Critical Imbalance of TNF-α and Soluble TNF Receptor 1 in a Patient with Macrophage Activation Syndrome: Potential Implications for Diagnostics and Treatment

被引：22

作者：

Flammiger, Anna ^{[1
]}

Fiedler, Walter ^{[1
]}

Bacher, Ulrike ^{[2
]}

Bokemeyer, Carsten ^{[1
]}

Schneider, Marion ^{[3
]}

Binder, Mascha ^{[1
]}

机构：

[1] Univ Med Ctr Hamburg Eppendorf, Hubertus Wald Canc Ctr, Dept Hematol & Oncol, DE-20246 Hamburg, Germany

[2] Univ Med Ctr Hamburg Eppendorf, Hubertus Wald Canc Ctr, Dept Stem Cell Transplantat, DE-20246 Hamburg, Germany

[3] Univ Med Ctr Ulm, Dept Anesthesiol, Sect Expt Anesthesiol, Ulm, Germany

来源：

ACTA HAEMATOLOGICA | 2012年 / 128卷 / 02期

关键词：

Macrophage activation syndrome; Hemophagocytic syndrome; TNF-alpha; Soluble TNF receptor 1; Etanercept; TUMOR-NECROSIS-FACTOR; PLASMA-LEVELS; INFLAMMATION; DISEASE; INJURY;

D O I：

10.1159/000338179

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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

引用

页码：69 / 72

页数：4

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