Cargando…
Secondary hemophagocytic lymphohistiocytosis in children (Review)
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in t...
Autores principales: | , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
D.A. Spandidos
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10433411/ https://www.ncbi.nlm.nih.gov/pubmed/37602304 http://dx.doi.org/10.3892/etm.2023.12122 |
_version_ | 1785091641812451328 |
---|---|
author | Benevenuta, Chiara Mussinatto, Ilaria Orsi, Cecilia Timeus, Fabio S. |
author_facet | Benevenuta, Chiara Mussinatto, Ilaria Orsi, Cecilia Timeus, Fabio S. |
author_sort | Benevenuta, Chiara |
collection | PubMed |
description | Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in the absence of an aggressive therapeutic approach. The present literature review focuses on secondary HLH at pediatric age, which represents a heterogeneous group in terms of etiology and therapeutic approach. It summarizes the most recent evidence on epidemiology, pathophysiology, diagnosis, treatment and prognosis, and provides a detailed description and comparison of the major subtypes of secondary HLH. Finally, it addresses the open questions with a focus on diagnosis and new treatment insights. |
format | Online Article Text |
id | pubmed-10433411 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | D.A. Spandidos |
record_format | MEDLINE/PubMed |
spelling | pubmed-104334112023-08-18 Secondary hemophagocytic lymphohistiocytosis in children (Review) Benevenuta, Chiara Mussinatto, Ilaria Orsi, Cecilia Timeus, Fabio S. Exp Ther Med Review Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in the absence of an aggressive therapeutic approach. The present literature review focuses on secondary HLH at pediatric age, which represents a heterogeneous group in terms of etiology and therapeutic approach. It summarizes the most recent evidence on epidemiology, pathophysiology, diagnosis, treatment and prognosis, and provides a detailed description and comparison of the major subtypes of secondary HLH. Finally, it addresses the open questions with a focus on diagnosis and new treatment insights. D.A. Spandidos 2023-07-17 /pmc/articles/PMC10433411/ /pubmed/37602304 http://dx.doi.org/10.3892/etm.2023.12122 Text en Copyright: © Benevenuta et al. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. |
spellingShingle | Review Benevenuta, Chiara Mussinatto, Ilaria Orsi, Cecilia Timeus, Fabio S. Secondary hemophagocytic lymphohistiocytosis in children (Review) |
title | Secondary hemophagocytic lymphohistiocytosis in children (Review) |
title_full | Secondary hemophagocytic lymphohistiocytosis in children (Review) |
title_fullStr | Secondary hemophagocytic lymphohistiocytosis in children (Review) |
title_full_unstemmed | Secondary hemophagocytic lymphohistiocytosis in children (Review) |
title_short | Secondary hemophagocytic lymphohistiocytosis in children (Review) |
title_sort | secondary hemophagocytic lymphohistiocytosis in children (review) |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10433411/ https://www.ncbi.nlm.nih.gov/pubmed/37602304 http://dx.doi.org/10.3892/etm.2023.12122 |
work_keys_str_mv | AT benevenutachiara secondaryhemophagocyticlymphohistiocytosisinchildrenreview AT mussinattoilaria secondaryhemophagocyticlymphohistiocytosisinchildrenreview AT orsicecilia secondaryhemophagocyticlymphohistiocytosisinchildrenreview AT timeusfabios secondaryhemophagocyticlymphohistiocytosisinchildrenreview |