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Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease

Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological condition resulting from dysregulation of the immune system. This unusual clinical syndrome is characterized by fever, cytopenia, liver dysfunction, increased ferritin level, and evidence of hemophagocytosis in the bone marrow. We repo...

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Autores principales: Barman, Bhupen, Jamil, Md, Kalita, Pranjal, Dey, Biswajit
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8520583/
https://www.ncbi.nlm.nih.gov/pubmed/34671525
http://dx.doi.org/10.7759/cureus.18037
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author Barman, Bhupen
Jamil, Md
Kalita, Pranjal
Dey, Biswajit
author_facet Barman, Bhupen
Jamil, Md
Kalita, Pranjal
Dey, Biswajit
author_sort Barman, Bhupen
collection PubMed
description Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological condition resulting from dysregulation of the immune system. This unusual clinical syndrome is characterized by fever, cytopenia, liver dysfunction, increased ferritin level, and evidence of hemophagocytosis in the bone marrow. We report a case of a 21-year-old female who presented with recurrent high-grade fever, transient rash, and polyarthritis who was subsequently diagnosed with adult-onset Still’s disease (AOSD) with secondary HLH. The patient improved with aggressive management. Our case highlights HLH as a life-threatening and underdiagnosed complication of AOSD.
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spelling pubmed-85205832021-10-19 Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease Barman, Bhupen Jamil, Md Kalita, Pranjal Dey, Biswajit Cureus Internal Medicine Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological condition resulting from dysregulation of the immune system. This unusual clinical syndrome is characterized by fever, cytopenia, liver dysfunction, increased ferritin level, and evidence of hemophagocytosis in the bone marrow. We report a case of a 21-year-old female who presented with recurrent high-grade fever, transient rash, and polyarthritis who was subsequently diagnosed with adult-onset Still’s disease (AOSD) with secondary HLH. The patient improved with aggressive management. Our case highlights HLH as a life-threatening and underdiagnosed complication of AOSD. Cureus 2021-09-17 /pmc/articles/PMC8520583/ /pubmed/34671525 http://dx.doi.org/10.7759/cureus.18037 Text en Copyright © 2021, Barman 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
Barman, Bhupen
Jamil, Md
Kalita, Pranjal
Dey, Biswajit
Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease
title Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease
title_full Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease
title_fullStr Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease
title_full_unstemmed Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease
title_short Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease
title_sort hemophagocytic lymphohistiocytosis secondary to adult-onset still’s disease
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8520583/
https://www.ncbi.nlm.nih.gov/pubmed/34671525
http://dx.doi.org/10.7759/cureus.18037
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AT deybiswajit hemophagocyticlymphohistiocytosissecondarytoadultonsetstillsdisease