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Clonal T cell receptor rearrangement

MedGen UID:
1717265
•Concept ID:
C5397604
•
Finding
HPO: HP:0032577

Definition

Presence of a predominant T cell clone. In PCR-based assays, this finding is inferred on the basis of one or two prominent bands within a valid size range. In NGS-based assays, this finding is inferred on the basis of a high number of reads that map to a single T cell receptor clone. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVClonal T cell receptor rearrangement

Professional guidelines

PubMed

Genetic alterations and MRD refine risk assessment for KMT2A-rearranged B-cell precursor ALL in adults: a GRAALL study.
Kim R, Bergugnat H, Pastoret C, Pasquier F, Raffoux E, Larcher L, Passet M, Grardel N, Delabesse E, Kubetzko S, Caye-Eude A, Meyer C, Marschalek R, Lafage-Pochitaloff M, Thiebaut-Bertrand A, Balsat M, Escoffre-Barbe M, Blum S, Baumann M, Banos A, Straetmans N, Gallego-Hernanz MP, Chalandon Y, Graux C, Soulier J, Leguay T, Hunault M, Huguet F, Lhéritier V, Dombret H, Boissel N, Clappier E
Blood 2023 Nov 23;142(21):1806-1817. doi: 10.1182/blood.2023021501. PMID: 37595275
AGA Clinical Practice Update on Management of Refractory Celiac Disease: Expert Review.
Green PHR, Paski S, Ko CW, Rubio-Tapia A
Gastroenterology 2022 Nov;163(5):1461-1469. Epub 2022 Sep 19 doi: 10.1053/j.gastro.2022.07.086. PMID: 36137844
Advances in diagnosis and treatment of large granular lymphocyte syndrome.
Prochorec-Sobieszek M
Curr Opin Hematol 2011 Jan;18(1):55-62. doi: 10.1097/MOH.0b013e328340dc12. PMID: 20975557

Recent clinical studies

Etiology

Clonal T-cell large granular lymphocyte proliferations in childhood and young adult immune dysregulation conditions.
Savaşan S, Al-Qanber B, Buck S, Wakeling E, Gadgeel M
Pediatr Blood Cancer 2020 May;67(5):e28231. Epub 2020 Mar 2 doi: 10.1002/pbc.28231. PMID: 32124536
CD56 expression in T-cell acute lymphoblastic leukemia is associated with non-thymic phenotype and resistance to induction therapy but no inferior survival after risk-adapted therapy.
Fischer L, Gökbuget N, Schwartz S, Burmeister T, Rieder H, Brüggemann M, Hoelzer D, Thiel E
Haematologica 2009 Feb;94(2):224-9. Epub 2008 Dec 23 doi: 10.3324/haematol.13543. PMID: 19109214Free PMC Article

Diagnosis

Subcutaneous panniculitis-like T-cell lymphoma: Morphological, immunophenotypical and clonality assessment in six cats.
Roccabianca P, Dell'Aere S, Avallone G, Zamboni C, Bertazzolo W, Crippa L, Giudice C, Caniatti M, Affolter VK
Vet Dermatol 2024 Apr;35(2):207-218. Epub 2023 Oct 31 doi: 10.1111/vde.13211. PMID: 37904626
Intralymphatic Proliferation of T-cell Lymphoid Blasts in the Setting of Hidradenitis Suppurativa.
Calamaro P, Cerroni L
Am J Dermatopathol 2016 Jul;38(7):536-40. doi: 10.1097/DAD.0000000000000466. PMID: 26844617
CD56 expression in T-cell acute lymphoblastic leukemia is associated with non-thymic phenotype and resistance to induction therapy but no inferior survival after risk-adapted therapy.
Fischer L, Gökbuget N, Schwartz S, Burmeister T, Rieder H, Brüggemann M, Hoelzer D, Thiel E
Haematologica 2009 Feb;94(2):224-9. Epub 2008 Dec 23 doi: 10.3324/haematol.13543. PMID: 19109214Free PMC Article
Myositis in a patient with large granular leukocyte leukemia.
Rosche B, Jacobsen M, Cepok S, Barth P, Sommer N, Hemmer B
Muscle Nerve 2004 Jun;29(6):873-7. doi: 10.1002/mus.10570. PMID: 15170622

Therapy

CD56 expression in T-cell acute lymphoblastic leukemia is associated with non-thymic phenotype and resistance to induction therapy but no inferior survival after risk-adapted therapy.
Fischer L, Gökbuget N, Schwartz S, Burmeister T, Rieder H, Brüggemann M, Hoelzer D, Thiel E
Haematologica 2009 Feb;94(2):224-9. Epub 2008 Dec 23 doi: 10.3324/haematol.13543. PMID: 19109214Free PMC Article
Clonal T cell-mediated cyclic thrombocytopenia.
Füreder W, Mitterbauer G, Thalhammer R, Geissler K, Panzer S, Krebs M, Simonitsch-Klupp I, Sperr WR, Lechner K, Kyrle PA
Br J Haematol 2002 Dec;119(4):1059-61. doi: 10.1046/j.1365-2141.2002.03951.x. PMID: 12472588

Prognosis

Clonal T-cell large granular lymphocyte proliferations in childhood and young adult immune dysregulation conditions.
Savaşan S, Al-Qanber B, Buck S, Wakeling E, Gadgeel M
Pediatr Blood Cancer 2020 May;67(5):e28231. Epub 2020 Mar 2 doi: 10.1002/pbc.28231. PMID: 32124536
Intralymphatic Proliferation of T-cell Lymphoid Blasts in the Setting of Hidradenitis Suppurativa.
Calamaro P, Cerroni L
Am J Dermatopathol 2016 Jul;38(7):536-40. doi: 10.1097/DAD.0000000000000466. PMID: 26844617
CD56 expression in T-cell acute lymphoblastic leukemia is associated with non-thymic phenotype and resistance to induction therapy but no inferior survival after risk-adapted therapy.
Fischer L, Gökbuget N, Schwartz S, Burmeister T, Rieder H, Brüggemann M, Hoelzer D, Thiel E
Haematologica 2009 Feb;94(2):224-9. Epub 2008 Dec 23 doi: 10.3324/haematol.13543. PMID: 19109214Free PMC Article

Clinical prediction guides

Clonal T-cell large granular lymphocyte proliferations in childhood and young adult immune dysregulation conditions.
Savaşan S, Al-Qanber B, Buck S, Wakeling E, Gadgeel M
Pediatr Blood Cancer 2020 May;67(5):e28231. Epub 2020 Mar 2 doi: 10.1002/pbc.28231. PMID: 32124536
Intralymphatic Proliferation of T-cell Lymphoid Blasts in the Setting of Hidradenitis Suppurativa.
Calamaro P, Cerroni L
Am J Dermatopathol 2016 Jul;38(7):536-40. doi: 10.1097/DAD.0000000000000466. PMID: 26844617
CD56 expression in T-cell acute lymphoblastic leukemia is associated with non-thymic phenotype and resistance to induction therapy but no inferior survival after risk-adapted therapy.
Fischer L, Gökbuget N, Schwartz S, Burmeister T, Rieder H, Brüggemann M, Hoelzer D, Thiel E
Haematologica 2009 Feb;94(2):224-9. Epub 2008 Dec 23 doi: 10.3324/haematol.13543. PMID: 19109214Free PMC Article

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