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Decreased proportion of CD4+CD25+ regulatory T cells

MedGen UID:
1688709
Concept ID:
C5139086
Finding
Synonyms: Decreased proportion of CD4+CD25+ Treg cells; Reduced proportion of CD4+CD25+ regulatory T cells
 
HPO: HP:0020113

Definition

An abnormally decreased proportion of CD4-positive, CD25-positive, alpha-beta regulatory T cells in circulation, relative to another population of cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDecreased proportion of CD4+CD25+ regulatory T cells

Conditions with this feature

Immunodeficiency due to CD25 deficiency
MedGen UID:
377894
Concept ID:
C1853392
Disease or Syndrome
Immunodeficiency-41 is an autosomal recessive complex disorder of immune dysregulation. Affected individuals present in infancy with recurrent viral, fungal, and bacterial infections, lymphadenopathy, and variable autoimmune features, such as autoimmune enteropathy and eczematous skin lesions. Immunologic studies show a defect in T-cell regulation (summary by Goudy et al., 2013).
Severe combined immunodeficiency due to CARD11 deficiency
MedGen UID:
767600
Concept ID:
C3554686
Disease or Syndrome
Immunodeficiency-11A is an autosomal recessive primary immunodeficiency characterized by normal numbers of T and B lymphocytes, but defective intracellular signaling. There is a block in B-cell differentiation with increased numbers of transitional B cells and hypogammaglobulinemia, as well as decreased numbers of regulatory T cells and defects in T-cell function (summary by Greil et al., 2013 and Stepensky et al., 2013).
Immunodeficiency 97 with autoinflammation
MedGen UID:
1802936
Concept ID:
C5676946
Disease or Syndrome
Immunodeficiency-97 with autoinflammation (IMD97) is an autosomal recessive complex immunologic disorder with variable features. Affected individuals present in the first decade of life with inflammatory interstitial lung disease or colitis due to abnormal tissue infiltration by activated T cells. Patients develop autoimmune cytopenias and may have lymphadenopathy; 1 reported patient had features of hemophagocytic lymphohistiocytosis (HLH; see FHL1, 267700). Some patients may have recurrent infections associated with mild lymphopenia, hypogammaglobulinemia, and NK cell dysfunction. Immunologic workup indicates signs of significant immune dysregulation with elevation of inflammatory serum markers, variable immune cell defects involving neutrophils, NK cells, and myeloid cells, and disrupted levels of T regulatory cells (Tregs). Two unrelated patients have been reported (summary by Takeda et al., 2019 and Thian et al., 2020).
Immunodeficiency 99 with hypogammaglobulinemia and autoimmune cytopenias
MedGen UID:
1801342
Concept ID:
C5676971
Disease or Syndrome
Immunodeficiency-99 with hypogammaglobulinemia and autoimmune cytopenias (IMD99) is an autosomal recessive immunologic disorder characterized by the onset of recurrent sinopulmonary infections in early childhood. Laboratory studies reveal hypogammaglobulinemia with decreased memory B cells that show impaired class-switch recombination (CSR) and decreased somatic hypermutation (SHM). Due to abnormal antibody production and impaired self-tolerance, patients may develop autoimmune cytopenias, such as thrombocytopenia, or autoimmune features, such as vitiligo. There are also defects in the T-cell compartment (Kuhny et al., 2020).

Professional guidelines

PubMed

Zhang J, Yu M, Li X, Huang X, Wang H
Oral Surg Oral Med Oral Pathol Oral Radiol 2018 Oct;126(4):307-316. Epub 2018 May 29 doi: 10.1016/j.oooo.2018.05.004. PMID: 29958936
Shen L, Shi H, Gao Y, Ou Q, Liu Q, Liu Y, Wu J, Zhang W, Fan L, Shao L
Tuberculosis (Edinb) 2016 Dec;101:146-150. Epub 2016 Oct 1 doi: 10.1016/j.tube.2016.10.001. PMID: 27865385
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Arthritis Res Ther 2015 Jan 21;17(1):10. doi: 10.1186/s13075-015-0526-4. PMID: 25604867Free PMC Article

