Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 6, 2020; 8(3): 560-567
Published online Feb 6, 2020. doi: 10.12998/wjcc.v8.i3.560
Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report
Gui Wang, Xiao-Rong Jin, De-Xun Jiang
Gui Wang, Beijing University of Chinese Medicine, Beijing 100029, China
Gui Wang, China-Japan Friendship Hospital, Beijing 100029, China
Xiao-Rong Jin, De-Xun Jiang, Rheumatism and Immunology Department, Seventh Medical Center of PLA General Hospital, Beijing 100700, China
Author contributions: Wang G and Jin XR contributed equally to this work; Jin XR and Jiang DX designed the case report; Jin XR collected the patient data; Wang G and Jin XR wrote and revised the paper; All authors approved the final version.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case report and accompanying images.
Conflict-of-interest statement: The authors declare that there is no conflict of interest related to this report.
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: De-Xun Jiang, MD, Director, Doctor, Rheumatism and Immunology Department, Seventh Medical Center of PLA General Hospital, No. 5, Nanmencang, Dong40, Dongcheng District, Beijing 100010, China. jiangdx1@163.com
Received: November 14, 2019
Peer-review started: November 14, 2019
First decision: December 23, 2019
Revised: December 24, 2019
Accepted: January 2, 2020
Article in press: January 2, 2020
Published online: February 6, 2020
Processing time: 83 Days and 8.9 Hours
Abstract
BACKGROUND

Adult-onset still disease (AOSD) and hemophagocytic syndrome (HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.

CASE SUMMARY

A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage. However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids; further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.

CONCLUSION

For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death.

Keywords: Hemophagocytic syndrome; Adult-onset still disease; Hemophagocytosis; Case report

Core tip: The clinical manifestations of hemophagocytic syndrome (HPS) and adult-onset still disease (AOSD) are very similar. HPS is one of the most serious complications of AOSD and its risk of death is very high. For AOSD patients with high suspicion of HPS, bone marrow biopsies of the liver and spleen should be conducted to actively find pathological evidence. This would then enable clinicians to provide an early diagnosis and treatment strategy. Additionally, clinical symptoms and laboratory data should be taken into comprehensive consideration during treatment, to closely observe whether the disease is effectively controlled and adjust steroid dosage in time. If this is considered in the future, the occurrence of HPS could be avoided.