Cargando…
Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency
Interleukin 12 receptor beta 1 (IL12Rβ1) deficiency is the most common cause of Mendelian susceptibility to mycobacterial disease (MSMD). MSMD usually predisposes the affected individuals to infections with weakly virulent mycobacteria such as Bacille Calmette-Guérin (BCG), environmental mycobacteri...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10425967/ https://www.ncbi.nlm.nih.gov/pubmed/37588305 http://dx.doi.org/10.7759/cureus.41946 |
_version_ | 1785089953822146560 |
---|---|
author | Alabbas, Ali Alasmari, Badriah G Saeed, Muhammad Al-Tala, Saeed M Abualama, Ayman E |
author_facet | Alabbas, Ali Alasmari, Badriah G Saeed, Muhammad Al-Tala, Saeed M Abualama, Ayman E |
author_sort | Alabbas, Ali |
collection | PubMed |
description | Interleukin 12 receptor beta 1 (IL12Rβ1) deficiency is the most common cause of Mendelian susceptibility to mycobacterial disease (MSMD). MSMD usually predisposes the affected individuals to infections with weakly virulent mycobacteria such as Bacille Calmette-Guérin (BCG), environmental mycobacteria, non-typhoidal Salmonella, and certain other intracellular pathogens. MSMD usually presents with disseminated BCG infection after exposure to the BCG vaccine. Infections with non-typhoidal Salmonella are considered the second most common manifestation of MSMD; however, severe presentation with such organisms is unusual. In this report, we describe a case of a previously healthy infant who was found to have IL12Rβ1 deficiency after she presented with hemophagocytic lymphohistiocytosis (HLH) secondary to severe Salmonella enterica sepsis. This case report highlights the importance of considering the diagnosis of MSMD in any patient presenting with severe non-typhoidal Salmonella infections even in the absence of any exposure to low-virulent mycobacteria. |
format | Online Article Text |
id | pubmed-10425967 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-104259672023-08-16 Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency Alabbas, Ali Alasmari, Badriah G Saeed, Muhammad Al-Tala, Saeed M Abualama, Ayman E Cureus Pediatrics Interleukin 12 receptor beta 1 (IL12Rβ1) deficiency is the most common cause of Mendelian susceptibility to mycobacterial disease (MSMD). MSMD usually predisposes the affected individuals to infections with weakly virulent mycobacteria such as Bacille Calmette-Guérin (BCG), environmental mycobacteria, non-typhoidal Salmonella, and certain other intracellular pathogens. MSMD usually presents with disseminated BCG infection after exposure to the BCG vaccine. Infections with non-typhoidal Salmonella are considered the second most common manifestation of MSMD; however, severe presentation with such organisms is unusual. In this report, we describe a case of a previously healthy infant who was found to have IL12Rβ1 deficiency after she presented with hemophagocytic lymphohistiocytosis (HLH) secondary to severe Salmonella enterica sepsis. This case report highlights the importance of considering the diagnosis of MSMD in any patient presenting with severe non-typhoidal Salmonella infections even in the absence of any exposure to low-virulent mycobacteria. Cureus 2023-07-16 /pmc/articles/PMC10425967/ /pubmed/37588305 http://dx.doi.org/10.7759/cureus.41946 Text en Copyright © 2023, Alabbas 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 | Pediatrics Alabbas, Ali Alasmari, Badriah G Saeed, Muhammad Al-Tala, Saeed M Abualama, Ayman E Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency |
title | Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency |
title_full | Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency |
title_fullStr | Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency |
title_full_unstemmed | Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency |
title_short | Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency |
title_sort | hemophagocytic lymphohistiocytosis (hlh) due to fulminant salmonella sepsis in the setting of il12rβ1 (interleukin 12 receptor beta 1) deficiency |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10425967/ https://www.ncbi.nlm.nih.gov/pubmed/37588305 http://dx.doi.org/10.7759/cureus.41946 |
work_keys_str_mv | AT alabbasali hemophagocyticlymphohistiocytosishlhduetofulminantsalmonellasepsisinthesettingofil12rb1interleukin12receptorbeta1deficiency AT alasmaribadriahg hemophagocyticlymphohistiocytosishlhduetofulminantsalmonellasepsisinthesettingofil12rb1interleukin12receptorbeta1deficiency AT saeedmuhammad hemophagocyticlymphohistiocytosishlhduetofulminantsalmonellasepsisinthesettingofil12rb1interleukin12receptorbeta1deficiency AT altalasaeedm hemophagocyticlymphohistiocytosishlhduetofulminantsalmonellasepsisinthesettingofil12rb1interleukin12receptorbeta1deficiency AT abualamaaymane hemophagocyticlymphohistiocytosishlhduetofulminantsalmonellasepsisinthesettingofil12rb1interleukin12receptorbeta1deficiency |