Recent clinical studies

Etiology

Alves LHV, Ito JT, Almeida FM, Oliveira LM, Stelmach R, Tibério LFLC, Sato MN, Lopes FDTQS
Int Immunopharmacol 2024 Oct 25;140:112765. Epub 2024 Jul 30 doi: 10.1016/j.intimp.2024.112765. PMID: 39083931
Yong F, Wang H, Li C, Liu W, Wang Z, Jia H
Cell Mol Biol (Noisy-le-grand) 2023 Aug 31;69(8):214-220. doi: 10.14715/cmb/2023.69.8.33. PMID: 37715379
Zahran AM, Saad K, Elsayh KI, Khalaf SM, Mahmoud KH, Elhoufey A, Hetta HF
Pediatr Res 2022 Apr;91(5):1203-1206. Epub 2021 Jun 18 doi: 10.1038/s41390-021-01627-y. PMID: 34145401
Jia W, Fu ZL, Wang X, Luo J, Yan CL, Cao JP, Yan-Liu, Xie JF, Liu GY, Gao C, Li XF
Front Immunol 2021;12:768244. Epub 2021 Dec 23 doi: 10.3389/fimmu.2021.768244. PMID: 35006213Free PMC Article
Hu Y, Tian W, Zhang LL, Liu H, Yin GP, He BS, Mao XM
Eur J Endocrinol 2012 Apr;166(4):641-6. Epub 2012 Jan 4 doi: 10.1530/EJE-11-0879. PMID: 22219499

Diagnosis

Jia W, Fu ZL, Wang X, Luo J, Yan CL, Cao JP, Yan-Liu, Xie JF, Liu GY, Gao C, Li XF
Front Immunol 2021;12:768244. Epub 2021 Dec 23 doi: 10.3389/fimmu.2021.768244. PMID: 35006213Free PMC Article
Eriksson Ström J, Pourazar J, Linder R, Blomberg A, Lindberg A, Bucht A, Behndig AF
Respir Res 2020 Dec 14;21(1):330. doi: 10.1186/s12931-020-01593-9. PMID: 33317530Free PMC Article
Lai NL, Zhang SX, Wang J, Zhang JQ, Wang CH, Gao C, Li XF
J Immunol Res 2019;2019:1058738. Epub 2019 Oct 23 doi: 10.1155/2019/1058738. PMID: 31772947Free PMC Article
Zhang J, Zhang Q, Li Y, Tao L, Wu F, Shen Y, Tao Q, Xu X, Wu C, Ruan Y, Wang J, Wang J, Wang Y, Zhai Z
Hematology 2018 Sep;23(8):510-516. Epub 2018 Feb 6 doi: 10.1080/10245332.2018.1435021. PMID: 29409398
Gong Y, Zhao C, Zhao P, Wang M, Zhou G, Han F, Cui Y, Qian J, Zhang H, Xiong H, Sheng J, Jiang T
Dig Dis Sci 2015 May;60(5):1308-14. Epub 2014 Sep 27 doi: 10.1007/s10620-014-3358-1. PMID: 25260658

Therapy

Yong F, Wang H, Li C, Liu W, Wang Z, Jia H
Cell Mol Biol (Noisy-le-grand) 2023 Aug 31;69(8):214-220. doi: 10.14715/cmb/2023.69.8.33. PMID: 37715379
Eriksson Ström J, Pourazar J, Linder R, Blomberg A, Lindberg A, Bucht A, Behndig AF
Respir Res 2020 Dec 14;21(1):330. doi: 10.1186/s12931-020-01593-9. PMID: 33317530Free PMC Article
Ma Y, Li J, Wang H, Chiu Y, Kingsley CV, Fry D, Delaney SN, Wei SC, Zhang J, Maitra A, Yee C
Gastroenterology 2020 Jul;159(1):306-319.e12. Epub 2020 Mar 14 doi: 10.1053/j.gastro.2020.03.018. PMID: 32179091Free PMC Article
Lai NL, Zhang SX, Wang J, Zhang JQ, Wang CH, Gao C, Li XF
J Immunol Res 2019;2019:1058738. Epub 2019 Oct 23 doi: 10.1155/2019/1058738. PMID: 31772947Free PMC Article
Wei K, Jiang BC, Guan JH, Zhang DN, Zhang MX, Wu JL, Zhu GZ
Viral Immunol 2018 Oct;31(8):559-567. Epub 2018 Aug 1 doi: 10.1089/vim.2018.0055. PMID: 30067145

