U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Idiopathic CD4-positive T-lymphocytopenia

MedGen UID:
64647
•Concept ID:
C0206744
•
Disease or Syndrome
Synonyms: CD4 Positive T Lymphocytopenia, Idiopathic; CD4+ T Lymphocytopenia; CD4+ T Lymphocytopenia, Idiopathic; CD4+ T-Lymphocytopenia; CD4+ T-Lymphocytopenia, Idiopathic; CD4+ T-Lymphocytopenias; CD4-Positive T-Lymphocytopenia, Idiopathic; Idiopathic CD4 Positive T-Lymphocytopenia; Idiopathic CD4+ T Lymphocytopenia; Idiopathic CD4+ T-Lymphocytopenia; Idiopathic CD4-Positive T-Lymphocytopenia; T Lymphocytopenia, Idiopathic CD4 Positive; T Lymphocytopenia, Idiopathic CD4+; T-Lymphocytopenia, CD4+; T-Lymphocytopenia, Idiopathic CD4+; T-Lymphocytopenia, Idiopathic CD4-Positive; T-Lymphocytopenias, CD4+
 
Monarch Initiative: MONDO:0005807

Definition

A rare immunodeficiency syndrome characterized by the decrease of the CD4-positive lymphocytes below 300 per cubic millimeter in the absence of identifiable immunodeficiency causes. Patients with this syndrome are at an increased risk of opportunistic infections. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic CD4-positive T-lymphocytopenia

Professional guidelines

PubMed

Idiopathic CD4 lymphocytopenia: Pathogenesis, etiologies, clinical presentations and treatment strategies.
Yarmohammadi H, Cunningham-Rundles C
Ann Allergy Asthma Immunol 2017 Oct;119(4):374-378. doi: 10.1016/j.anai.2017.07.021. PMID: 28958376Free PMC Article
A 6-year treatment experience for pemphigus: retrospective study of 69 Chinese patients.
Bai YX, Zhang LM, Xiao T, Chen HD
Dermatol Ther 2016 Mar-Apr;29(2):84-7. Epub 2015 Nov 2 doi: 10.1111/dth.12302. PMID: 27060935
Newborn screening for SCID: three years of experience.
Chase NM, Verbsky JW, Routes JM
Ann N Y Acad Sci 2011 Nov;1238:99-105. doi: 10.1111/j.1749-6632.2011.06241.x. PMID: 22129057

Recent clinical studies

Etiology

Disseminated Cryptococcosis in Idiopathic CD4+ Lymphocytopenia.
Dewangan A, Singh J, Kumar D, Kumar N, Kumar K, Dinkar A
Infect Disord Drug Targets 2023;23(1):e210622206242. doi: 10.2174/1871526522666220621110723. PMID: 35726810
Prevalence and pathogenicity of autoantibodies in patients with idiopathic CD4 lymphopenia.
Perez-Diez A, Wong CS, Liu X, Mystakelis H, Song J, Lu Y, Sheikh V, Bourgeois JS, Lisco A, Laidlaw E, Cudrici C, Zhu C, Li QZ, Freeman AF, Williamson PR, Anderson M, Roby G, Tsang JS, Siegel R, Sereti I
J Clin Invest 2020 Oct 1;130(10):5326-5337. doi: 10.1172/JCI136254. PMID: 32634122Free PMC Article
Idiopathic CD4 lymphocytopenia: Pathogenesis, etiologies, clinical presentations and treatment strategies.
Yarmohammadi H, Cunningham-Rundles C
Ann Allergy Asthma Immunol 2017 Oct;119(4):374-378. doi: 10.1016/j.anai.2017.07.021. PMID: 28958376Free PMC Article
Cryptococcosis and idiopathic CD4 lymphocytopenia.
Zonios DI, Falloon J, Huang CY, Chaitt D, Bennett JE
Medicine (Baltimore) 2007 Mar;86(2):78-92. doi: 10.1097/md.0b013e31803b52f5. PMID: 17435588
Idiopathic CD4+ T lymphocytopenia: a review and current perspective.
Heredia A, Hewlett IK, Soriano V, Epstein JS
Transfus Med Rev 1994 Oct;8(4):223-31. doi: 10.1016/s0887-7963(94)70114-0. PMID: 7841666

Diagnosis

Disseminated Cryptococcosis in Idiopathic CD4+ Lymphocytopenia.
Dewangan A, Singh J, Kumar D, Kumar N, Kumar K, Dinkar A
Infect Disord Drug Targets 2023;23(1):e210622206242. doi: 10.2174/1871526522666220621110723. PMID: 35726810
Idiopathic CD4+ T lymphocytopenia: A case report.
Umamaheshwari S, Sumana MN, Shetty MS, Gopal S
J Postgrad Med 2020 Apr-Jun;66(2):102-104. doi: 10.4103/jpgm.JPGM_324_19. PMID: 31898594Free PMC Article
Idiopathic CD4 Lymphocytopenia.
Ramalingam PK, Gayathri K, Santhakumar R, Manjunath BV, Karuppuswamy N, Vetriveeran B, Selvamani S, Vishnuram P, Natarajan K
J Assoc Physicians India 2016 May;64(5):81-82. PMID: 27735162
Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells.
Zonios D, Sheikh V, Sereti I
Arthritis Res Ther 2012 Aug 31;14(4):222. doi: 10.1186/ar4027. PMID: 22971990Free PMC Article
Idiopathic CD4 lymphocytopenia and opportunistic infection--an update.
Luo L, Li T
FEMS Immunol Med Microbiol 2008 Dec;54(3):283-9. doi: 10.1111/j.1574-695X.2008.00490.x. PMID: 19049641

