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Large granular lymphocyte leukemia

MedGen UID:
279467
•Concept ID:
C1522378
•
Neoplastic Process
Synonyms: Aggressive Natural Killer Cell Leukemia; Aggressive NK Cell Leukemia; Large Granular Lymphocyte Leukemia; Leukemia, Large Granular Lymphocytic; Leukemia, LGL; Leukemia, Lymphocytic, Large Granular; Leukemias, LGL; LGL Leukemia; LGL Leukemias; Lymphoproliferative Disease of Granular Lymphocytes; Lymphoproliferative Disease of Large Granular Lymphocytes
SNOMED CT: Large granular lymphocytosis (128819001); Large granular lymphocytic leukemia (277569004); LGLL - Large granular lymphocytic leukemia (277569004)
 
Orphanet: ORPHA512034

Definition

A spectrum of disorders characterized by clonal expansions of the peripheral blood LYMPHOCYTE populations known as large granular lymphocytes which contain abundant cytoplasm and azurophilic granules. Subtypes develop from either CD3-negative NATURAL KILLER CELLS or CD3-positive T-CELLS. The clinical course of both subtypes can vary from spontaneous regression to progressive, malignant disease. [from MeSH]

Professional guidelines

PubMed

Advances in the Diagnosis and Treatment of Large Granular Lymphocytic Leukemia.
Cheon H, Dziewulska KH, Moosic KB, Olson KC, Gru AA, Feith DJ, Loughran TP Jr
Curr Hematol Malig Rep 2020 Apr;15(2):103-112. doi: 10.1007/s11899-020-00565-6. PMID: 32062772Free PMC Article
The pathogenesis and treatment of large granular lymphocyte leukemia.
Steinway SN, LeBlanc F, Loughran TP Jr
Blood Rev 2014 May;28(3):87-94. Epub 2014 Mar 7 doi: 10.1016/j.blre.2014.02.001. PMID: 24679833Free PMC Article
Purine nucleoside analogs in the treatment of rarer chronic lymphoid leukemias.
Robak T, Robak P
Curr Pharm Des 2012;18(23):3373-88. doi: 10.2174/138161212801227005. PMID: 22591387

Recent clinical studies

Etiology

Pro-inflammatory cells sustain leukemic clonal expansion in T-cell large granular lymphocyte leukemia.
Vicenzetto C, Gasparini VR, Barila G, Teramo A, Calabretto G, Rampazzo E, Carraro S, Trimarco V, Trentin L, Facco M, Semenzato G, Zambello R
Haematologica 2024 Jan 1;109(1):163-174. doi: 10.3324/haematol.2022.282306. PMID: 37439335Free 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
Advances in the Diagnosis and Treatment of Large Granular Lymphocytic Leukemia.
Cheon H, Dziewulska KH, Moosic KB, Olson KC, Gru AA, Feith DJ, Loughran TP Jr
Curr Hematol Malig Rep 2020 Apr;15(2):103-112. doi: 10.1007/s11899-020-00565-6. PMID: 32062772Free PMC Article
A population-based study of large granular lymphocyte leukemia.
Shah MV, Hook CC, Call TG, Go RS
Blood Cancer J 2016 Aug 5;6(8):e455. doi: 10.1038/bcj.2016.59. PMID: 27494824Free PMC Article
Autoimmune manifestations in large granular lymphocyte leukemia.
Bockorny B, Dasanu CA
Clin Lymphoma Myeloma Leuk 2012 Dec;12(6):400-5. Epub 2012 Sep 19 doi: 10.1016/j.clml.2012.06.006. PMID: 22999943

Diagnosis

Large granular lymphocyte leukemia: An indolent clonal proliferative disease associated with an array of various immunologic disorders.
Drillet G, Pastoret C, Moignet A, Lamy T, Marchand T
Rev Med Interne 2023 Jun;44(6):295-306. Epub 2023 Apr 21 doi: 10.1016/j.revmed.2023.03.014. PMID: 37087371
Neutropenia and Large Granular Lymphocyte Leukemia: From Pathogenesis to Therapeutic Options.
Calabretto G, Teramo A, Barilà G, Vicenzetto C, Gasparini VR, Semenzato G, Zambello R
Cells 2021 Oct 19;10(10) doi: 10.3390/cells10102800. PMID: 34685780Free PMC Article
Emerging Role of T-cell Receptor Constant β Chain-1 (TRBC1) Expression in the Flow Cytometric Diagnosis of T-cell Malignancies.
Horna P, Shi M, Olteanu H, Johansson U
Int J Mol Sci 2021 Feb 12;22(4) doi: 10.3390/ijms22041817. PMID: 33673033Free PMC Article
Advances in the Diagnosis and Treatment of Large Granular Lymphocytic Leukemia.
Cheon H, Dziewulska KH, Moosic KB, Olson KC, Gru AA, Feith DJ, Loughran TP Jr
Curr Hematol Malig Rep 2020 Apr;15(2):103-112. doi: 10.1007/s11899-020-00565-6. PMID: 32062772Free PMC Article
Large granular lymphocyte leukemia.
Sokol L, Loughran TP Jr
Oncologist 2006 Mar;11(3):263-73. doi: 10.1634/theoncologist.11-3-263. PMID: 16549811

