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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

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
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Recent clinical studies

Etiology

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
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
Kose M, Ozturk M, Patiroglu T, Konuskan B
Ann Saudi Med 2008 Sep-Oct;28(5):390-3. doi: 10.5144/0256-4947.2008.390. PMID: 18779633Free PMC Article
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Transfus Med Rev 1994 Oct;8(4):223-31. doi: 10.1016/s0887-7963(94)70114-0. PMID: 7841666

Diagnosis

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
Jundt MC, Ayalew AM, Hartman TE, Roden AC, Koo CW
Am J Respir Crit Care Med 2019 Apr 15;199(8):e35-e36. doi: 10.1164/rccm.201802-0267IM. PMID: 30321488
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
Wladis EJ, Kapila R, Chu DS
Arch Ophthalmol 2005 Jul;123(7):1012. doi: 10.1001/archopht.123.7.1012-a. PMID: 16009850
Wakankar R, Deoskar RB, Rajput AK, Rajan KE
J Assoc Physicians India 2002 Oct;50:1334-5. PMID: 12568230

Therapy

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
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
Gholamin M, Bazi A, Abbaszadegan MR
Curr Opin Hematol 2015 Jan;22(1):46-52. doi: 10.1097/MOH.0000000000000102. PMID: 25463685
Wladis EJ, Kapila R, Chu DS
Arch Ophthalmol 2005 Jul;123(7):1012. doi: 10.1001/archopht.123.7.1012-a. PMID: 16009850
Wakankar R, Deoskar RB, Rajput AK, Rajan KE
J Assoc Physicians India 2002 Oct;50:1334-5. PMID: 12568230

Prognosis

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
Zonios D, Sheikh V, Sereti I
Arthritis Res Ther 2012 Aug 31;14(4):222. doi: 10.1186/ar4027. PMID: 22971990Free PMC Article
Kose M, Ozturk M, Patiroglu T, Konuskan B
Ann Saudi Med 2008 Sep-Oct;28(5):390-3. doi: 10.5144/0256-4947.2008.390. PMID: 18779633Free PMC Article
Wakankar R, Deoskar RB, Rajput AK, Rajan KE
J Assoc Physicians India 2002 Oct;50:1334-5. PMID: 12568230
Bordin G, Ballaré M, Paglino S, Ravanini P, Dulio D, Malosso MC, Boldorini R, Monteverde A
J Intern Med 1996 Jul;240(1):37-41. doi: 10.1046/j.1365-2796.1996.447785000.x. PMID: 8708590

Clinical prediction guides

Zhu W, Lv J, Xie X, Tian C, Liu J, Zhou H, Sun C, Li J, Hu Z, Li X
Immunol Lett 2022 Jan;241:15-22. Epub 2021 Nov 12 doi: 10.1016/j.imlet.2021.11.002. PMID: 34774916
Gholamin M, Bazi A, Abbaszadegan MR
Curr Opin Hematol 2015 Jan;22(1):46-52. doi: 10.1097/MOH.0000000000000102. PMID: 25463685
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
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
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

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