Therapy

Progressive multifocal leukoencephalopathy secondary to idiopathic CD4 lymphocytopenia treated with pembrolizumab.
Varmpompiti K, Westwood AJ, Ben-Joseph A, Sibtain N, Ibrahim MAA, Stanton B, Zuckerman M, Hadden R, Ritter LM
J Neuroimmunol 2023 Dec 15;385:578248. Epub 2023 Nov 18 doi: 10.1016/j.jneuroim.2023.578248. PMID: 37995595
Idiopathic CD4 lymphocytopenia in neurological disorders.
Gupta D, Mehta A, Shetty N, R P, Javali M, T Acharya P, Srinivasa R
Clin Neurol Neurosurg 2020 Aug;195:105923. Epub 2020 May 15 doi: 10.1016/j.clineuro.2020.105923. PMID: 32442803
Idiopathic CD4 lymphocytopenia: Pathogenesis, etiologies, clinical presentations and treatment strategies.
Yarmohammadi H, Cunningham-Rundles C
Ann Allergy Asthma Immunol 2017 Oct;119(4):374-378. doi: 10.1016/j.anai.2017.07.021. PMID: 28958376Free PMC Article
Idiopathic lymphocytopenia.
Gholamin M, Bazi A, Abbaszadegan MR
Curr Opin Hematol 2015 Jan;22(1):46-52. doi: 10.1097/MOH.0000000000000102. PMID: 25463685
Cryptococcosis and idiopathic CD4 lymphocytopenia.
Zonios DI, Falloon J, Huang CY, Chaitt D, Bennett JE
Medicine (Baltimore) 2007 Mar;86(2):78-92. doi: 10.1097/md.0b013e31803b52f5. PMID: 17435588

Prognosis

Disseminated Cryptococcosis in Idiopathic CD4+ Lymphocytopenia.
Dewangan A, Singh J, Kumar D, Kumar N, Kumar K, Dinkar A
Infect Disord Drug Targets 2023;23(1):e210622206242. doi: 10.2174/1871526522666220621110723. PMID: 35726810
Idiopathic CD4 lymphocytopenia in neurological disorders.
Gupta D, Mehta A, Shetty N, R P, Javali M, T Acharya P, Srinivasa R
Clin Neurol Neurosurg 2020 Aug;195:105923. Epub 2020 May 15 doi: 10.1016/j.clineuro.2020.105923. PMID: 32442803
Progressive multifocal leukoencephalopathy and idiopathic CD4 lymphocytopenia.
Delgado-Alvarado M, Sedano MJ, González-Quintanilla V, de Lucas EM, Polo JM, Berciano J
J Neurol Sci 2013 Apr 15;327(1-2):75-9. Epub 2013 Mar 5 doi: 10.1016/j.jns.2013.02.002. PMID: 23465508
Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells.
Zonios D, Sheikh V, Sereti I
Arthritis Res Ther 2012 Aug 31;14(4):222. doi: 10.1186/ar4027. PMID: 22971990Free PMC Article
Cryptococcosis and idiopathic CD4 lymphocytopenia.
Zonios DI, Falloon J, Huang CY, Chaitt D, Bennett JE
Medicine (Baltimore) 2007 Mar;86(2):78-92. doi: 10.1097/md.0b013e31803b52f5. PMID: 17435588

Clinical prediction guides

Disseminated cryptococcosis with granuloma formation in idiopathic CD4 lymphocytopenia.
Shimizu H, Hara S, Nishioka H
J Infect Chemother 2020 Feb;26(2):257-260. Epub 2019 Jul 23 doi: 10.1016/j.jiac.2019.07.006. PMID: 31345742
Idiopathic lymphocytopenia.
Gholamin M, Bazi A, Abbaszadegan MR
Curr Opin Hematol 2015 Jan;22(1):46-52. doi: 10.1097/MOH.0000000000000102. PMID: 25463685
Primary Sjögren's syndrome and B-non-Hodgkin lymphoma: role of CD4+ T lymphocytopenia.
Ismail F, Mahmoud A, Abdelhaleem H, Mamdoh A, Geneidy M, Kamal E
Rheumatol Int 2013 Apr;33(4):1021-5. Epub 2012 Aug 12 doi: 10.1007/s00296-012-2464-7. PMID: 22886469
Prolonged CD4 T cell lymphopenia increases morbidity and mortality after renal transplantation.
Ducloux D, Courivaud C, Bamoulid J, Vivet B, Chabroux A, Deschamps M, Rebibou JM, Ferrand C, Chalopin JM, Tiberghien P, Saas P
J Am Soc Nephrol 2010 May;21(5):868-75. Epub 2010 Mar 4 doi: 10.1681/ASN.2009090976. PMID: 20203160Free PMC Article
Cryptococcosis and idiopathic CD4 lymphocytopenia.
Zonios DI, Falloon J, Huang CY, Chaitt D, Bennett JE
Medicine (Baltimore) 2007 Mar;86(2):78-92. doi: 10.1097/md.0b013e31803b52f5. PMID: 17435588

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...