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T-cell immunodeficiency

MedGen UID:: 226894
•Concept ID:: C1274233
•: Disease or Syndrome

Synonyms:	T lymphocyte deficiency; T-Cell Immunodeficiency; T-lymphocyte deficiency (finding); T-lymphocyte immunodeficiency
SNOMED CT:	T-lymphocyte immunodeficiency (402792003)

Monarch Initiative:	MONDO:0003780

Definition

A broad classification of disorders that affect the cell-mediated aspect of the immune response. Circulating numbers of T lymphocytes are decreased or ineffective. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVT-cell immunodeficiency

Phenotypic abnormality
- Abnormality of the immune system
  - Abnormality of immune system physiology
    - Immunodeficiency
      - T-cell immunodeficiency
        AIDS
        Congenital T-cell immunodeficiency
        DiGeorge syndrome
        Idiopathic CD4-positive T-lymphocytopenia

Professional guidelines

PubMed

Nonsevere combined immunodeficiency T-cell lymphopenia identified through newborn screening.

Patrawala M, Kobrynski L
Curr Opin Allergy Clin Immunol 2019 Dec;19(6):586-593. doi: 10.1097/ACI.0000000000000586. PMID: 31490207

Targeted screening for primary immunodeficiency disorders in the neonatal period and early infancy.

Galal N, Ohida M, Meshaal S, Elaziz DA, Elhawary I
Afr Health Sci 2019 Mar;19(1):1449-1459. doi: 10.4314/ahs.v19i1.18. PMID: 31148972 Free PMC Article

Treatment and prevention of respiratory syncytial virus infection.

Kneyber MC, Moll HA, de Groot R
Eur J Pediatr 2000 Jun;159(6):399-411. doi: 10.1007/s004310051296. PMID: 10867843

See all (9)

Recent clinical studies

Etiology

The Role of Telomeres in Human Disease.

Armanios M
Annu Rev Genomics Hum Genet 2022 Aug 31;23:363-381. Epub 2022 Jun 24 doi: 10.1146/annurev-genom-010422-091101. PMID: 35609925 Free PMC Article

Spectrum of Genetic T-Cell Disorders from 22q11.2DS to CHARGE.

Urschel D, Hernandez-Trujillo VP
Clin Rev Allergy Immunol 2022 Aug;63(1):99-105. Epub 2022 Feb 8 doi: 10.1007/s12016-022-08927-z. PMID: 35133619

Inborn errors of thymic stromal cell development and function.

Kreins AY, Maio S, Dhalla F
Semin Immunopathol 2021 Feb;43(1):85-100. Epub 2020 Nov 30 doi: 10.1007/s00281-020-00826-9. PMID: 33257998 Free PMC Article

Autoimmunity in primary T-cell immunodeficiencies.

Azizi G, Ghanavatinejad A, Abolhassani H, Yazdani R, Rezaei N, Mirshafiey A, Aghamohammadi A
Expert Rev Clin Immunol 2016 Sep;12(9):989-1006. Epub 2016 Apr 28 doi: 10.1080/1744666X.2016.1177458. PMID: 27063703

T cell immunodeficiency.

Edgar JD
J Clin Pathol 2008 Sep;61(9):988-93. doi: 10.1136/jcp.2007.051144. PMID: 18755723

See all (86)

Diagnosis

Telomere-mediated lung disease.

Alder JK, Armanios M
Physiol Rev 2022 Oct 1;102(4):1703-1720. Epub 2022 May 9 doi: 10.1152/physrev.00046.2021. PMID: 35532056 Free PMC Article

Spectrum of Genetic T-Cell Disorders from 22q11.2DS to CHARGE.

Urschel D, Hernandez-Trujillo VP
Clin Rev Allergy Immunol 2022 Aug;63(1):99-105. Epub 2022 Feb 8 doi: 10.1007/s12016-022-08927-z. PMID: 35133619

Impaired T cell receptor signaling and development of T cell-mediated autoimmune arthritis.

Takeuchi Y, Hirota K, Sakaguchi S
Immunol Rev 2020 Mar;294(1):164-176. Epub 2020 Jan 16 doi: 10.1111/imr.12841. PMID: 31944330

A semiautomated whole-exome sequencing workflow leads to increased diagnostic yield and identification of novel candidate variants.

Ji J, Shen L, Bootwalla M, Quindipan C, Tatarinova T, Maglinte DT, Buckley J, Raca G, Saitta SC, Biegel JA, Gai X
Cold Spring Harb Mol Case Stud 2019 Apr;5(2) Epub 2019 Apr 1 doi: 10.1101/mcs.a003756. PMID: 30755392 Free PMC Article

T cell immunodeficiency.

