Gao SP, Luo XF, Kosari M, Li WJ, Yang L, Tu W, Zhong JX. Successful management of infection and macrophage activation syndrome patient using low-dose etoposide: A case report. World J Radiol 2024; 16(10): 579-585 [PMID: 39494131 DOI: 10.4329/wjr.v16.i10.579]
Corresponding Author of This Article
Wei Tu, Department of Rheumatology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, No. 1095 Jiefang Ave., Wuhan 430030, Hubei Province, China. zhongjixin620@163.com
Research Domain of This Article
Immunology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Shu-Pei Gao, Xiao-Fang Luo, Mohammadreza Kosari, Wen-Juan Li, Department of Rheumatology, Tongji Hospital, Wuhan 430030, Hubei Province, China
Liu Yang, Reproductive Medicine Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
Wei Tu, Department of Rheumatology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
Ji-Xin Zhong, Department of Rheumatology and Immunology, Tongji Hospital of Tongji Medical College, Wuhan 430030, Hubei Province, China
Co-corresponding authors: Wei Tu and Liu Yang.
Author contributions: Gao SP, Luo XF and Kosari M contributed equally to this study (responsible for data collection and analysis); Zhong JX, Tu W, and Yang L contribute equally to the study design and data analysis; Gao SP, Li WJ, and Kosari M drew the data and drafted the manuscript; Zhong JX revised the manuscript. All authors have confirmed the final approval.
Supported byHubei Provincial Natural Science Foundation of China, No. 2023AFB771; and National Natural Science Foundation of China, No. 82270903 and No. 81974254.
Informed consent statement: The patient provided a verbal informed consent for the documentation of her case.
Conflict-of-interest statement: The authors declare no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Wei Tu, Department of Rheumatology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, No. 1095 Jiefang Ave., Wuhan 430030, Hubei Province, China. zhongjixin620@163.com
Received: May 16, 2024 Revised: September 5, 2024 Accepted: September 24, 2024 Published online: October 28, 2024 Processing time: 165 Days and 4.1 Hours
Abstract
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.
Core Tip: Etoposide has been utilized in severe and refractory cases of macrophage activation syndrome (MAS). However, in cases where severe infections and autoimmune disorders are present concurrently, the use of etoposide carries the risk of bone marrow suppression and exacerbation of infections. In this case report, we successfully avoided bone marrow suppression and controlled both MAS and severe infection in patients in such condition by using a modified etoposide regimen.
