Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 6, 2023; 11(34): 8212-8218
Published online Dec 6, 2023. doi: 10.12998/wjcc.v11.i34.8212
Hemophagocytic lymphohistiocytosis with jaundice as first manifestation: A case report
Dan-Dan Wang, Sheng Wu, Bing-Bing Kong, Lin-Lin Song
Dan-Dan Wang, Sheng Wu, Bing-Bing Kong, Lin-Lin Song, Department of Emergency Medicine, Beijing Tsinghua Changgung Hospital, Medical Center, Tsinghua University, Beijing 102218, China
Author contributions: Wu S and Kong BB Participated in the treatment process and tried hard to save this patient; Wang DD wrote the manuscript; Wu S and Song LL revised this manuscript; all authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this case report and any accompanying images.
Conflict-of-interest statement: The authors declare that there is no conflict of interest to disclose.
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: Lin-Lin Song, PhD, Chief Physician, Doctor, Department of Emergency Medicine, Beijing Tsinghua Changgung Hospital, Medical Center, Tsinghua University, No. 168 Litang Road, Changping District, Beijing 102218, China. slla01139@btch.edu.cn
Received: October 3, 2023
Peer-review started: October 3, 2023
First decision: November 1, 2023
Revised: November 11, 2023
Accepted: November 27, 2023
Article in press: November 27, 2023
Published online: December 6, 2023
Processing time: 63 Days and 17.7 Hours
Abstract
BACKGROUND

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition. It is an immune-mediated disease that has a wide range of causes, elicits a hyperinflammatory response, and results in multiple organ damage. Clinical presentations vary, and in some cases, jaundice occurs as the first symptom.

CASE SUMMARY

We report the case of a 71-year-old female patient who presented with jaundice. She was admitted to our hospital because of the occurrence of “jaundice for half a month”, and upon examination, obstructive jaundice with choledocholithiasis and gallstones was suggested. Cholecystectomy and choledocholithotomy were performed. However, the jaundice did not improve after surgery. We found splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and elevated ferritin. Bone marrow biopsy revealed hemophagocytosis. Later, cardiac arrest occurred when she returned 3 wk after the surgery. We considered that HLH was triggered by septic shock. The patient’s condition deteriorated rapidly, with multiple organ dysfunction and severe gastrointestinal bleeding. Corticosteroid therapy and symptomatic treatment failed to save her life.

CONCLUSION

Jaundice rarely presents as the first symptom in HLH patients. The HLH in this case was triggered by septic shock with jaundice as the first symptom. Clinicians should try hard to reduce missed diagnoses and misdiagnoses.

Keywords: Hemophagocytic lymphohistiocytosis; Jaundice; Common bile duct stones; Bile duct obstruction; Septic shock; Case report

Core Tip: We report the case of a 71-year-old female patient who presented with jaundice. Obstructive jaundice with choledocholithiasis and gallstones was suggested. Then, we found splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and elevated ferritin. Bone marrow biopsy revealed hemophagocytosis. Later, cardiac arrest occurred when she returned after the surgery. We considered that hemophagocytic lymphohistiocytosis (HLH) was triggered by septic shock. The patient’s condition deteriorated rapidly, with multiple organ dysfunction. Corticosteroid therapy and symptomatic treatment failed to save her life. Jaundice rarely presents as the first symptom in HLH patients. Elevated direct bilirubin was associated with prognosis. Clinicians should try to reduce missed diagnoses and misdiagnoses.