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Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by a pathologic immune response in the setting of infection, malignancy, acute illness, or any immunological stimulus. Infection is the most common etiology of HLH. HLH involves aberrant activatio...

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Autores principales: Abou-El-Hassan, Hadi, Kantono, Melvin, Bhagat, Ankur, Hu, Janie, Karp, David, Jerome, Renard, Randhawa, Johnny S, Shafer, Drake, Farmand, Farbod
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10104683/
https://www.ncbi.nlm.nih.gov/pubmed/37065339
http://dx.doi.org/10.7759/cureus.36199
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author Abou-El-Hassan, Hadi
Kantono, Melvin
Bhagat, Ankur
Hu, Janie
Karp, David
Jerome, Renard
Randhawa, Johnny S
Shafer, Drake
Farmand, Farbod
author_facet Abou-El-Hassan, Hadi
Kantono, Melvin
Bhagat, Ankur
Hu, Janie
Karp, David
Jerome, Renard
Randhawa, Johnny S
Shafer, Drake
Farmand, Farbod
author_sort Abou-El-Hassan, Hadi
collection PubMed
description Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by a pathologic immune response in the setting of infection, malignancy, acute illness, or any immunological stimulus. Infection is the most common etiology of HLH. HLH involves aberrant activation of lymphocytes and macrophages with resultant hypercytokinemia due to an inappropriately stimulated and ineffective immune response. Here, we present the case of a previously healthy 19-year-old male presenting with hiccups and scleral icterus, who was found to have HLH due to a severe Epstein-Barr virus infection. Despite a morphologically normal bone marrow biopsy, the patient met the diagnostic criteria for HLH, including a low natural killer cell count and elevated soluble interleukin-2 receptor. Notably, ferritin was severely elevated at 85,810 ng/mL. The patient was treated with an induction course of dexamethasone intravenously for eight weeks. Since HLH can progress into multi-organ failure, timely diagnosis and prompt initiation of treatment are critical. Novel disease-modifying therapies and further clinical trials are warranted to treat this potentially fatal immunological disease with multisystem ramifications.
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spelling pubmed-101046832023-04-15 Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups Abou-El-Hassan, Hadi Kantono, Melvin Bhagat, Ankur Hu, Janie Karp, David Jerome, Renard Randhawa, Johnny S Shafer, Drake Farmand, Farbod Cureus Internal Medicine Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by a pathologic immune response in the setting of infection, malignancy, acute illness, or any immunological stimulus. Infection is the most common etiology of HLH. HLH involves aberrant activation of lymphocytes and macrophages with resultant hypercytokinemia due to an inappropriately stimulated and ineffective immune response. Here, we present the case of a previously healthy 19-year-old male presenting with hiccups and scleral icterus, who was found to have HLH due to a severe Epstein-Barr virus infection. Despite a morphologically normal bone marrow biopsy, the patient met the diagnostic criteria for HLH, including a low natural killer cell count and elevated soluble interleukin-2 receptor. Notably, ferritin was severely elevated at 85,810 ng/mL. The patient was treated with an induction course of dexamethasone intravenously for eight weeks. Since HLH can progress into multi-organ failure, timely diagnosis and prompt initiation of treatment are critical. Novel disease-modifying therapies and further clinical trials are warranted to treat this potentially fatal immunological disease with multisystem ramifications. Cureus 2023-03-15 /pmc/articles/PMC10104683/ /pubmed/37065339 http://dx.doi.org/10.7759/cureus.36199 Text en Copyright © 2023, Abou-El-Hassan et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Abou-El-Hassan, Hadi
Kantono, Melvin
Bhagat, Ankur
Hu, Janie
Karp, David
Jerome, Renard
Randhawa, Johnny S
Shafer, Drake
Farmand, Farbod
Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups
title Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups
title_full Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups
title_fullStr Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups
title_full_unstemmed Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups
title_short Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups
title_sort hemophagocytic lymphohistiocytosis caused by a severe epstein-barr virus infection in a young patient presenting with hiccups
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10104683/
https://www.ncbi.nlm.nih.gov/pubmed/37065339
http://dx.doi.org/10.7759/cureus.36199
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