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T-Cell Large Granular Lymphocyte Leukemia Expressing the T-Cell Receptor Gamma-Delta

MedGen UID:
272804
•Concept ID:
C1515129
•
Neoplastic Process
Synonym: T-Cell Large Granular Lymphocyte Leukemia Expressing the TCR Gamma-Delta

Definition

An uncommon variant of T-cell large granular lymphocyte leukemia expressing the gamma-delta T-cell receptor. Approximately 60% of these cases are CD8-positive and the remainder are CD4/CD8-negative. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVT-Cell Large Granular Lymphocyte Leukemia Expressing the T-Cell Receptor Gamma-Delta

Recent clinical studies

Etiology

Genomic landscape of TCRαβ and TCRγδ T-large granular lymphocyte leukemia.
Cheon H, Xing JC, Moosic KB, Ung J, Chan VW, Chung DS, Toro MF, Elghawy O, Wang JS, Hamele CE, Hardison RC, Olson TL, Tan SF, Feith DJ, Ratan A, Loughran TP
Blood 2022 May 19;139(20):3058-3072. doi: 10.1182/blood.2021013164. PMID: 35015834Free PMC Article
Dysregulated signaling, proliferation and apoptosis impact on the pathogenesis of TCRγδ+ T cell large granular lymphocyte leukemia.
Kallemeijn MJ, de Ridder D, Schilperoord-Vermeulen J, van der Klift MY, Sandberg Y, van Dongen JJ, Langerak AW
PLoS One 2017;12(4):e0175670. Epub 2017 Apr 13 doi: 10.1371/journal.pone.0175670. PMID: 28407008Free PMC Article
Clinicopathologic, Immunophenotypic, Cytogenetic, and Molecular Features of γδ T-Cell Large Granular Lymphocytic Leukemia: An Analysis of 14 Patients Suggests Biologic Differences With αβ T-Cell Large Granular Lymphocytic Leukemia. [corrected].
Yabe M, Medeiros LJ, Wang SA, Konoplev S, Ok CY, Loghavi S, Lu G, Flores L, Khoury JD, Cason RC, Young KH, Miranda RN
Am J Clin Pathol 2015 Oct;144(4):607-19. doi: 10.1309/AJCPJSA1E1YWSZEY. PMID: 26386082
Natural killer cell lymphoma shares strikingly similar molecular features with a group of non-hepatosplenic γδ T-cell lymphoma and is highly sensitive to a novel aurora kinase A inhibitor in vitro.
Iqbal J, Weisenburger DD, Chowdhury A, Tsai MY, Srivastava G, Greiner TC, Kucuk C, Deffenbacher K, Vose J, Smith L, Au WY, Nakamura S, Seto M, Delabie J, Berger F, Loong F, Ko YH, Sng I, Liu X, Loughran TP, Armitage J, Chan WC; International Peripheral T-cell Lymphoma Project
Leukemia 2011 Feb;25(2):348-58. Epub 2010 Nov 5 doi: 10.1038/leu.2010.255. PMID: 21052088
Immunophenotypic attributes of benign peripheral blood gammadelta T cells and conditions associated with their increase.
Roden AC, Morice WG, Hanson CA
Arch Pathol Lab Med 2008 Nov;132(11):1774-80. doi: 10.5858/132.11.1774. PMID: 18976014

