Peng LY, Liu JB, Zuo HJ, Shen GF. Unusual presentation of systemic lupus erythematosus as hemophagocytic lymphohistiocytosis in a female patient: A case report. World J Clin Cases 2023; 11(4): 909-917 [PMID: 36818616 DOI: 10.12998/wjcc.v11.i4.909]
Corresponding Author of This Article
Gui-Fen Shen, MD, Doctor, Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1095 Jiefang Avenue, Wuhan 430030, Hubei Province, China. guifenshen@126.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/
Li-Yuan Peng, Jing-Bo Liu, Hou-Juan Zuo, Division of Cardiology, Department of Internal Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
Gui-Fen Shen, Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
Author contributions: Peng LY and Liu JB reviewed the literature and drafted the manuscript; Zuo HJ and Shen GF were responsible for the revision of the manuscript for important intellectual content; all authors issued final approval for the version to be submitted.
Informed consent statement: Written informed consent was obtained from the patient’s legal guardian(s) for publication of this case report and any accompanying images.
Conflict-of-interest statement: We all 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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Gui-Fen Shen, MD, Doctor, Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1095 Jiefang Avenue, Wuhan 430030, Hubei Province, China. guifenshen@126.com
Received: October 13, 2022 Peer-review started: October 13, 2022 First decision: November 30, 2022 Revised: December 29, 2022 Accepted: January 12, 2023 Article in press: January 12, 2023 Published online: February 6, 2023 Processing time: 116 Days and 2.9 Hours
Abstract
BACKGROUND
Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disorder, often resulting in the immune-mediated injury of multiple organ systems, including primary HLH and secondary HLH (sHLH). Among them, sHLH results from infections, malignant, or autoimmune conditions, which have quite poor outcomes even with aggressive management and are more common in adults.
CASE SUMMARY
We report a rare case of a 36-year-old female manifested with sHLH on background with systemic lupus erythematosus (SLE). During hospitalization, the patient was characterized by recurrent high-grade fever, petechiae and ecchymoses of abdominal skin, and pulmonary infection. Whole exon gene sequencing revealed decreased activity of natural killer cells. She received systematic treatment with Methylprednisolone, Etoposide, and anti-infective drugs. Intravenous immunoglobulin and plasmapheresis were applied when the condition was extremely acute and progressive. The patient recovered and did not present any relapse of the HLH for one year of follow-up.
CONCLUSION
The case showed sHLH, thrombotic microvascular, and infection in the whole course of the disease, which was rarely reported by now. The treatment of the patient emphasizes that early recognition and treatment of sHLH in SLE patients was of utmost importance to improve the prognosis and survival rate of patients.
Core Tip: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disorder, including primary HLH and secondary HLH (sHLH). We report a rare case of a 36-year-old female manifested with sHLH on background with systemic lupus erythematosus (SLE) and related with decreased activity of natural killer cells according to whole exon gene sequencing. Our study expanded the thoughts on the diagnosis and treatment of HLH in SLE patients.