Prognosis

Yong F, Wang H, Li C, Liu W, Wang Z, Jia H
Cell Mol Biol (Noisy-le-grand) 2023 Aug 31;69(8):214-220. doi: 10.14715/cmb/2023.69.8.33. PMID: 37715379
Ni H, Zhang H, Li L, Huang H, Guo H, Zhang L, Li C, Xu JX, Nie CP, Li K, Zhang X, Xia X, Li J
J Immunother Cancer 2022 Sep;10(9) doi: 10.1136/jitc-2022-005151. PMID: 36126994Free PMC Article
Lin L, Chen S, Wang H, Gao B, Kallakury B, Bhuvaneshwar K, Cahn K, Gusev Y, Wang X, Wu Y, Marshall JL, Zhi X, He AR
Theranostics 2021;11(9):4232-4250. Epub 2021 Feb 20 doi: 10.7150/thno.49819. PMID: 33754058Free PMC Article
Hu Y, Tian W, Zhang LL, Liu H, Yin GP, He BS, Mao XM
Eur J Endocrinol 2012 Apr;166(4):641-6. Epub 2012 Jan 4 doi: 10.1530/EJE-11-0879. PMID: 22219499
Zhang D, Wang S, Guan Y, Wang L, Xie W, Li N, Zhao P, Su G
J Cardiovasc Pharmacol 2011 May;57(5):536-41. doi: 10.1097/FJC.0b013e318211d016. PMID: 21297491

Clinical prediction guides

Ni H, Zhang H, Li L, Huang H, Guo H, Zhang L, Li C, Xu JX, Nie CP, Li K, Zhang X, Xia X, Li J
J Immunother Cancer 2022 Sep;10(9) doi: 10.1136/jitc-2022-005151. PMID: 36126994Free PMC Article
Jia W, Fu ZL, Wang X, Luo J, Yan CL, Cao JP, Yan-Liu, Xie JF, Liu GY, Gao C, Li XF
Front Immunol 2021;12:768244. Epub 2021 Dec 23 doi: 10.3389/fimmu.2021.768244. PMID: 35006213Free PMC Article
Shimojima Y, Ichikawa T, Kishida D, Takamatsu R, Sekijima Y
Clin Exp Immunol 2021 Nov;206(2):184-195. Epub 2021 Aug 12 doi: 10.1111/cei.13648. PMID: 34319596Free PMC Article
Lin L, Chen S, Wang H, Gao B, Kallakury B, Bhuvaneshwar K, Cahn K, Gusev Y, Wang X, Wu Y, Marshall JL, Zhi X, He AR
Theranostics 2021;11(9):4232-4250. Epub 2021 Feb 20 doi: 10.7150/thno.49819. PMID: 33754058Free PMC Article
Wang D, Huang S, Yuan X, Liang J, Xu R, Yao G, Feng X, Sun L
Cell Mol Immunol 2017 May;14(5):423-431. Epub 2015 Oct 5 doi: 10.1038/cmi.2015.89. PMID: 26435067Free PMC Article

Recent systematic reviews

Huang Z, Nie S, Wang H, Yan W, Tian D, Liu M
Int Immunopharmacol 2023 Sep;122:110576. Epub 2023 Jun 28 doi: 10.1016/j.intimp.2023.110576. PMID: 37390643
Su QY, Zhang SX, Yang L, Luo J, Li XF, Zhang JQ, Zhang Y, Liu JQ, Shi L
Adv Ther 2023 Jan;40(1):102-116. Epub 2022 Oct 26 doi: 10.1007/s12325-022-02337-5. PMID: 36287319
Li M, Zhou X, Zhou L, Yu Z, Fu L, Yang P
Biomed Res Int 2020;2020:8709804. Epub 2020 Feb 19 doi: 10.1155/2020/8709804. PMID: 32149142Free PMC Article
Li YF, Zhang SX, Ma XW, Xue YL, Gao C, Li XY, Xu AD
Mult Scler Relat Disord 2019 Feb;28:75-80. Epub 2018 Dec 14 doi: 10.1016/j.msard.2018.12.019. PMID: 30572285

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