 |
Case Report
Liver recovery in a child with hemophagocytic lymphohistiocytosis-induced acute liver failure
1 Chief Resident of Internal Medicine-Pediatrics, Department of Internal Medicine, Division of Internal Medicine-Pediatrics, MedStar Georgetown University Hospital, Washington, DC, USA
2 Resident Physician, Department of Pediatrics, University of Vermont Children’s Hospital, Burlington, VT, USA
3 Resident Physician, Department of Pediatrics, Inova Children’s Hospital, Falls Church, VA, USA
4 Professor, Departments of Oncology and Pediatrics, Division of Pediatric Adolescent and Young Adult Hematology and Oncology, MedStar Georgetown University Hospital, Washington, DC, USA
5 Assistant Professor, Department of Pediatrics, Division of Pediatric Hematology and Oncology, MedStar Georgetown University Hospital, Washington, DC, USA
6 Assistant Professor, Department of Pediatrics, Division of Pediatric Critical Care Medicine, MedStar Georgetown University Hospital, Washington, DC, USA
Address correspondence to:
Charles Kreisel
MD, 3800 Reservoir Road NW, Washington, DC 20007,
USA
Message to Corresponding Author
Article ID: 100015Z19CK2022
Access full text article on other devices
Access PDF of article on other devices
How to cite this article
Kreisel C, Meshnick A, Carlisle G, Toretsky J, Terao M, Monde A. Liver recovery in a child with hemophagocytic lymphohistiocytosis-induced acute liver failure. J Case Rep Images Pediatr 2022;4(1):1–6.ABSTRACT
Introduction: Hemophagocytic lymphohistiocytosis is a rare condition with dysregulated multi-organ inflammation that may cause acute liver failure. It often presents with non-specific clinical features and can be difficult to diagnose.
Case Report: We present the case of a 2-year-old girl with lethargy, encephalopathy, poor oral intake, vomiting, and jaundice. Her labs indicated acute liver failure with additional findings of bicytopenia and elevated ferritin. Given high concern for hemophagocytic lymphohistiocytosis, we promptly consulted the hematology/oncology, transplant hepatology, and pharmacy services. Bone marrow biopsy initially showed no evidence of hemophagocytosis. Given our high clinical index of suspicion for hemophagocytic lymphohistiocytosis and its high mortality and morbidity, we initiated treatment with high dose dexamethasone, etoposide, and the recently approved biologic drug emapalumab, a monoclonal antibody against interferon gamma, despite not meeting full HLH-2004 diagnostic criteria. One day after treatment initiation, the final review of the bone marrow biopsy showed evidence of hemophagocytosis. Ultimately, with implementation of multidisciplinary rounds, close neurologic examinations, aggressive management of evolving hyperammonemia and cerebral edema, and early initiation of treatment, our patient achieved full liver recovery.
Conclusion: This patient’s presentation emphasizes the importance of having a broad differential when a patient presents with liver failure and cytopenias. Our management of this patient showcases the importance of expedient, multidisciplinary management for a complex critically ill pediatric patient. The patient’s survival and complete liver recovery with the treatment protocol given suggests emapalumab should be studied in future clinical trials as an important adjunctive treatment for patients with hemophagocytic lymphohistiocytosis with acute liver failure.
Keywords: Acute liver failure, Emapalumab, Hemophagocytic lymphohistiocytosis
SUPPORTING INFORMATION
Author Contributions
Charles Kreisel - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Andrew Meshnick - Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Gregory Carlisle - Acquisition of data, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Jeffrey Toretsky - Conception of the work, Design of the work, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Michael Terao - Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Alexandra Monde - Conception of the work, Design of the work, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2022 Charles Kreisel et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
