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CC BY 4.0 · Journal of Child Science 2021; 11(01): e141-e144
DOI: 10.1055/s-0041-1731078
Case Report

Early Detection and Rescue of an Unusual Case of Pediatric Acute Liver Failure Associated with Secondary Hemophagocytic Lymphophagocytosis: Case Report from a Liver Transplant Center in India

Richa Mittal 
1   Department of Pediatric Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India
,
Smita Malhotra
1   Department of Pediatric Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India
,
Nameet Jerath
2   Department of Pediatric Intensive Care, Indraprastha Apollo Hospital, New Delhi, India
,
Amita Mahajan
3   Department of Pediatric Hematooncology, Indraprastha Apollo Hospital, New Delhi, India
,
Anupam Sibal
1   Department of Pediatric Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India
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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, multisystem, potentially fatal clinicopathologic syndrome. HLH presenting predominantly as pediatric acute liver failure (PALF) has been rarely reported. Early recognition is imperative to initiate life-saving treatment but is often hampered due to the rarity of this syndrome, variable clinical presentations, and nonspecific clinical and laboratory findings. In this article, we reported a case of secondary HLH (H1N1 and RSV positive) presenting as PALF from India. A previously healthy 22-month-old boy presented with fever, vomiting, and altered sensorium for 10 days. He had coagulopathy and deranged liver functions. He was evaluated for underlying etiology and managed on lines of PALF. Due to persistent bicytopenia and excessively high ferritin levels, HLH was strongly suspected though he did not fulfill all clinical criteria for the diagnosis of HLH. Presence of seizures and cerebrospinal fluid analysis was suggestive of central nervous system involvement. There was no evidence of primary HLH on genetic evaluation. Real-time polymerase chain reaction amplifications were positive for RSV and influenza A H1N1, confirming the causative triggers. After the administration of immunosuppressants and oseltamivir, the patient's symptoms improved dramatically and he recovered completely. To the best of our knowledge, this is the fourth case reported worldwide till date of successful rescue of ALF in a child associated with HLH completely without resorting to liver transplantation. Clinical vigilance is crucial for possible presence of HLH with varied initial presentations in PALF despite incomplete diagnostic criteria, with detailed etiological workup for commencing life-saving therapy in time.

Keywords

acute liver failure - hemophagocytosis - pediatrics

Authors' Contributions

R.M. supported in data collection, data analysis, and first draft of manuscript. S.M., N.J., and A.M. contributed in editing and preparing second draft of manuscript. A.S. dedicated in supervision, editing. All authors approved of the final manuscript.




Publication History

Received: 16 December 2020

Accepted: 10 April 2021

Article published online:
22 June 2021

© 2021. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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