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Sim, Kim, and Kim: A Case of Macropharge Activation Syndrome Successfully Treated with Combination Therapy Including Etanercept
Case Report
Journal of Rheumatic Diseases

Journal of Rheumatic Diseases 2012; 19(4): 225-229.

Published online: 31 August 2012

DOI: https://doi.org/10.4078/jrd.2012.19.4.225

A Case of Macropharge Activation Syndrome Successfully Treated with Combination Therapy Including Etanercept

Young Seok Sim1, Hyun Soo Kim2, Kwang Nam Kim1

1Department of Pediatrics, Hallym University Medical Center, Hallym University College of Medicine, Seoul, Korea.

2Department of Laboratory Medicine, Hallym University Medical Center, Hallym University College of Medicine, Seoul, Korea.

Corresponding to: Kwang Nam Kim, Department of Pediatrics, Hallym University Medical Center, Hallym University College of Medicine, 896, Pyeongchon-dong, Anyang 431-070, Korea. rheumatol@korea.com

Received 14 September 2011       Revised 13 October 2011       Accepted 13 October 2011

Copyright © 2012 by The Korean College of Rheumatology

(free-access):

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of childhood systemic inflammatory disorder, primarily systemic onset juvenile rheumatoid arthritis (SoJRA). It is characterized by pancytopenia, liver insufficiency, coagulopathy, and neurologic symptoms. The clinical manifestations are caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to cytokine overproduction including tumor necrosis factor-α (TNF-α). Methylprednisolone pulse therapy and cyclosporine A have made a considerable progress in the treatment of MAS. However, the mortality rate remains high suggesting the need of another therapeutic agent. Several cases of MAS successfully treated with TNF-α inhibitor (etanercept) have been reported. We report the first Korean case of MAS successfully treated with combination therapy of corticosteroid, cyclosporine A and etanercept.

Keywords: Macrophage activation syndrome (MAS), Etanercept

Figures and Tables

Figure 1
A typical skin rash of macropharge activation syndrome (MAS).
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Figure 2
Bone marrow aspirate smears of a macropharge activation syndrome (MAS) demonstrating hemophagocytosis (arrow) (Wright stain, ×1,000).
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Table 1
Cotemporary classification of histiocytic disorder (5)
jrd-19-225-i001
Table 2
Diagnostic guidelines for hemophagocytic lymphohistiocytosis: HLH 2004 protocol (3)
jrd-19-225-i002

The diagnosis of hemophagocytic lymphohistiocytosis (HLH) can be established by one or both of the criteria described in the table.

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