Cargando…
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...
Autores principales: | , , , |
---|---|
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 |
_version_ | 1784584699291631616 |
---|---|
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. |
format | Online Article Text |
id | pubmed-8520583 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT barmanbhupen hemophagocyticlymphohistiocytosissecondarytoadultonsetstillsdisease AT jamilmd hemophagocyticlymphohistiocytosissecondarytoadultonsetstillsdisease AT kalitapranjal hemophagocyticlymphohistiocytosissecondarytoadultonsetstillsdisease AT deybiswajit hemophagocyticlymphohistiocytosissecondarytoadultonsetstillsdisease |