Therapy

Single-cell RNA sequencing coupled to TCR profiling of large granular lymphocyte leukemia T cells.
Gao S, Wu Z, Arnold B, Diamond C, Batchu S, Giudice V, Alemu L, Raffo DQ, Feng X, Kajigaya S, Barrett J, Ito S, Young NS
Nat Commun 2022 Apr 11;13(1):1982. doi: 10.1038/s41467-022-29175-x. PMID: 35411048Free PMC Article
Advances in the Diagnosis and Treatment of Large Granular Lymphocytic Leukemia.
Cheon H, Dziewulska KH, Moosic KB, Olson KC, Gru AA, Feith DJ, Loughran TP Jr
Curr Hematol Malig Rep 2020 Apr;15(2):103-112. doi: 10.1007/s11899-020-00565-6. PMID: 32062772Free PMC Article
Pure red cell aplasia.
Means RT Jr
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):51-56. doi: 10.1182/asheducation-2016.1.51. PMID: 27913462Free PMC Article
Autoimmune neutropenia.
Autrel-Moignet A, Lamy T
Presse Med 2014 Apr;43(4 Pt 2):e105-18. Epub 2014 Mar 27 doi: 10.1016/j.lpm.2014.02.007. PMID: 24680423
Large granular lymphocyte leukemia.
Sokol L, Loughran TP Jr
Curr Hematol Malig Rep 2007 Oct;2(4):278-82. doi: 10.1007/s11899-007-0038-7. PMID: 20425381

Prognosis

The complex relationship between large granular lymphocyte leukemia and rheumatic disease.
Marchand T, Lamy T
Expert Rev Clin Immunol 2024 Mar;20(3):291-303. Epub 2023 Dec 18 doi: 10.1080/1744666X.2023.2292758. PMID: 38105745
Single-cell RNA sequencing coupled to TCR profiling of large granular lymphocyte leukemia T cells.
Gao S, Wu Z, Arnold B, Diamond C, Batchu S, Giudice V, Alemu L, Raffo DQ, Feng X, Kajigaya S, Barrett J, Ito S, Young NS
Nat Commun 2022 Apr 11;13(1):1982. doi: 10.1038/s41467-022-29175-x. PMID: 35411048Free PMC Article
Neutropenia and Large Granular Lymphocyte Leukemia: From Pathogenesis to Therapeutic Options.
Calabretto G, Teramo A, Barilà G, Vicenzetto C, Gasparini VR, Semenzato G, Zambello R
Cells 2021 Oct 19;10(10) doi: 10.3390/cells10102800. PMID: 34685780Free PMC Article
The pathogenesis and treatment of large granular lymphocyte leukemia.
Steinway SN, LeBlanc F, Loughran TP Jr
Blood Rev 2014 May;28(3):87-94. Epub 2014 Mar 7 doi: 10.1016/j.blre.2014.02.001. PMID: 24679833Free PMC Article
Large granular lymphocyte leukemia.
Sokol L, Loughran TP Jr
Oncologist 2006 Mar;11(3):263-73. doi: 10.1634/theoncologist.11-3-263. PMID: 16549811

Clinical prediction guides

Single-cell RNA sequencing coupled to TCR profiling of large granular lymphocyte leukemia T cells.
Gao S, Wu Z, Arnold B, Diamond C, Batchu S, Giudice V, Alemu L, Raffo DQ, Feng X, Kajigaya S, Barrett J, Ito S, Young NS
Nat Commun 2022 Apr 11;13(1):1982. doi: 10.1038/s41467-022-29175-x. PMID: 35411048Free 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
Advances in the Diagnosis and Treatment of Large Granular Lymphocytic Leukemia.
Cheon H, Dziewulska KH, Moosic KB, Olson KC, Gru AA, Feith DJ, Loughran TP Jr
Curr Hematol Malig Rep 2020 Apr;15(2):103-112. doi: 10.1007/s11899-020-00565-6. PMID: 32062772Free PMC Article
The spectrum of large granular lymphocyte leukemia and Felty's syndrome.
Liu X, Loughran TP Jr
Curr Opin Hematol 2011 Jul;18(4):254-9. doi: 10.1097/MOH.0b013e32834760fb. PMID: 21546829Free PMC Article
Current concepts: large granular lymphocyte leukemia.
Lamy T, Loughran TP Jr
Blood Rev 1999 Dec;13(4):230-40. doi: 10.1054/blre.1999.0118. PMID: 10741898

Recent systematic reviews

Aggressive T-cell large granular lymphocyte leukemia: a case report and review of the literature.
Alekshun TJ, Tao J, Sokol L
Am J Hematol 2007 Jun;82(6):481-5. doi: 10.1002/ajh.20853. PMID: 17205534

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