Idiopathic CD4 lymphocytopenia

Background: Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of

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Veröffentlicht in:Allergy and asthma proceedings 2016-11, Vol.37 (6), p.501-504
Hauptverfasser: Brooks, Joel P., Ghaffari, Gisoo
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creator Brooks, Joel P.
Ghaffari, Gisoo
description Background: Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of
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Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of &lt;300 cells/mL or &lt;20% total lymphocyte on multiple occasions. Objective: To increase awareness of ICL and to provide a review of the clinical characteristics, diagnosis, and management of this disease process. Presently, many of these patients receive prophylactic treatment similar to other T-cell deficient conditions, most notably patients with human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome; however, the same indications may not necessarily apply to this patient population because the T cells are not affected by a virus as in HIV. Methods: A literature search of salient articles that describe case reports and case series of ICL and their management were analyzed. Results: A case of ICL with a literature review is presented with emphasis on clinical pearls and pitfalls. The patients with ICL are usually identified with a CD4 count of &lt;200 cells/mL when complications develop. Opportunistic infections are the most common initial manifestations. Current therapies used to increase CD4 levels include interleukin 2, interferon gamma, interleukin 7, and allogeneic hematopoietic stem cell transplantation, with variable results. Conclusion: ICL is a diagnosis of exclusion when there is no identifiable underlying cause for a low CD4 count. Although the nature of the T-cell problem is not the same in ICL and HIV, in the absence of specific guidelines, patients receive the same prophylactic treatment as patients with HIV.</description><identifier>ISSN: 1088-5412</identifier><identifier>EISSN: 1539-6304</identifier><identifier>DOI: 10.2500/aap.2016.37.3992</identifier><identifier>PMID: 27931306</identifier><language>eng</language><publisher>United States: OceanSide Publications, Inc</publisher><subject>antibiotic prophylaxis ; Cd4 Count ; CD4 Lymphocyte Count ; CD4-CD8 Ratio ; CD4-Positive T-Lymphocytes ; Cytology ; Female ; HIV ; Human immunodeficiency virus ; Humans ; Icl ; Idiopathic Cd4 Lymphocytopenia ; Immunodeficiency ; Lentivirus ; Lymphopenia ; Lymphopenia - diagnosis ; Lymphopenia - drug therapy ; Lymphopenia - etiology ; Lymphopenia - prevention &amp; control ; Middle Aged ; Opportunistic Infections ; Retroviridae ; Sulfamethoxazole - therapeutic use ; T-Cells ; Treatment Outcome ; Trimethoprim - therapeutic use</subject><ispartof>Allergy and asthma proceedings, 2016-11, Vol.37 (6), p.501-504</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c463t-c5eca313e01b00989b8eab4e37e8278c0b055460026363b2068a7936b3d553333</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27931306$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Brooks, Joel P.</creatorcontrib><creatorcontrib>Ghaffari, Gisoo</creatorcontrib><title>Idiopathic CD4 lymphocytopenia</title><title>Allergy and asthma proceedings</title><addtitle>Allergy Asthma Proc</addtitle><description>Background: Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of &lt;300 cells/mL or &lt;20% total lymphocyte on multiple occasions. Objective: To increase awareness of ICL and to provide a review of the clinical characteristics, diagnosis, and management of this disease process. Presently, many of these patients receive prophylactic treatment similar to other T-cell deficient conditions, most notably patients with human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome; however, the same indications may not necessarily apply to this patient population because the T cells are not affected by a virus as in HIV. Methods: A literature search of salient articles that describe case reports and case series of ICL and their management were analyzed. Results: A case of ICL with a literature review is presented with emphasis on clinical pearls and pitfalls. The patients with ICL are usually identified with a CD4 count of &lt;200 cells/mL when complications develop. Opportunistic infections are the most common initial manifestations. Current therapies used to increase CD4 levels include interleukin 2, interferon gamma, interleukin 7, and allogeneic hematopoietic stem cell transplantation, with variable results. Conclusion: ICL is a diagnosis of exclusion when there is no identifiable underlying cause for a low CD4 count. Although the nature of the T-cell problem is not the same in ICL and HIV, in the absence of specific guidelines, patients receive the same prophylactic treatment as patients with HIV.