T-LGL
MCID: TCL002
MIFTS: 48

T-Cell Large Granular Lymphocyte Leukemia (T-LGL) malady

Blood diseases, Cancer diseases, Rare diseases, Immune diseases categories
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Summaries for T-Cell Large Granular Lymphocyte Leukemia

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8Disease Ontology, 65Wikipedia, 33MalaCards
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Disease Ontology:8 A chronic lymphocytic leukemia that exhibits an unexplained, chronic (> 6 months) elevation in large granular lymphocytes (lgls) in the peripheral blood.

MalaCards: T-Cell Large Granular Lymphocyte Leukemia, also known as proliferation of large granular lymphocytes, is related to leukemia and myeloma. An important gene associated with T-Cell Large Granular Lymphocyte Leukemia is KIR2DL1 (killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 1), and among its related pathways are IL12 signaling mediated by STAT4 and IL2-mediated signaling events. The compounds tuberculin and cuprophan have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone marrow and bone, and related mouse phenotypes are hematopoietic system and immune system.

Wikipedia:65 Large granular lymphocytic leukemia is a disease that exhibits an unexplained, chronic (> 6 months)... more...

Aliases & Classifications for T-Cell Large Granular Lymphocyte Leukemia

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8Disease Ontology, 49Orphanet, 62UMLS, 45Novoseek, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases, Rare diseases
Anatomical: Blood diseases, Immune diseases


Characteristics (Orphanet epidemiological data):

49

Aliases & Descriptions:

t-cell large granular lymphocyte leukemia 8 49 62
proliferation of large granular lymphocytes 49
leukemia, large granular lymphocytic 62
large granular lymphocytic leukemia 8
leukemia lymphocytic large granular 45
lown-ganong-levine syndrome 62
t-cell lgl leukemia 49
t-lgl 49


External Ids:

Disease Ontology8 DOID:0050751
MESH via Orphanet36 D054066
ICD10 via Orphanet26 C91.5
SNOMED-CT via Orphanet59 128819001, 55081009, 277545003
UMLS via Orphanet63 C1955861

Related Diseases for T-Cell Large Granular Lymphocyte Leukemia

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17GeneCards, 18GeneDecks
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Diseases related to T-Cell Large Granular Lymphocyte Leukemia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 73)
idRelated DiseaseScoreTop Affiliating Genes
1leukemia31.8LBR, STAT3, IL2RA, KLRK1
2myeloma30.9KLRK1, STAT3
3rheumatoid arthritis30.7LBR, IL2RA, KIR2DL1, KLRK1, KLRD1
4t-cell leukemia30.6IL2RA, STAT3, LBR
5systemic lupus erythematosus30.5LBR, IL2RA, KLRK1, KLRD1
6celiac disease30.4KLRK1, MICA
7multiple sclerosis30.4LBR, IL2RA, KLRK1
8large granular lymphocyte leukemia11.3
9natural killer cell leukemia10.6
10arthritis10.6
11neutropenia10.6
12splenomegaly10.6
13hepatosplenic t-cell lymphoma10.6
14hemolytic anemia10.5
15autoimmune hemolytic anemia10.4
16hepatitis10.4
17purpura10.4
18chronic active epstein-barr virus infection10.4
19graft versus host disease10.4
20crohn's disease10.4
21pure red-cell aplasia10.4
22gamma heavy chain disease10.4
23protein-losing enteropathy10.4
24sjogren's syndrome10.4
25hairy cell leukemia10.4
26heavy chain disease10.4
27lupus erythematosus10.4
28neuropathy10.4
29prolymphocytic leukemia10.4
30chronic graft versus host disease10.4
31chronic myelomonocytic leukemia10.4
32essential thrombocythemia10.4
33human t-cell leukemia virus type 110.4
34myelodysplastic syndromes10.4
35t-cell prolymphocytic leukemia10.4
36hepatitis c10.3
37autoimmune hepatitis10.3
38ulcerative colitis10.3
39malignant histiocytosis10.3
40lambert-eaton myasthenic syndrome10.3
41common variable immunodeficiency10.3
42felty's syndrome10.3
43granuloma annulare10.3
44aplastic anemia10.3
45candidiasis10.3
46chronic lymphocytic leukemia10.3
47encephalitis10.3
48hepatocellular carcinoma10.3
49histiocytosis10.3
50myelofibrosis10.3

Graphical network of the top 20 diseases related to T-Cell Large Granular Lymphocyte Leukemia:



Diseases related to t-cell large granular lymphocyte leukemia

Symptoms for T-Cell Large Granular Lymphocyte Leukemia

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Drugs & Therapeutics for T-Cell Large Granular Lymphocyte Leukemia

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for T-Cell Large Granular Lymphocyte Leukemia

Search NIH Clinical Center for T-Cell Large Granular Lymphocyte Leukemia

Genetic Tests for T-Cell Large Granular Lymphocyte Leukemia

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Anatomical Context for T-Cell Large Granular Lymphocyte Leukemia

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33MalaCards
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MalaCards organs/tissues related to T-Cell Large Granular Lymphocyte Leukemia:

33
T cells, Bone marrow, Bone, Nk cells, Spleen, B cells

Animal Models for T-Cell Large Granular Lymphocyte Leukemia or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to T-Cell Large Granular Lymphocyte Leukemia:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053978.3KLRK1, IL15RA, IL2RA, STAT3, LBR
2MP:00053877.1KLRD1, LBR, STAT3, IL2RA, IL15RA, KLRK1

Publications for T-Cell Large Granular Lymphocyte Leukemia

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52PubMed
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Articles related to T-Cell Large Granular Lymphocyte Leukemia:

(show top 50)    (show all 55)
idTitleAuthorsYear
1
Lack of common TCRA and TCRB clonotypes in CD8(+)/TCRI+I^(+) T-cell large granular lymphocyte leukemia: a review on the role of antigenic selection in the immunopathogenesis of CD8(+) T-LGL. (24413066)
2014
2
Treatment of T-cell large granular lymphocyte leukemia with cyclosporine A: experience in a Chinese single institution. (23395383)
2013
3
T-cell large granular lymphocyte leukemia in a child with anemia and Crohn's disease. (23409990)
2013
4
Long-term remission of T-cell large granular lymphocyte leukemia associated with rheumatoid arthritis after rituximab therapy. (23869084)
2013
5
STAT3 mutations are frequent in T-cell large granular lymphocytic leukemia with pure red cell aplasia. (24283217)
2013
6
Lymphocytosis: T-cell large granular lymphocyte leukemia. (23082329)
2012
7
Differential diagnosis of CD4-/CD8- I^I' T-cell large granular lymphocytic leukemia and hepatosplenic T-cell lymphoma. (22338065)
2012
8
Increased serum soluble IL-15RI+ levels in T-cell large granular lymphocyte leukemia. (22049515)
2012
9
STAT3 mutations unify the pathogenesis of chronic lymphoproliferative disorders of NK cells and T-cell large granular lymphocyte leukemia. (22859607)
2012
10
Bone marrow failure due to T-cell large granular lymphocytic leukemia in a patient with essential thrombocythemia. (20934219)
2011
11
A case report: Concurrent chronic myelomonocytic leukemia and T-cell large granular lymphocytic leukemia-type clonal proliferation as detected by multiparametric flow cytometry. (21337493)
2011
12
Imatinib mesylate in T-cell large granular lymphocyte leukemia associated with chronic graft-versus-host disease. (21663506)
2011
13
A case of concomitant T-cell large granular lymphocyte leukemia and plasma cell myeloma. (22252668)
2011
14
Long-term safety and efficacy of cyclosporin A therapy for T-cell large granular lymphocyte leukemia. (20038217)
2010
15
A novel mouse model for the aggressive variant of NK cell and T cell large granular lymphocyte leukemia. (19660811)
2010
16
CD20dim-positive T-cell large granular lymphocytic leukemia in a patient with concurrent hairy cell leukemia and plasma cell myeloma. (21151394)
2010
17
Lessons learned from an unusual presentation of CD3+, CD56- T-cell large granular lymphocyte leukemia. (20679608)
2010
18
MICA polymorphism identified by whole genome array associated with NKG2D-mediated cytotoxicity in T-cell large granular lymphocyte leukemia. (20460636)
2010
19
T-cell large granular lymphocyte leukemia associated with myelodysplastic syndrome: a clinicopathologic study of nine cases. (19228641)
2009
20
T cell large granular lymphocyte leukemia associated with rheumatoid arthritis and neutropenia. (19394280)
2009
21
T-cell large granular lymphocyte leukemia presenting as end-stage renal disease: the diagnostic role of flow-cytometric and clonality analysis of the urine sediment. (19203516)
