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Macrophage activation syndrome resistant to medical therapy in a patient with systemic lupus erythematosus and its remission with splenectomy

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Author(s)
Ji Min KimSeung Ki KwokJi Hyeon JuKyung Su ParkGyeong Sin ParkHo Youn KimSung Hwan Park
Keimyung Author(s)
Kim, Ji Min
Department
Dept. of Internal Medicine (내과학)
Journal Title
Rheumatology International
Issued Date
2013
Volume
33
Issue
3
Keyword
Macrophage activation syndromeSpleenHemophagocytosisSystemic lupusErythematosusPositron emission tomography
Abstract
Macrophage activation syndrome (MAS) is a rare, but potentially life-threatening complication of systemic lupus erythematosus (SLE). A bone marrow biopsy often provides pathologic evidence of MAS, and MAS usually responds to corticosteroids alone or with the addition of cyclosporine A. We describe a case of MAS developing in a pregnant patient with SLE, who presented with fever and pancytopenia. Extensive investigations could not find the evidence of infection. Although intensive medical treatment was performed with a suspicion of MAS based on clinical grounds, no response was observed and bone marrow biopsy showed no evidence of hemophagocytosis. Positron emission tomography/computed tomography (PET/CT) suggested the possible cause of fever was in the spleen where Xuorodeoxyglucose uptake was markedly increased. After splenectomy, the patient was improved and numerous hemophagocytic macrophages were proved in the splenic tissue. With this unique case, we would like to emphasize that bone marrow biopsy cannot always be relied on in making a diagnosis of MAS and PET/CT can provide helpful information in the diagnosis of MAS.
Keimyung Author(s)(Kor)
김지민
Publisher
School of Medicine
Citation
Ji Min Kim et al. (2013). Macrophage activation syndrome resistant to medical therapy in a patient with systemic lupus erythematosus and its remission with splenectomy. Rheumatology International, 33(3), 767–771. doi: 10.1007/s00296-010-1654-4
Type
Article
ISSN
0172-8172
Source
http://lps3.link.springer.com.proxy.dsmc.or.kr/article/10.1007%2Fs00296-010-1654-4
DOI
10.1007/s00296-010-1654-4
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/34353
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Internal Medicine (내과학)
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