Diagnosis

Defining TCRγδ lymphoproliferative disorders by combined immunophenotypic and molecular evaluation.
Teramo A, Binatti A, Ciabatti E, Schiavoni G, Tarrini G, Barilà G, Calabretto G, Vicenzetto C, Gasparini VR, Facco M, Petrini I, Grossi R, Pisanti N, Bortoluzzi S, Falini B, Tiacci E, Galimberti S, Semenzato G, Zambello R
Nat Commun 2022 Jun 8;13(1):3298. doi: 10.1038/s41467-022-31015-x. PMID: 35676278Free PMC Article
Clinicopathologic, Immunophenotypic, Cytogenetic, and Molecular Features of γδ T-Cell Large Granular Lymphocytic Leukemia: An Analysis of 14 Patients Suggests Biologic Differences With αβ T-Cell Large Granular Lymphocytic Leukemia. [corrected].
Yabe M, Medeiros LJ, Wang SA, Konoplev S, Ok CY, Loghavi S, Lu G, Flores L, Khoury JD, Cason RC, Young KH, Miranda RN
Am J Clin Pathol 2015 Oct;144(4):607-19. doi: 10.1309/AJCPJSA1E1YWSZEY. PMID: 26386082
Unusual immunophenotype of T-cell large granular lymphocytic leukemia: report of two cases.
Rabade N, Mansukhani D, Khodaiji S, Padte B, Bhave A, Tembhare P, Subramanian PG, Sehgal K
Indian J Pathol Microbiol 2015 Jan-Mar;58(1):108-12. doi: 10.4103/0377-4929.151204. PMID: 25673609
Transformed aggressive γδ-variant T-cell large granular lymphocytic leukemia with acquired copy neutral loss of heterozygosity at 17q11.2q25.3 and additional aberrations.
Zhang L, Ramchandren R, Papenhausen P, Loughran TP, Sokol L
Eur J Haematol 2014 Sep;93(3):260-4. Epub 2014 Apr 7 doi: 10.1111/ejh.12313. PMID: 24635703
Natural killer cell lymphoma shares strikingly similar molecular features with a group of non-hepatosplenic γδ T-cell lymphoma and is highly sensitive to a novel aurora kinase A inhibitor in vitro.
Iqbal J, Weisenburger DD, Chowdhury A, Tsai MY, Srivastava G, Greiner TC, Kucuk C, Deffenbacher K, Vose J, Smith L, Au WY, Nakamura S, Seto M, Delabie J, Berger F, Loong F, Ko YH, Sng I, Liu X, Loughran TP, Armitage J, Chan WC; International Peripheral T-cell Lymphoma Project
Leukemia 2011 Feb;25(2):348-58. Epub 2010 Nov 5 doi: 10.1038/leu.2010.255. PMID: 21052088

Prognosis

Defining TCRγδ lymphoproliferative disorders by combined immunophenotypic and molecular evaluation.
Teramo A, Binatti A, Ciabatti E, Schiavoni G, Tarrini G, Barilà G, Calabretto G, Vicenzetto C, Gasparini VR, Facco M, Petrini I, Grossi R, Pisanti N, Bortoluzzi S, Falini B, Tiacci E, Galimberti S, Semenzato G, Zambello R
Nat Commun 2022 Jun 8;13(1):3298. doi: 10.1038/s41467-022-31015-x. PMID: 35676278Free PMC Article
Clinicopathologic, Immunophenotypic, Cytogenetic, and Molecular Features of γδ T-Cell Large Granular Lymphocytic Leukemia: An Analysis of 14 Patients Suggests Biologic Differences With αβ T-Cell Large Granular Lymphocytic Leukemia. [corrected].
Yabe M, Medeiros LJ, Wang SA, Konoplev S, Ok CY, Loghavi S, Lu G, Flores L, Khoury JD, Cason RC, Young KH, Miranda RN
Am J Clin Pathol 2015 Oct;144(4):607-19. doi: 10.1309/AJCPJSA1E1YWSZEY. PMID: 26386082
Unusual immunophenotype of T-cell large granular lymphocytic leukemia: report of two cases.
Rabade N, Mansukhani D, Khodaiji S, Padte B, Bhave A, Tembhare P, Subramanian PG, Sehgal K
Indian J Pathol Microbiol 2015 Jan-Mar;58(1):108-12. doi: 10.4103/0377-4929.151204. PMID: 25673609

Clinical prediction guides

Defining TCRγδ lymphoproliferative disorders by combined immunophenotypic and molecular evaluation.
Teramo A, Binatti A, Ciabatti E, Schiavoni G, Tarrini G, Barilà G, Calabretto G, Vicenzetto C, Gasparini VR, Facco M, Petrini I, Grossi R, Pisanti N, Bortoluzzi S, Falini B, Tiacci E, Galimberti S, Semenzato G, Zambello R
Nat Commun 2022 Jun 8;13(1):3298. doi: 10.1038/s41467-022-31015-x. PMID: 35676278Free PMC Article
Genomic landscape of TCRαβ and TCRγδ T-large granular lymphocyte leukemia.
Cheon H, Xing JC, Moosic KB, Ung J, Chan VW, Chung DS, Toro MF, Elghawy O, Wang JS, Hamele CE, Hardison RC, Olson TL, Tan SF, Feith DJ, Ratan A, Loughran TP
Blood 2022 May 19;139(20):3058-3072. doi: 10.1182/blood.2021013164. PMID: 35015834Free PMC Article
Dysregulated signaling, proliferation and apoptosis impact on the pathogenesis of TCRγδ+ T cell large granular lymphocyte leukemia.
Kallemeijn MJ, de Ridder D, Schilperoord-Vermeulen J, van der Klift MY, Sandberg Y, van Dongen JJ, Langerak AW
PLoS One 2017;12(4):e0175670. Epub 2017 Apr 13 doi: 10.1371/journal.pone.0175670. PMID: 28407008Free PMC Article

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