Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 6, 2018; 6(13): 659-665
Published online Nov 6, 2018. doi: 10.12998/wjcc.v6.i13.659
Successful rescue of acute liver failure and hemophagocytic lymphohistiocytosis following varicella infection: A case report and review of literature
Li-Na Zhang, Wei Guo, Ji-Hong Zhu, Yang Guo
Li-Na Zhang, Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China
Wei Guo, Ji-Hong Zhu, Yang Guo, Department of Emergency, Peking University People’s Hospital, Beijing 100044, China
Author contributions: Guo Y and Zhu JH designed the report; Guo W and Zhang LN collected the patient’s clinical data; Zhang LN analyzed the data and wrote the paper.
Supported by Capital Characteristic Clinic Project, No. Z161100000516045.
Informed consent statement: Consent was obtained from the patient and his parent for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
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/
Correspondence to: Yang Guo, MD, Professor, Department of Emergency, Peking University People’s Hospital, 11 Xizhimen South St, Beijing 100044, China. edguoyang@163.com
Telephone: +86-10-88324690 Fax: +86-10-88324690
Received: August 7, 2018
Peer-review started: August 7, 2018
First decision: August 24, 2018
Revised: September 10, 2018
Accepted: October 9, 2018
Article in press: October 9, 2018
Published online: November 6, 2018
Processing time: 92 Days and 0 Hours
ARTICLE HIGHLIGHTS
Case characteristics

A previously healthy 16-year-old boy developed acute liver failure (ALF) and hemophagocytic lymphohistiocytosis (HLH) soon after varicella infection.

Clinical diagnosis

Generalized skin rash with various stages of development including maculopapules, vesicles, pustules, and crusts were typical features of chickenpox [primary infection of varicella-zoster virus(VZV)].

Differential diagnosis

The differential diagnosis for extremely high level of ferritin (> 50000 ng/mL) could be limited to few clinical circumstances, such as Still’s disease, HLH, and systemic histoplasmosis.

Laboratory diagnosis

A sudden onset of liver injury with soared transaminases and decreased prothrombin activity pointed to ALF. Pancytopenia, hypofibrinogenemia, and hyperferritinemia were clues for HLH. The polymerase chain reaction (PCR) amplifications of VZV confirmed varicella infection.

Imaging diagnosis

An ultrasound of the abdomen showed splenomegaly, but neither hepatomegaly nor ascites.

Pathological diagnosis

Not applicable.

Treatment

The patient underwent a combination therapy of acyclovir (10 mg/kg every 8 h), supportive care, and immunosuppression with dexamethasone and etoposide.

Related reports

ALF associated with HLH is extremely fatal and rarely reported. In recent years, HLH first presenting as ALF was becoming increasingly noticed while the mortality remained high.

Term explanation

HLH, also known as hemophagocytic syndrome, is a devastating disorder characterized by fever, splenomegaly, cytopenia and the finding of activated macrophages in hemopoietic organs.

Experiences and lessons

Accumulating evidence pointed towards a similar immune dysregulation pattern in ALF and HLH. It is important to maintain a high suspicion for HLH in ALF with or without an identified trigger. Patients might benefit from therapies targeted to halt any underlying trigger and control the overactive immune system.