2009
22
CD20-positive T-cell large granular lymphocyte leukemia: case report and review of the literature. (19687595)
2009
23
Protein-losing enteropathy due to T-cell large granular lymphocyte leukemia. (19307498)
2009
24
The gammadelta variant of T cell large granular lymphocyte leukemia is very similar to the common alphabeta type: report of two cases. (19669213)
2008
25
Outcomes of splenectomy in T-cell large granular lymphocyte leukemia with splenomegaly and cytopenia. (18550263)
2008
26
Malignant transformation of T-cell large granular lymphocyte leukemia in a dog. (17611371)
2007
27
Aggressive T-cell large granular lymphocyte leukemia: a case report and review of the literature. (17205534)
2007
28
CD20- and CD56-positive T-cell large granular lymphocyte leukemia in a human T-cell leukemia virus type 1 carrier. (18055343)
2007
29
T-cell large granular lymphocyte leukemia of donor origin after cord blood transplantation. (17875238)
2007
30
T-cell large granular lymphocyte leukemia: a cause of complex aphthosis not to be missed. (17637384)
2007
31
Fludarabine, mitoxantrone and dexamethasone as first-line treatment for T-cell large granular lymphocyte leukemia. (17525720)
2007
32
Characterization of T-cell large granular lymphocyte leukemia associated with Sjogren's syndrome-an important but under-recognized association. (16616153)
2006
33
Preclinical and phase I clinical trial of blockade of IL-15 using Mikbeta1 monoclonal antibody in T cell large granular lymphocyte leukemia. (16387851)
2006
34
Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review. (15958859)
2005
35
T-cell large granular lymphocyte leukemias have multiple phenotypic abnormalities involving pan-T-cell antigens and receptors for MHC molecules. (16416744)
2005
36
T-cell large granular lymphocytic leukemia of donor origin occurring after allogeneic bone marrow transplantation for B-cell lymphoproliferative disorders. (15842042)
2005
37
CD52 expression in T-cell large granular lymphocyte leukemia--implications for treatment with alemtuzumab. (16019510)
2005
38
Reversible paraneoplastic neuropathy associated with T-cell large granular lymphocyte leukemia. (15304606)
2004
39
T-cell large granular lymphocyte leukemia and related disorders. (15169980)
2004
40
Increased serum interleukin-18 in a patient with systemic lupus erythematosus and T-cell large granular lymphocytic leukemia. (17143689)
2004
41
T-cell large granular lymphocyte leukemia of donor origin after allogeneic bone marrow transplantation. (14560574)
2003
42
T-cell large granular lymphocyte leukemia is characterized by massive TCRBV-restricted clonal CD8 expansion and a generalized overexpression of the effector cell marker CD57. (12692516)
2003
43
Deletion 6q as a recurrent chromosomal aberration in T-cell large granular lymphocyte leukemia. (12547164)
2002
44
Pathologic quiz case: chronic anemia with red cell aplasia and lymphocytosis in a middle-aged man. T-cell large granular lymphocyte leukemia. (12503585)
2002
45
SjAPgren's syndrome associated T cell large granular lymphocyte leukemia: a possible common etiopathogenesis. (12180753)
2002
46
Establishment of the T-cell large granular lymphocyte leukemia cell line MOTN-1 carrying natural killer-cell antigens. (12127564)
2002
47
New t(11;12)(q12;q11) characterized by RxFISH in a patient with T-cell large granular lymphocyte leukemia. (11297771)
2001
48
Neutropenia associated with T-cell large granular lymphocyte leukemia: long-term response to cyclosporine therapy despite persistence of abnormal cells. (9558395)
1998
49
Humoral immune abnormalities in T-cell large granular lymphocyte leukemia. (9031118)
1996
50
Aggressive acute CD3+, CD56- T cell large granular lymphocyte leukemia with two stages of maturation arrest. (8751472)
1996

Variations for T-Cell Large Granular Lymphocyte Leukemia

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Expression for genes affiliated with T-Cell Large Granular Lymphocyte Leukemia

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with T-Cell Large Granular Lymphocyte Leukemia

Search GEO for disease gene expression data for T-Cell Large Granular Lymphocyte Leukemia.

