASEL
SN - 0001-5792
IS - 2
M1 - 2
ER -