Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 26, 2020; 8(20): 4946-4952
Published online Oct 26, 2020. doi: 10.12998/wjcc.v8.i20.4946
Systemic autoimmune abnormalities complicated by cytomegalovirus-induced hemophagocytic lymphohistiocytosis: A case report
Shu-Xian Miao, Zhi-Qi Wu, Hua-Guo Xu
Shu-Xian Miao, Zhi-Qi Wu, Hua-Guo Xu, Department of Laboratory Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, Jiangsu Province, China
Author contributions: Xu HG and Wu ZQ designed the report; Miao SX collected the patient’s clinical data; All authors participated in the writing of the manuscript.
Supported by Natural Science Foundation of Jiangsu Province of China, No. BK20181492; the National Key Clinical Department of Laboratory Medicine of China in Nanjing, Key laboratory for Laboratory Medicine of Jiangsu Province, No. ZDXKB2016005.
Informed consent statement: Informed consent was obtained from the patient.
Conflict-of-interest statement: We declare that we have no conflicts 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: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Hua-Guo Xu, MD, PhD, Professor, Research Scientist, Department of Laboratory Medicine, The First Affiliated Hospital, Nanjing Medical University, No. 300 Guangzhou Road, Nanjing 210029, Jiangsu Province, China. huaguoxu@njmu.edu.cn
Received: July 12, 2020
Peer-review started: July 12, 2020
First decision: August 8, 2020
Revised: August 21, 2020
Accepted: September 10, 2020
Article in press: September 10, 2020
Published online: October 26, 2020
Processing time: 105 Days and 20.2 Hours
Abstract
BACKGROUND

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disorder, characterized by a hyperimmune response. The mortality is high despite progress being made in the diagnosis and treatment of the disease. HLH is traditionally divided into primary (familial or genetic) and secondary (reactive) according to the etiology. Secondary HLH (sHLH), more common in adults, is often associated with underlying conditions including severe infections, malignancies, autoimmune diseases, or other etiologies.

CASE SUMMARY

The case involves a 31-year-old woman, presented with a high persistent fever, rash, and splenomegaly. She met the diagnostic criteria of the HLH-2004 guideline and thus was diagnosed with HLH, with positive anti-nuclear antibody (ANA) and positive cytomegalovirus (CMV)-DNA. The patient responded well to a combination of immunomodulatory, chemotherapy, and supportive treatments. When her PCR evaluation for CMV turned negative, her serum ferritin also dropped significantly. Her clinical symptoms improved dramatically, and except for ANA, the abnormal laboratory findings associated with HLH returned to normal. Our previous study has shown that the median overall survival of HLH patients is only 6 mo; however, our patient has been cured and has not presented with any relapse of the disease for 6 years.

CONCLUSION

This case emphasizes that thorough early removal of the CMV infection is significant for the prognosis of this HLH patient.

Keywords: Hemophagocytic lymphohistiocytosis; Autoimmune abnormalities; Cytomegalovirus; Anti-nuclear antibody; Serum ferritin; Case report

Core Tip: Hemophagocytic lymphohistiocytosis (HLH) is a rare immune-mediated life-threatening disease. Active HLH develops rapidly, and the mortality rate is high if reasonable and effective interventions are not promptly undertaken. Herein, we report a case of a 31-year-old Chinese woman diagnosed with systemic autoimmune abnormalities complicated by cytomegalovirus (CMV)-induced HLH. The patient has been cured and has not relapsed for 6 years. This report may act as a reference for HLH therapy in cases positive for anti-nuclear antibody and CMV.