Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases

Idiopathic CD4 lymphocytopenia (ICL) was first defined in 1992 by the US Centers for Disease Control and Prevention (CDC) as the repeated presence of a CD4+ T lymphocyte count of fewer than 300 cells per cubic millimeter or of less than 20% of total T cells with no evidence of human immunodeficiency...

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Autores principales: Dina S Ahmad, Mohammad Esmadi, William C Steinmann
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Publicado: Thieme Medical and Scientific Publishers Pvt. Ltd. 2013
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spelling oai:doaj.org-article:358b6a4db52243f3a5fac7f14441550e2021-12-02T17:59:10ZIdiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases2231-07702249-446410.4103/2231-0770.114121https://doaj.org/article/358b6a4db52243f3a5fac7f14441550e2013-04-01T00:00:00Zhttp://www.thieme-connect.de/DOI/DOI?10.4103/2231-0770.114121https://doaj.org/toc/2231-0770https://doaj.org/toc/2249-4464Idiopathic CD4 lymphocytopenia (ICL) was first defined in 1992 by the US Centers for Disease Control and Prevention (CDC) as the repeated presence of a CD4+ T lymphocyte count of fewer than 300 cells per cubic millimeter or of less than 20% of total T cells with no evidence of human immunodeficiency virus (HIV) infection and no condition that might cause depressed CD4 counts. Most of our knowledge about ICL comes from scattered case reports. The aim of this study was to collect comprehensive data from the previously published cases to understand the characteristics of this rare condition. We searched the PubMed database and Science Direct for case reports since 1989 for Idiopathic CD4 lymphocytopenia cases. We found 258 cases diagnosed with ICL in 143 published papers. We collected data about age, sex, pathogens, site of infections, CD4 count, CD8 count, CD4:CD8 ratio, presence of HIV risk factors, malignancies, autoimmune diseases and whether the patients survived or died. The mean age at diagnosis of first opportunistic infection (or ICL if no opportunistic infection reported) was 40.7 ± 19.2 years (standard deviation), with a range of 1 to 85. One-sixty (62%) patients were males, 91 (35.2%) were females, and 7 (2.7%) patients were not identified whether males or females. Risk factors for HIV were documented in 36 (13.9%) patients. The mean initial CD4 count was 142.6 ± 103.9/mm 3 (standard deviation). The mean initial CD8 count was 295 ± 273.6/mm 3 (standard deviation). The mean initial CD4:CD8 ratio was 0.6 ± 0.7 (standard deviation). The mean lowest CD4 count was 115.4 ± 87.1/mm 3 (standard deviation). The majority of patients 226 (87.6%) had at least one infection. Cryptococcal infections were the most prevalent infections in ICL patients (26.6%), followed by mycobacterial infections (17%), candidal infections (16.2%), and VZV infections (13.1%). Malignancies were reported in 47 (18.1%) patients. Autoimmune diseases were reported in 37 (14.2%) patients.Dina S AhmadMohammad EsmadiWilliam C SteinmannThieme Medical and Scientific Publishers Pvt. Ltd.articleautoimmune diseasesidiopathic cd4 lymphocytopenialymphopeniaopportunistic infectionsMedicineRENAvicenna Journal of Medicine, Vol 03, Iss 02, Pp 37-47 (2013)
institution DOAJ
collection DOAJ
language EN
topic autoimmune diseases
idiopathic cd4 lymphocytopenia
lymphopenia
opportunistic infections
Medicine
R
spellingShingle autoimmune diseases
idiopathic cd4 lymphocytopenia
lymphopenia
opportunistic infections
Medicine
R
Dina S Ahmad
Mohammad Esmadi
William C Steinmann
Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases
description Idiopathic CD4 lymphocytopenia (ICL) was first defined in 1992 by the US Centers for Disease Control and Prevention (CDC) as the repeated presence of a CD4+ T lymphocyte count of fewer than 300 cells per cubic millimeter or of less than 20% of total T cells with no evidence of human immunodeficiency virus (HIV) infection and no condition that might cause depressed CD4 counts. Most of our knowledge about ICL comes from scattered case reports. The aim of this study was to collect comprehensive data from the previously published cases to understand the characteristics of this rare condition. We searched the PubMed database and Science Direct for case reports since 1989 for Idiopathic CD4 lymphocytopenia cases. We found 258 cases diagnosed with ICL in 143 published papers. We collected data about age, sex, pathogens, site of infections, CD4 count, CD8 count, CD4:CD8 ratio, presence of HIV risk factors, malignancies, autoimmune diseases and whether the patients survived or died. The mean age at diagnosis of first opportunistic infection (or ICL if no opportunistic infection reported) was 40.7 ± 19.2 years (standard deviation), with a range of 1 to 85. One-sixty (62%) patients were males, 91 (35.2%) were females, and 7 (2.7%) patients were not identified whether males or females. Risk factors for HIV were documented in 36 (13.9%) patients. The mean initial CD4 count was 142.6 ± 103.9/mm 3 (standard deviation). The mean initial CD8 count was 295 ± 273.6/mm 3 (standard deviation). The mean initial CD4:CD8 ratio was 0.6 ± 0.7 (standard deviation). The mean lowest CD4 count was 115.4 ± 87.1/mm 3 (standard deviation). The majority of patients 226 (87.6%) had at least one infection. Cryptococcal infections were the most prevalent infections in ICL patients (26.6%), followed by mycobacterial infections (17%), candidal infections (16.2%), and VZV infections (13.1%). Malignancies were reported in 47 (18.1%) patients. Autoimmune diseases were reported in 37 (14.2%) patients.
format article
author Dina S Ahmad
Mohammad Esmadi
William C Steinmann
author_facet Dina S Ahmad
Mohammad Esmadi
William C Steinmann
author_sort Dina S Ahmad
title Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases
title_short Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases
title_full Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases
title_fullStr Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases
title_full_unstemmed Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases
title_sort idiopathic cd4 lymphocytopenia: spectrum of opportunistic infections, malignancies, and autoimmune diseases
publisher Thieme Medical and Scientific Publishers Pvt. Ltd.
publishDate 2013
url https://doaj.org/article/358b6a4db52243f3a5fac7f14441550e
work_keys_str_mv AT dinasahmad idiopathiccd4lymphocytopeniaspectrumofopportunisticinfectionsmalignanciesandautoimmunediseases
AT mohammadesmadi idiopathiccd4lymphocytopeniaspectrumofopportunisticinfectionsmalignanciesandautoimmunediseases
AT williamcsteinmann idiopathiccd4lymphocytopeniaspectrumofopportunisticinfectionsmalignanciesandautoimmunediseases
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