Edgar JD
J Clin Pathol 2008 Sep;61(9):988-93. doi: 10.1136/jcp.2007.051144. PMID: 18755723

See all (79)

Therapy

Donor-Derived CD7 Chimeric Antigen Receptor T Cells for T-Cell Acute Lymphoblastic Leukemia: First-in-Human, Phase I Trial.

Pan J, Tan Y, Wang G, Deng B, Ling Z, Song W, Seery S, Zhang Y, Peng S, Xu J, Duan J, Wang Z, Yu X, Zheng Q, Xu X, Yuan Y, Yan F, Tian Z, Tang K, Zhang J, Chang AH, Feng X
J Clin Oncol 2021 Oct 20;39(30):3340-3351. Epub 2021 Jul 29 doi: 10.1200/JCO.21.00389. PMID: 34324392

Gene therapy for severe combined immunodeficiencies and beyond.

Fischer A, Hacein-Bey-Abina S
J Exp Med 2020 Jan 6;217(2) doi: 10.1084/jem.20190607. PMID: 31826240 Free PMC Article

Concise Review: Boosting T-Cell Reconstitution Following Allogeneic Transplantation-Current Concepts and Future Perspectives.

Simons L, Cavazzana M, André I
Stem Cells Transl Med 2019 Jul;8(7):650-657. Epub 2019 Mar 18 doi: 10.1002/sctm.18-0248. PMID: 30887712 Free PMC Article

The T Cell in Myeloma.

Joshua D, Suen H, Brown R, Bryant C, Ho PJ, Hart D, Gibson J
Clin Lymphoma Myeloma Leuk 2016 Oct;16(10):537-542. Epub 2016 Aug 10 doi: 10.1016/j.clml.2016.08.003. PMID: 27601001

T cell immunodeficiency.

Edgar JD
J Clin Pathol 2008 Sep;61(9):988-93. doi: 10.1136/jcp.2007.051144. PMID: 18755723

See all (63)

Prognosis

Immunologic Heterogeneity in 2 Cartilage-Hair Hypoplasia Patients With a Distinct Clinical Course.

Gamliel A, Lee YN, Lev A, AbuZaitun O, Rechavi E, Levy S, Simon AJ, Somech R
J Investig Allergol Clin Immunol 2023 Jul 27;33(4):263-270. Epub 2022 Feb 15 doi: 10.18176/jiaci.0792. PMID: 35166674

Telomere-mediated lung disease.

Alder JK, Armanios M
Physiol Rev 2022 Oct 1;102(4):1703-1720. Epub 2022 May 9 doi: 10.1152/physrev.00046.2021. PMID: 35532056 Free PMC Article

Donor-Derived CD7 Chimeric Antigen Receptor T Cells for T-Cell Acute Lymphoblastic Leukemia: First-in-Human, Phase I Trial.

Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host.

Worth AJ, Houldcroft CJ, Booth C
Br J Haematol 2016 Nov;175(4):559-576. Epub 2016 Oct 17 doi: 10.1111/bjh.14339. PMID: 27748521

Fusarium infections in immunocompromised patients.

Nucci M, Anaissie E
Clin Microbiol Rev 2007 Oct;20(4):695-704. doi: 10.1128/CMR.00014-07. PMID: 17934079 Free PMC Article

See all (47)

Clinical prediction guides

Thymic Engraftment by in vitro-Derived Progenitor T Cells in Young and Aged Mice.

Singh J, Mohtashami M, Anderson G, Zúñiga-Pflücker JC
Front Immunol 2020;11:1850. Epub 2020 Aug 18 doi: 10.3389/fimmu.2020.01850. PMID: 32973763 Free PMC Article

Gene therapy for severe combined immunodeficiencies and beyond.

Fischer A, Hacein-Bey-Abina S
J Exp Med 2020 Jan 6;217(2) doi: 10.1084/jem.20190607. PMID: 31826240 Free PMC Article

Defining combined immunodeficiency.

Roifman CM, Somech R, Kavadas F, Pires L, Nahum A, Dalal I, Grunebaum E
J Allergy Clin Immunol 2012 Jul;130(1):177-83. Epub 2012 Jun 2 doi: 10.1016/j.jaci.2012.04.029. PMID: 22664165

T-cell immunodeficiency following cytotoxic antineoplastic therapy: a review.

Mackall CL
Stem Cells 2000;18(1):10-8. doi: 10.1634/stemcells.18-1-10. PMID: 10661568

T-cell immunodeficiency following cytotoxic antineoplastic therapy: a review.

Mackall CL
Oncologist 1999;4(5):370-8. PMID: 10551553

See all (46)

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T-cell immunodeficiency

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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