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Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome in which widespread activation of the immune system causes multi-organ tissue damage. HLH is a serious and potentially fatal disorder characterized by excessive immune system activation. It is characterized by a dysregulation in natural kil...

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Autores principales: Hofstrand, Reese, Casey, Bradley, Onyeaso, Elizabeth
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10418134/
https://www.ncbi.nlm.nih.gov/pubmed/37575709
http://dx.doi.org/10.7759/cureus.41779
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author Hofstrand, Reese
Casey, Bradley
Onyeaso, Elizabeth
author_facet Hofstrand, Reese
Casey, Bradley
Onyeaso, Elizabeth
author_sort Hofstrand, Reese
collection PubMed
description Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome in which widespread activation of the immune system causes multi-organ tissue damage. HLH is a serious and potentially fatal disorder characterized by excessive immune system activation. It is characterized by a dysregulation in natural killer (NK) T-cell function, causing activation of lymphocytes and histiocytes. These cells secrete a large number of inflammatory cytokines and infiltrate various tissues causing multi-organ system failure. The spectrum consists of hereditary or “primary” HLH that comprises genetically heterogeneous conditions, occurring during childhood. The secondary form presents later in life and is associated with several conditions mainly malignancy, autoimmune diseases, viral or bacterial infections, and hematological diseases. Here we present an interesting case in which a 39-year-old patient presented with a complaint of shortness of breath. He was diagnosed with obstructive uropathy in the emergency department and subsequently developed acute liver injury, acute kidney injury, Escherichia coli bacteremia, and was diagnosed with HLH with comorbid bacteremia.
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spelling pubmed-104181342023-08-12 Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia Hofstrand, Reese Casey, Bradley Onyeaso, Elizabeth Cureus Gastroenterology Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome in which widespread activation of the immune system causes multi-organ tissue damage. HLH is a serious and potentially fatal disorder characterized by excessive immune system activation. It is characterized by a dysregulation in natural killer (NK) T-cell function, causing activation of lymphocytes and histiocytes. These cells secrete a large number of inflammatory cytokines and infiltrate various tissues causing multi-organ system failure. The spectrum consists of hereditary or “primary” HLH that comprises genetically heterogeneous conditions, occurring during childhood. The secondary form presents later in life and is associated with several conditions mainly malignancy, autoimmune diseases, viral or bacterial infections, and hematological diseases. Here we present an interesting case in which a 39-year-old patient presented with a complaint of shortness of breath. He was diagnosed with obstructive uropathy in the emergency department and subsequently developed acute liver injury, acute kidney injury, Escherichia coli bacteremia, and was diagnosed with HLH with comorbid bacteremia. Cureus 2023-07-12 /pmc/articles/PMC10418134/ /pubmed/37575709 http://dx.doi.org/10.7759/cureus.41779 Text en Copyright © 2023, Hofstrand 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 Gastroenterology
Hofstrand, Reese
Casey, Bradley
Onyeaso, Elizabeth
Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia
title Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia
title_full Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia
title_fullStr Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia
title_full_unstemmed Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia
title_short Hemophagocytic Lymphohistiocytosis Secondary to Obstructive Uropathy and Escherichia coli Bacteremia
title_sort hemophagocytic lymphohistiocytosis secondary to obstructive uropathy and escherichia coli bacteremia
topic Gastroenterology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10418134/
https://www.ncbi.nlm.nih.gov/pubmed/37575709
http://dx.doi.org/10.7759/cureus.41779
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