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：23

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

共 16 条

[1] STABILIZATION OF THE BIOACTIVITY OF TUMOR-NECROSIS-FACTOR BY ITS SOLUBLE RECEPTORS [J].

ADERKA, D ;

ENGELMANN, H ;

MAOR, Y ;

BRAKEBUSCH, C ;

WALLACH, D .

JOURNAL OF EXPERIMENTAL MEDICINE, 1992, 175 (02) :323-329

[2] Concentrations of CXCL8, CXCL9 and sTNFR1 in plasma of patients with pulmonary tuberculosis undergoing treatment [J].

Alessandri, A. L. ;

Souza, A. L. ;

Oliveira, S. C. ;

Macedo, G. C. ;

Teixeira, M. M. ;

Teixeira, A. L. .

INFLAMMATION RESEARCH, 2006, 55 (12) :528-533

[3] Macrophage activation syndrome:: characteristic findings on liver biopsy illustrating the key role of activated, IFN-γ-producing lymphocytes and IL-6- and TNF-α-producing macrophages [J].

Billiau, AD ;

Roskams, T ;

Van Damme-Lombaerts, R ;

Matthys, P ;

Wouters, C .

BLOOD, 2005, 105 (04) :1648-1651

[4] Macrophage activation syndrome in juvenile idiopathic arthritis [J].

Cortis, Elisabetta ;

Insalaco, Antonella .

ACTA PAEDIATRICA, 2006, 95 :38-41

[5] Review of haemophagocytic lymphohistiocytosis [J].

Freeman, H. R. ;

Ramanan, A. V. .

ARCHIVES OF DISEASE IN CHILDHOOD, 2011, 96 (07) :688-693

[6] Soluble receptors in human disease [J].

Heaney, ML ;

Golde, DW .

JOURNAL OF LEUKOCYTE BIOLOGY, 1998, 64 (02) :135-146

[7] The TNF receptor-associate periodic syndrome (TRAPS) - Emerging concepts of an autoinflammatory disorder [J].

Hull, KM ;

Drewe, E ;

Aksentijevich, I ;

Singh, HK ;

Wong, K ;

McDermott, EM ;

Dean, J ;

Powell, RJ ;

Kastner, DL .

MEDICINE, 2002, 81 (05) :349-368

[8] Etanercept for therapy-resistant macrophage activation syndrome [J].

Makay, Balahan ;

Yilmaz, Sebnem ;

Tuerkyilmaz, Zeynep ;

Uenal, Nurettin ;

Oeren, Hale ;

Uensal, Erbil .

PEDIATRIC BLOOD & CANCER, 2008, 50 (02) :419-421

[9] Anti-TNF-α therapies:: The next generation [J].

Palladino, MA ;

Bahjat, FR ;

Theodorakis, EA ;

Moldawer, LL .

NATURE REVIEWS DRUG DISCOVERY, 2003, 2 (09) :736-746

[10] Elevated plasma levels of soluble TNF receptors are associated with morbidity and mortality in patients with acute lung injury [J].

Parsons, PE ;

Matthay, MA ;

Ware, LB ;

Eisner, MD .

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY, 2005, 288 (03) :L426-L431

← 1 2 →