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Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells

Idiopathic CD4 lymphocytopenia (ICL) is a presumed heterogenous syndrome with key element low CD4 T-cell counts (below 300/mm(3)) without evidence of HIV infection or other known immunodeficiency. The etiology, pathogenesis, and management of ICL remain poorly understood and inadequately defined. Th...

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Autores principales: Zonios, Dimitrios, Sheikh, Virginia, Sereti, Irini
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3580591/
https://www.ncbi.nlm.nih.gov/pubmed/22971990
http://dx.doi.org/10.1186/ar4027
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author Zonios, Dimitrios
Sheikh, Virginia
Sereti, Irini
author_facet Zonios, Dimitrios
Sheikh, Virginia
Sereti, Irini
author_sort Zonios, Dimitrios
collection PubMed
description Idiopathic CD4 lymphocytopenia (ICL) is a presumed heterogenous syndrome with key element low CD4 T-cell counts (below 300/mm(3)) without evidence of HIV infection or other known immunodeficiency. The etiology, pathogenesis, and management of ICL remain poorly understood and inadequately defined. The clinical presentation can range from serious opportunistic infections to incidentally diagnosed asymptomatic individuals. Cryptococcal and non-tuberculous mycobacterial infections and progressive multifocal leukoencephalopathy are the most significant presenting infections, although the spectrum of opportunistic diseases can be similar to that in patients with lymphopenia and HIV infection. Malignancy is common and related to opportunistic pathogens with an oncogenic potential. Autoimmune diseases are also seen in ICL with an increased incidence. The etiology of ICL is unknown. Mechanisms implicated in CD4 reduction may include decreased production, increased destruction, and tissue sequestration. New distinct genetic defects have been identified in certain patients with ICL, supporting the hypothesis of the lack of a common etiology in this syndrome. The management of ICL is focused on the treatment of opportunistic infections, appropriate prophylactic antibiotics, and close monitoring. In selected patients with life-threatening infections or profound immunodeficiency, strategies to increase T-cell counts or enhance immune function could be considered and have included interleukin-2, interferon-gamma, interleukin-7, and hematopoietic stem cell transplantation. The prognosis is influenced by the accompanying opportunistic infections and may be affected by publication bias of severe cases with unfavorable outcomes. As newer laboratory investigation techniques are being developed and targeted experimental treatments become available, our comprehension and prognosis of this rare syndrome could be significantly improved.
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spelling pubmed-35805912013-03-03 Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells Zonios, Dimitrios Sheikh, Virginia Sereti, Irini Arthritis Res Ther Review Idiopathic CD4 lymphocytopenia (ICL) is a presumed heterogenous syndrome with key element low CD4 T-cell counts (below 300/mm(3)) without evidence of HIV infection or other known immunodeficiency. The etiology, pathogenesis, and management of ICL remain poorly understood and inadequately defined. The clinical presentation can range from serious opportunistic infections to incidentally diagnosed asymptomatic individuals. Cryptococcal and non-tuberculous mycobacterial infections and progressive multifocal leukoencephalopathy are the most significant presenting infections, although the spectrum of opportunistic diseases can be similar to that in patients with lymphopenia and HIV infection. Malignancy is common and related to opportunistic pathogens with an oncogenic potential. Autoimmune diseases are also seen in ICL with an increased incidence. The etiology of ICL is unknown. Mechanisms implicated in CD4 reduction may include decreased production, increased destruction, and tissue sequestration. New distinct genetic defects have been identified in certain patients with ICL, supporting the hypothesis of the lack of a common etiology in this syndrome. The management of ICL is focused on the treatment of opportunistic infections, appropriate prophylactic antibiotics, and close monitoring. In selected patients with life-threatening infections or profound immunodeficiency, strategies to increase T-cell counts or enhance immune function could be considered and have included interleukin-2, interferon-gamma, interleukin-7, and hematopoietic stem cell transplantation. The prognosis is influenced by the accompanying opportunistic infections and may be affected by publication bias of severe cases with unfavorable outcomes. As newer laboratory investigation techniques are being developed and targeted experimental treatments become available, our comprehension and prognosis of this rare syndrome could be significantly improved. BioMed Central 2012 2012-08-31 /pmc/articles/PMC3580591/ /pubmed/22971990 http://dx.doi.org/10.1186/ar4027 Text en Copyright ©2012 BioMed Central Ltd
spellingShingle Review
Zonios, Dimitrios
Sheikh, Virginia
Sereti, Irini
Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells
title Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells
title_full Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells
title_fullStr Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells
title_full_unstemmed Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells
title_short Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells
title_sort idiopathic cd4 lymphocytopenia: a case of missing, wandering or ineffective t cells
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3580591/
https://www.ncbi.nlm.nih.gov/pubmed/22971990
http://dx.doi.org/10.1186/ar4027
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