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作 者:Shu-Pei Gao Xiao-Fang Luo Mohammadreza Kosari Wen-Juan Li Liu Yang Wei Tu Ji-Xin Zhong
机构地区:[1]Department of Rheumatology,Tongji Hospital,Wuhan 430030,Hubei Province,China [2]Reproductive Medicine Center,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,Hubei Province,China [3]Department of Rheumatology and Immunology,Tongji Hospital,Huazhong University of Science and Technology,Wuhan 430030,Hubei Province,China [4]Department of Rheumatology and Immunology,Tongji Hospital of Tongji Medical College,Wuhan 430030,Hubei Province,China
出 处:《World Journal of Radiology》2024年第10期579-585,共7页世界放射学杂志(英文)
基 金:Supported by Hubei Provincial Natural Science Foundation of China,No.2023AFB771;National Natural Science Foundation of China,No.82270903 and No.81974254.
摘 要:BACKGROUND Macrophage activation syndrome(MAS),a sub-type of hemophagocytic lymphohistiocytosis(HLH)secondary to autoimmune rheumatic diseases,is a critical and potentially fatal condition characterized by an excessive inflammatory response.Despite the established efficacy of the HLH-2004 guideline in diagnosing and treating HLH over the years,ongoing discussion persists regarding its application,especially for HLH secondary to complicated conditions,such as autoimmune rheumatic diseases combined with severe infection.Etoposide(VP-16),a topoisomerase II inhibitor that effectively induces DNA damage and subsequent apoptosis in hyperactivated immune cells,has been widely used for the treatment of HLH.However,its suppressive effect on immune system may also cause potential exacerbation of infection in autoimmune rheumatic disease-induced HLH patients complicated with severe infection.Therefore,the use of VP-16 in such cases was inconclusive.CASE SUMMARY In this case study,we propose a potentially effective strategy for managing a patient diagnosed with secondary HLH complicated with systemic lupus erythematosus(SLE)and chronic coronavirus disease 2019 infection.Our approach involves early administration of low-dose VP-16(100 mg twice a week,300 mg in total),combined with methylprednisolone,cyclophosphamide,and cyclosporine A.The administration of etoposide effectively led to improvements in various indices of HLH.CONCLUSION Low dose etoposide proves to be an effective approach in alleviating HLH while mitigating the risk of infection.
关 键 词:Macrophage activation syndrome Hemophagocytic lymphohistiocytosis INFECTION Systemic lupus erythematosus ETOPOSIDE Case report
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