Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome marked by excessive immune activation. It can be triggered by various factors, including infections, malignancies, and autoimmune diseases, making the diagnosis challenging due to its overlap...

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Main Authors: Rita Noversa de Sousa, Andreia Sá Lima, Susana Viana, Filipa Guimarães, Marta Pereira, Luís Miguel Afonso
Format: Article
Language:English
Published: SMC MEDIA SRL 2024-12-01
Series:European Journal of Case Reports in Internal Medicine
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Online Access:https://www.ejcrim.com/index.php/EJCRIM/article/view/5040
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author Rita Noversa de Sousa
Andreia Sá Lima
Susana Viana
Filipa Guimarães
Marta Pereira
Luís Miguel Afonso
author_facet Rita Noversa de Sousa
Andreia Sá Lima
Susana Viana
Filipa Guimarães
Marta Pereira
Luís Miguel Afonso
author_sort Rita Noversa de Sousa
collection DOAJ
description Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome marked by excessive immune activation. It can be triggered by various factors, including infections, malignancies, and autoimmune diseases, making the diagnosis challenging due to its overlap with other severe conditions. Case reports: We discuss two intensive care unit (ICU) cases illustrating the diverse manifestations of HLH and the critical importance of early recognition and treatment. The first case involves natural killer-cell leukaemia, and the second, a suspected viral trigger. Both highlight the necessity of a multidisciplinary approach in diagnosis and management, emphasizing the complexity of HLH in ICU settings. Conclusions: High mortality rates, particularly in malignancy-associated HLH, underscore the importance of tailored treatment strategies based on the underlying aetiology.
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institution Kabale University
issn 2284-2594
language English
publishDate 2024-12-01
publisher SMC MEDIA SRL
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series European Journal of Case Reports in Internal Medicine
spelling doaj-art-039b93b0dd6e4420b7934751b231d7172025-01-07T13:40:28ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942024-12-0110.12890/2024_0050404575Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experienceRita Noversa de Sousa0Andreia Sá Lima1Susana Viana2Filipa Guimarães3Marta Pereira4Luís Miguel Afonso5Internal Medicine Service, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PortugalInternal Medicine Service, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PortugalInternal Medicine Service, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PortugalIntensive Care Unit, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PortugalIntensive Care Unit, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PortugalIntensive Care Unit, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PortugalBackground: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome marked by excessive immune activation. It can be triggered by various factors, including infections, malignancies, and autoimmune diseases, making the diagnosis challenging due to its overlap with other severe conditions. Case reports: We discuss two intensive care unit (ICU) cases illustrating the diverse manifestations of HLH and the critical importance of early recognition and treatment. The first case involves natural killer-cell leukaemia, and the second, a suspected viral trigger. Both highlight the necessity of a multidisciplinary approach in diagnosis and management, emphasizing the complexity of HLH in ICU settings. Conclusions: High mortality rates, particularly in malignancy-associated HLH, underscore the importance of tailored treatment strategies based on the underlying aetiology.https://www.ejcrim.com/index.php/EJCRIM/article/view/5040hemophagocytic lymphohistiocytosisepstein-barr virusnk-leukemiaorganizing pneumoniaviral infections
spellingShingle Rita Noversa de Sousa
Andreia Sá Lima
Susana Viana
Filipa Guimarães
Marta Pereira
Luís Miguel Afonso
Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience
European Journal of Case Reports in Internal Medicine
hemophagocytic lymphohistiocytosis
epstein-barr virus
nk-leukemia
organizing pneumonia
viral infections
title Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience
title_full Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience
title_fullStr Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience
title_full_unstemmed Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience
title_short Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience
title_sort prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis insights from an intensive care unit experience
topic hemophagocytic lymphohistiocytosis
epstein-barr virus
nk-leukemia
organizing pneumonia
viral infections
url https://www.ejcrim.com/index.php/EJCRIM/article/view/5040
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AT filipaguimaraes prognosticimpactofaetiologyinadulthemophagocyticlymphohistiocytosisinsightsfromanintensivecareunitexperience
AT martapereira prognosticimpactofaetiologyinadulthemophagocyticlymphohistiocytosisinsightsfromanintensivecareunitexperience
AT luismiguelafonso prognosticimpactofaetiologyinadulthemophagocyticlymphohistiocytosisinsightsfromanintensivecareunitexperience