Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 6, 2019; 7(19): 3069-3073
Published online Oct 6, 2019. doi: 10.12998/wjcc.v7.i19.3069
Hemophagocytic lymphohistiocytosis complicated by polyserositis: A case report
Ping Zhu, Qing Ye, Ting-Hong Li, Tao Han, Feng-Mei Wang
Ping Zhu, Qing Ye, Ting-Hong Li, Tao Han, Feng-Mei Wang, Department of Hepatology and Gastroenterology, Tianjin Third Central Hospital, The Third Central Clinical College of Tianjin Medical University, Tianjin 300170, China
Author contributions: Wang FM designed the report; Li TH and Ye Q collected the patient’s clinical data; Zhu P and Han T analyzed the data and wrote the paper.
Informed consent statement: Consent was obtained from relatives of the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: No conflict of interest exits.
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 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/
Corresponding author: Feng-Mei Wang, MD, Chief Doctor, Department of Hepatology and Gastroenterology, Tianjin Third Central Hospital, The Third Central Clinical College of Tianjin Medical University, 83 Jintang Road, Hedong District, Tianjin 300170, China. wangfengmeitj@126.com
Telephone: +86-22-84112122 Fax: +86-22-24315132
Received: June 27, 2019
Peer-review started: June 29, 2019
First decision: July 31, 2019
Revised: August 28, 2019
Accepted: September 9, 2019
Article in press: September 9, 2019
Published online: October 6, 2019
Processing time: 95 Days and 10 Hours
Abstract
BACKGROUND

Hemophagocytic lymphohistiocytosis (HLH) is a rare group of disorders of immune dysregulation characterized by clinical symptoms of severe inflammation. There are basically two types of clinical scenarios: Familial HLH and sporadic HLH. It is thought that the syndrome is implicated in the development of infections, malignancies, and autoimmune diseases. HLH, whether primary or secondary, is characterized by activated macrophages in hematopoietic organs, hepatosplenomegaly, cytopenia, and fever; however, HLH complicated with polyserositis (PS) has never been reported.

CASE SUMMARY

We present a case of fever in a 46-year-old previously healthy Chinese woman complicated by pericardial, pleural, and abdomen effusions. She had no contact with sick individuals, recent travel, illicit drug use, or new sexual contacts. She did not consume alcohol or tobacco and lacked a family history of other diseases. Antibiotics were prescribed for suspected infection, and acute liver injury subsequently occurred. Contrast-enhanced computed tomography showed mild pericardial effusion, pleural effusion, hepatosplenomegaly, and a large amount of ascites. A full blood count revealed leukopenia and thrombocytopenia. Increased ferritin and triglyceride levels were observed. The test for Epstein-Barr (EB) virus DNA was positive. This suggests that EB virus replication and EB virus infection existed. Additional studies showed hemophagocytosis in bone marrow biopsy specimens. The patient’s condition progressed rapidly. After providing symptomatic support treatment, eliminating immune stimuli, and administering comprehensive cyclosporine and dexamethasone treatment, the patient’s condition continued to progress, and the patient’s family members decided to stop treatment; the patient subsequently died.

CONCLUSION

This case shows the significance of considering HLH as part of the evaluation of unexplained fever and PS of unknown origin.

Keywords: Hemophagocytic lymphohistiocytosis; Polyserositis; Liver injury; Fever; Case report

Core tip: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the mononuclear phagocytic system, characterized by systemic proliferation of non-neoplastic histiocytes. HLH, whether primary or secondary, is characterized by activated macrophages in hematopoietic organs, hepatosplenomegaly, cytopenia, and fever, but complicated with polyserositis (PS) is rare. We present a case of HLH in a 46-year-old Chinese woman complicated by pericardial, pleural, and abdomen effusions. It could be important to focus attention on PS in order to broaden our outlook and expand our train of thought.