</description><subject>antibiotic prophylaxis</subject><subject>Cd4 Count</subject><subject>CD4 Lymphocyte Count</subject><subject>CD4-CD8 Ratio</subject><subject>CD4-Positive T-Lymphocytes</subject><subject>Cytology</subject><subject>Female</subject><subject>HIV</subject><subject>Human immunodeficiency virus</subject><subject>Humans</subject><subject>Icl</subject><subject>Idiopathic Cd4 Lymphocytopenia</subject><subject>Immunodeficiency</subject><subject>Lentivirus</subject><subject>Lymphopenia</subject><subject>Lymphopenia - diagnosis</subject><subject>Lymphopenia - drug therapy</subject><subject>Lymphopenia - etiology</subject><subject>Lymphopenia - prevention &amp; control</subject><subject>Middle Aged</subject><subject>Opportunistic Infections</subject><subject>Retroviridae</subject><subject>Sulfamethoxazole - therapeutic use</subject><subject>T-Cells</subject><subject>Treatment Outcome</subject><subject>Trimethoprim - therapeutic use</subject><issn>1088-5412</issn><issn>1539-6304</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkE1Lw0AQhhdRrFbvnopHL6mz35uj1K-CoAc9D5vttk1Jk5hNhPrr3ZjqTXAuMwzvPAwPIRcUpkwCXFtbTxlQNeV6ytOUHZATKnmaKA7iMM5gTCIFZSNyGsIGgAqu1DEZMZ1yykGdkMl8kVe1bde5u5zdistit63Xldu1Ve3L3J6Ro6Utgj_f9zF5u797nT0mT88P89nNU-KE4m3ipHc2Ej3QDCA1aWa8zYTn2humjYMMpBQKgCmueMZAGRtfUBlfSMljjcnVwK2b6r3zocVtHpwvClv6qgtIjQStjBbwj6jQJuVG91QYoq6pQmj8Eusm39pmhxSwF4hRIPYCkWvsBcaTyZ7eZVu_-D34MRYDL0MgL1e-bC1uqq4poxvMHVbO2xJ77b11_OC6VJHP4oYBUikELvzSdkWLrW1w9YmBmoi8-QM58H6fhO-Krw4DKLRNG3tkfAFPH5du</recordid><startdate>20161101</startdate><enddate>20161101</enddate><creator>Brooks, Joel P.</creator><creator>Ghaffari, Gisoo</creator><general>OceanSide Publications, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7T5</scope><scope>H94</scope></search><sort><creationdate>20161101</creationdate><title>Idiopathic CD4 lymphocytopenia</title><author>Brooks, Joel P. ; Ghaffari, Gisoo</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c463t-c5eca313e01b00989b8eab4e37e8278c0b055460026363b2068a7936b3d553333</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>antibiotic prophylaxis</topic><topic>Cd4 Count</topic><topic>CD4 Lymphocyte Count</topic><topic>CD4-CD8 Ratio</topic><topic>CD4-Positive T-Lymphocytes</topic><topic>Cytology</topic><topic>Female</topic><topic>HIV</topic><topic>Human immunodeficiency virus</topic><topic>Humans</topic><topic>Icl</topic><topic>Idiopathic Cd4 Lymphocytopenia</topic><topic>Immunodeficiency</topic><topic>Lentivirus</topic><topic>Lymphopenia</topic><topic>Lymphopenia - diagnosis</topic><topic>Lymphopenia - drug therapy</topic><topic>Lymphopenia - etiology</topic><topic>Lymphopenia - prevention &amp; control</topic><topic>Middle Aged</topic><topic>Opportunistic Infections</topic><topic>Retroviridae</topic><topic>Sulfamethoxazole - therapeutic use</topic><topic>T-Cells</topic><topic>Treatment Outcome</topic><topic>Trimethoprim - therapeutic use</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Brooks, Joel P.</creatorcontrib><creatorcontrib>Ghaffari, Gisoo</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><jtitle>Allergy and asthma proceedings</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Brooks, Joel P.</au><au>Ghaffari, Gisoo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Idiopathic CD4 lymphocytopenia</atitle><jtitle>Allergy and asthma proceedings</jtitle><addtitle>Allergy Asthma Proc</addtitle><date>2016-11-01</date><risdate>2016</risdate><volume>37</volume><issue>6</issue><spage>501</spage><epage>504</epage><pages>501-504</pages><issn>1088-5412</issn><eissn>1539-6304</eissn><abstract>Background: Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of &lt;300 cells/mL or &lt;20% total lymphocyte on multiple occasions. Objective: To increase awareness of ICL and to provide a review of the clinical characteristics, diagnosis, and management of this disease process. Presently, many of these patients receive prophylactic treatment similar to other T-cell deficient conditions, most notably patients with human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome; however, the same indications may not necessarily apply to this patient population because the T cells are not affected by a virus as in HIV. Methods: A literature search of salient articles that describe case reports and case series of ICL and their management were analyzed. Results: A case of ICL with a literature review is presented with emphasis on clinical pearls and pitfalls. The patients with ICL are usually identified with a CD4 count of &lt;200 cells/mL when complications develop. Opportunistic infections are the most common initial manifestations. Current therapies used to increase CD4 levels include interleukin 2, interferon gamma, interleukin 7, and allogeneic hematopoietic stem cell transplantation, with variable results. Conclusion: ICL is a diagnosis of exclusion when there is no identifiable underlying cause for a low CD4 count. Although the nature of the T-cell problem is not the same in ICL and HIV, in the absence of specific guidelines, patients receive the same prophylactic treatment as patients with HIV.</abstract><cop>United States</cop><pub>OceanSide Publications, Inc</pub><pmid>27931306</pmid><doi>10.2500/aap.2016.37.3992</doi><tpages>4</tpages></addata></record>
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subjects antibiotic prophylaxis
Cd4 Count
CD4 Lymphocyte Count
CD4-CD8 Ratio
CD4-Positive T-Lymphocytes
Cytology
Female
HIV
Human immunodeficiency virus
Humans
Icl
Idiopathic Cd4 Lymphocytopenia
Immunodeficiency
Lentivirus
Lymphopenia
Lymphopenia - diagnosis
Lymphopenia - drug therapy
Lymphopenia - etiology
Lymphopenia - prevention & control
Middle Aged
Opportunistic Infections
Retroviridae
Sulfamethoxazole - therapeutic use
T-Cells
Treatment Outcome
Trimethoprim - therapeutic use
title Idiopathic CD4 lymphocytopenia
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