Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 16, 2023; 11(26): 6183-6188
Published online Sep 16, 2023. doi: 10.12998/wjcc.v11.i26.6183
Postpartum hemophagocytic lymphohistiocytosis: A case report
Ju Ho An, Jung Hwan Ahn
Ju Ho An, Jung Hwan Ahn, Department of Emergency Medicine, Ajou University School of Medicine, Suwon 16499, Gyeonggi-do, South Korea
Author contributions: An JH contributed to manuscript writing, editing, and data collection; Ahn JH contributed to conceptualization and supervision; All authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any images.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
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: Jung Hwan Ahn, MD, Chief Doctor, Department of Emergency Medicine, Ajou University School of Medicine, No. 164, World Cup-ro, Yeongtong-gu, Suwon 16499, Gyeonggi-do, South Korea. erdrajh@naver.com
Received: April 26, 2023
Peer-review started: April 26, 2023
First decision: July 3, 2023
Revised: July 12, 2023
Accepted: August 15, 2023
Article in press: August 15, 2023
Published online: September 16, 2023
Processing time: 135 Days and 3.4 Hours
Abstract
BACKGROUND

Postpartum hemophagocytic lymphohistiocytosis (HLH) is a rare disease with unclear pathophysiology. It is a secondary HLH diagnosed using the pediatric diagnostic criteria; however, the clinical diagnosis of postpartum HLH remains challenging. Hence, HLH may remain undiagnosed, leading to poor patient prognosis. Therefore, improvements in the accuracy of postpartum HLH diagnoses and treatments are necessary.

CASE SUMMARY

We report the case of a 40-year-old female with postpartum HLH. The patient attended the postpartum care center for 3 wk after giving birth and underwent needle aspiration due to thyroid gland enlargement 11 d before an emergency department visit precipitated by fever and abdominal pain. Since no abnormal emergency room findings were noted, the patient was discharged with a prescription for broad-spectrum antibiotics. Three days later, she returned to the emergency room in a hemodynamically unstable state and was admitted to the intensive care unit with suspected sepsis or hematologic disease. The patient was treated, without effect, for sepsis using broad-spectrum antibiotics, and for suspected hematologic disease with steroid therapy. However, she died due to rapidly worsening symptoms.

CONCLUSION

Rapid recognition and appropriate treatment of postpartum HLH are needed to improve the prognosis.

Keywords: Bone marrow, Pregnancy, Lymphohistiocytosis, Infection, Steroids, Case report

Core Tip: Postpartum hemophagocytic lymphohistiocytosis (HLH) is a rare disease that is not often considered clinically and is difficult to diagnose because of its rarity and varied clinical presentations. Its clinical features are similar to those of sepsis or some pregnancy-related diseases, but the treatment is different. Therefore, an accurate diagnosis is necessary, and early recognition and treatment are needed, to improve patient prognoses. Although clinicians are unfamiliar with postpartum HLH, awareness of the disease is necessary to improve the prognoses of patients with postpartum HLH.