Pathways for genes affiliated with T-Cell Large Granular Lymphocyte Leukemia

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Sources:
50PathCards, 38NCBI BioSystems Database, 60Thomson Reuters, 54R&D Systems, 30KEGG, 53QIAGEN, 55Reactome
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Pathways related to T-Cell Large Granular Lymphocyte Leukemia according to GeneCards/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5IL2RA, STAT3
2
Show member pathways
IL-2 Signaling pathway38
9.5IL2RA, STAT3
39.5IL2RA, STAT3
4
Show member pathways
Immune response IL 12 induced IFN gamma production60
9.5IL2RA, STAT3
59.5IL2RA, STAT3
6
Show member pathways
9.5IL2RA, IL15RA
7
Show member pathways
Type III interferon signaling38
9.0STAT3, IL2RA, IL15RA
8
Show member pathways
IL-9 Signaling Pathway38
Development Thrombopoietin regulated cell processes60
IL-7 Signaling Pathway38
Immune response IL 9 signaling pathway60
9.0IL15RA, IL2RA, STAT3
98.7KLRD1, KLRK1, KIR2DL1, KIR2DL3
10
Show member pathways
8.7KIR2DL3, KIR2DL1, KLRK1, KLRD1
11
Show member pathways
Immune response CD16 signaling in NK cells60
8.3MICA, KIR2DL3, KIR2DL1, KLRK1, KLRD1
12
Show member pathways
8.2STAT3, KIR2DL3, KIR2DL1, KLRK1, KLRD1
13
Show member pathways
7.6KLRD1, KIR2DL1, KIR2DL3, IL15RA, IL2RA, MICA

Compounds for genes affiliated with T-Cell Large Granular Lymphocyte Leukemia

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45Novoseek, 11DrugBank, 51PharmGKB, 61Tocris Bioscience, 24HMDB, 29IUPHAR, 3BitterDB
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Compounds related to T-Cell Large Granular Lymphocyte Leukemia according to GeneCards/GeneDecks:

(show all 29)
idCompoundScoreTop Affiliating Genes
1tuberculin4510.0IL2RA, LBR
2cuprophan4510.0LBR, IL2RA
3phorbol 12,13-dibutyrate4510.0IL2RA, LBR
4okt34510.0IL2RA, LBR
5con a4510.0LBR, IL2RA
6diethyl dithiocarbamate4510.0IL2RA, LBR
7sodium arsenite459.9LBR, IL2RA
8ouabain45 1110.9IL2RA, LBR
9polyinosinic-polycytidylic acid459.9KLRK1, STAT3
10infliximab45 51 1111.9IL2RA, LBR
11as 10145 6110.9IL2RA, STAT3
12pentoxifylline45 1110.8LBR, IL2RA
13ribavirin45 51 1111.8STAT3, LBR
14neopterin459.8LBR, IL2RA
15malondialdehyde459.7LBR, IL2RA
16ifn-alpha459.6IL2RA, STAT3
17cadmium45 2410.5LBR, STAT3, IL2RA
18rituximab45 51 1111.5IL2RA, STAT3
19vitamin-e459.5IL2RA, STAT3, LBR
20mifepristone45 29 61 1112.5LBR, STAT3, IL2RA
21rantes459.5IL2RA, STAT3, LBR
22rapamycin459.4IL2RA, STAT3, LBR
23genistein45 29 61 3 24 1114.4LBR, STAT3, IL2RA
24cyclosporin a45 29 6111.3IL2RA, STAT3, LBR
25heparin45 29 24 1112.1IL2RA, STAT3, LBR
26thymidine45 2410.0LBR, STAT3, IL2RA
27cisplatin45 51 61 1111.8IL2RA, STAT3, LBR
28arginine458.5GZMK, KLRK1, IL2RA, STAT3, LBR
29tyrosine458.1LBR, STAT3, IL2RA, KIR2DL3, KIR2DL1, KLRD1

GO Terms for genes affiliated with T-Cell Large Granular Lymphocyte Leukemia

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16Gene Ontology
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Cellular components related to T-Cell Large Granular Lymphocyte Leukemia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1plasma membraneGO:0058867.5KLRD1, KIR2DL1, KIR2DL3, IL15RA, IL2RA, STAT3

Biological processes related to T-Cell Large Granular Lymphocyte Leukemia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1JAK-STAT cascadeGO:0072599.3IL15RA, STAT3
2immune responseGO:0069559.1IL2RA, KIR2DL3, KIR2DL1
3regulation of immune responseGO:0507769.0KLRD1, KIR2DL1, KIR2DL3
4cell proliferationGO:0082839.0IL15RA, IL2RA, STAT3

Molecular functions related to T-Cell Large Granular Lymphocyte Leukemia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1antigen bindingGO:0038239.7KIR2DL3, MICA

Products for genes affiliated with T-Cell Large Granular Lymphocyte Leukemia

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Sources for T-Cell Large Granular Lymphocyte Leukemia

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet