T-PLL
MCID: TCL005
MIFTS: 49

T-Cell Prolymphocytic Leukemia (T-PLL) malady

Cancer diseases, Blood diseases, Rare diseases, Immune diseases categories
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Summaries for T-Cell Prolymphocytic Leukemia

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65Wikipedia, 33MalaCards
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Wikipedia:65 T-cell-prolymphocytic leukemia (T-PLL) is a mature T-cell leukemia with aggressive behavior and... more...

MalaCards: T-Cell Prolymphocytic Leukemia, also known as t-cell chronic lymphocytic leukemia, is related to prolymphocytic leukemia and leukemia. An important gene associated with T-Cell Prolymphocytic Leukemia is MS4A1 (membrane-spanning 4-domains, subfamily A, member 1), and among its related pathways are DNA damage ATM ATR regulation of G1 S checkpoint and ATM Pathway. The compounds neocarzinostatin and bpde have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone marrow and skin, and related mouse phenotypes are tumorigenesis and hematopoietic system.

Aliases & Classifications for T-Cell Prolymphocytic Leukemia

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49Orphanet, 62UMLS, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet, 25ICD10
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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
t-cell chronic lymphocytic leukemia:
Age of onset: Adulthood


Aliases & Descriptions:

t-cell prolymphocytic leukemia 49
t-cell chronic lymphocytic leukemia 49
prolymphocytic leukemia 62
t-pll 49


External Ids:

MESH via Orphanet36 D015461
ICD10 via Orphanet26 C91.3
SNOMED-CT via Orphanet59 128821006, 277567002
UMLS via Orphanet63 C2363142
ICD1025 C91.6

Related Diseases for T-Cell Prolymphocytic Leukemia

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17GeneCards, 18GeneDecks
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Diseases in the B Cell Prolymphocytic Leukemia family:

t-cell prolymphocytic leukemia

Diseases related to T-Cell Prolymphocytic Leukemia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 45)
idRelated DiseaseScoreTop Affiliating Genes
1prolymphocytic leukemia32.2MTCP1
2leukemia31.8ATM, CHEK2, TCL1A, MTCP1, CDKN1B, CD52
3b-cell lymphomas30.6CDKN1B, MS4A1, TCL1A, ATM
4nijmegen breakage syndrome30.5ATM, CHEK2
5hodgkin's lymphoma30.5ATM, TCL1A, MS4A1
6t-cell leukemia30.5ATM, TCL1A, MTCP1
7chronic lymphocytic leukemia30.5ATM, TCL1A, MS4A1, CD52
8ataxia telangiectasia30.4ATM, CHEK2, TCL1A, MTCP1
9breast cancer30.3ATM, VEGFA, CHEK2, CDKN1B
10diffuse large b-cell lymphoma10.4
11sarcoma10.4
12t-cell prolymphocytic leukemia, somatic10.4
13acute liver failure10.3
14t-cell large granular lymphocyte leukemia10.3
15panuveitis10.3
16vasculitis10.3
17large granular lymphocyte leukemia10.3
18ataxia10.3
19endotheliitis10.3
20pemphigus10.3
21malignant histiocytosis10.3
22megaloblastic anemia10.3
23histiocytosis10.3
24pemphigus foliaceus10.3
25peripheral t-cell lymphoma10.1MS4A1, TCL1A
26bilateral breast cancer10.1ATM, CHEK2
27li-fraumeni syndrome10.1CHEK2, ATM
28burkitt's lymphoma10.0TCL1A, CHEK2, ATM
29follicular lymphoma10.0CDKN1B, MS4A1, TCL1A
30lung cancer10.0CHEK2, VEGFA, ATM
31hypersensitivity reaction type ii disease10.0HLA-S, MS4A1, CD52
32ipex syndrome10.0ATM, HLA-S, SMARCB1, MS4A1
33hematologic cancer10.0ATM, VEGFA, CDKN1B
34esophageal cancer10.0CDKN1B, CHEK2
35b-cell chronic lymphocytic leukemia10.0ATM, TCL1A, MS4A1, CDKN1B
36acute lymphoblastic leukemia, childhood10.0CDKN1B, ATM
37colorectal cancer10.0ATM, VEGFA, CDKN1B
38stomach cancer9.9CDKN1B, CHEK2, VEGFA
39non-hodgkin lymphoma9.9ATM, TCL1A, MS4A1, CDKN1B, CD52
40ovarian cancer9.9CDKN1B, CHEK2, VEGFA, ATM
41retinoblastoma9.9SMARCB1, CHEK2, CDKN1B
42colon cancer9.9VEGFA, CHEK2, CDKN1B
43melanoma9.9CDKN1B, CHEK2, VEGFA, ATM
44prostate cancer9.9CDKN1B, CHEK2, VEGFA, ATM
45glioblastoma multiforme9.9ATM, SMARCB1, VEGFA, CHEK2, CDKN1B

Graphical network of the top 20 diseases related to T-Cell Prolymphocytic Leukemia:



Diseases related to t-cell prolymphocytic leukemia

Symptoms for T-Cell Prolymphocytic Leukemia

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Drugs & Therapeutics for T-Cell Prolymphocytic Leukemia

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Sources:
42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for T-Cell Prolymphocytic Leukemia

Search NIH Clinical Center for T-Cell Prolymphocytic Leukemia

Genetic Tests for T-Cell Prolymphocytic Leukemia

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Anatomical Context for T-Cell Prolymphocytic Leukemia

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33MalaCards
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MalaCards organs/tissues related to T-Cell Prolymphocytic Leukemia:

33
T cells, Bone marrow, Skin, Liver, Spleen, Bone, Lymph node, B cells, Heart, Breast, Eye, Endothelial

Animal Models for T-Cell Prolymphocytic Leukemia or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to T-Cell Prolymphocytic Leukemia:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.6CDKN1B, CHEK2, VEGFA, SMARCB1, ATM
2MP:00053978.2ATM, SMARCB1, VEGFA, CHEK2, MS4A1, CDKN1B
3MP:00053898.1CDKN1B, TCL1A, VEGFA, SMARCB1, ATM
4MP:00053878.0ATM, SMARCB1, VEGFA, CHEK2, MS4A1, CDKN1B

Publications for T-Cell Prolymphocytic Leukemia

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52PubMed
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Articles related to T-Cell Prolymphocytic Leukemia:

(show top 50)    (show all 108)
idTitleAuthorsYear
1
Immunophenotypic characterization of T-cell prolymphocytic leukemia. (24124154)
2013
2
Recurrent JAK1 and JAK3 somatic mutations in T-cell prolymphocytic leukemia. (24048415)
2013
3
Cutaneous presentation of T-cell prolymphocytic leukemia. (23513557)
2013
4
Sequential chemoimmunotherapy of fludarabine, mitoxantrone, and cyclophosphamide induction followed by alemtuzumab consolidation is effective in T-cell prolymphocytic leukemia. (23512246)
2013
5
T-cell prolymphocytic leukemia (T-PLL) with overlapping cytomorphological features with T-CLL and T-ALL: a case initially diagnosed by fine-needle aspiration cytology and immunocytochemistry. (21987484)
2013
6
T-cell prolymphocytic leukemia in Japan: is it a variant? (22527853)
2012
7
Pediatric T-cell prolymphocytic leukemia with an isolated 12(p13) deletion and aberrant CD117 expression. (23211022)
2012
8
"T-cell prolymphocytic leukemia (T-PLL), a heterogeneous disease exemplified by two cases and the important role of cytogenetics: a multidisciplinary approach". (23211026)
2012
9
Erythematous eruption with marked conjunctival injection--quiz case. Diagnosis: leukemia cutis with conjunctival involvement in the setting of T-cell prolymphocytic leukemia (T-PLL). (23069962)
2012
10
Cost-effectiveness of alemtuzumab for T-cell prolymphocytic leukemia. (22980700)
2012
11
Case Study Interpretation--Houston: Case 4. T-cell prolymphocytic leukemia. (21638771)
2011
12
Guillain-BarrAc syndrome after use of alemtuzumab (Campath) in a patient with T-cell prolymphocytic leukemia: a case report and review of the literature. (20362332)
2010
13
T-cell prolymphocytic leukemia: A rare disease in an elderly female. (21129072)
2010
14
T cell-prolymphocytic leukemia detected in a patient of breast cancer at the time of recurrence: a case report. (20076807)
2010
15
The evolving management of a rare lymphoproliferative disorder-T-cell prolymphocytic leukemia. (19714590)
2009
16
Recurrent loss, but lack of mutations, of the SMARCB1 tumor suppressor gene in T-cell prolymphocytic leukemia with TCL1A-TCRAD juxtaposition. (19480937)
2009
17
Translocations t(X;14)(q28;q11) and t(Y;14)(q12;q11) in T-cell prolymphocytic leukemia. (18294235)
2009
18
T-cell prolymphocytic leukemia. (19275513)
2009
19
High TCL1 expression and intact T-cell receptor signaling define a hyperproliferative subset of T-cell prolymphocytic leukemia. (17890451)
2008
20
Detection of Epstein-Barr virus in T-cell prolymphocytic leukemia cells in vitro. (18790666)
2008
21
Rhodococcus equi infection after alemtuzumab therapy for T-cell prolymphocytic leukemia. (18258054)
2007
22
Short telomeres and high telomerase activity in T-cell prolymphocytic leukemia. (17898784)
2007
23
Clinically and genetically atypical T-cell prolymphocytic leukemia underlines the relevance of a multidisciplinary diagnostic approach. (17405754)
2007
24
Successful treatment of T-cell prolymphocytic leukemia with full-intensity conditioning followed by matched unrelated donor allogeneic stem cell transplantation. (18185486)
2007
25
Combined single nucleotide polymorphism-based genomic mapping and global gene expression profiling identifies novel chromosomal imbalances, mechanisms and candidate genes important in the pathogenesis of T-cell prolymphocytic leukemia with inv(14)(q11q32). (17713554)
2007
26
Bortezomib induces apoptosis in T-cell prolymphocytic leukemia (T-PLL). (17990182)
2007
27
T-cell prolymphocytic leukemia. (16645226)
2006
28
Small cell variant of T-cell prolymphocytic leukemia exhibiting suppressor phenotype. (16966296)
2006
29
Periorbital edema as the initial presentation of T-cell prolymphocytic leukemia. (16714934)
2006
30
Allogeneic stem cell transplantation after reduced-intensity conditioning in a patient with T-cell prolymphocytic leukemia: graft-versus-tumor effect and long-term remission. (16474410)
2006
31
T-cell prolymphocytic leukemia involving extramedullary sites. (15716243)
2005
32
Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review. (15958859)
2005
33
T- cell prolymphocytic leukemia - a rare case. (16141512)
2005
34
Mismatched unrelated cord blood transplantation in a patient with T-cell prolymphocytic leukemia. (15690068)
2005
35
T-cell prolymphocytic leukemia: update and focus on alemtuzumab (Campath-1H). (12623420)
2003
36
Indolent T-cell prolymphocytic leukemia: a case report and a review of the literature. (14508807)
2003
37
Campath-1H treatment of T-cell prolymphocytic leukemia in patients for whom at least one prior chemotherapy regimen has failed. (11773171)
2002
38
CD3-negative, CD20-positive T-cell prolymphocytic leukemia: case report and review of the literature. (12447967)
2002
39
T-cell prolymphocytic leukemia with hemorrhagic gastrointestinal involvement and a new chromosomal abnormality. (11999363)
2002
40
Recurrent molecular deletion of the 12p13 region, centromeric to ETV6/TEL, in T-cell prolymphocytic leukemia. (11920168)
2000
41
Improvement of quality of life after splenectomy in an HTLV-I carrier with T-cell prolymphocytic leukemia. (10609799)
1999
42
T-cell prolymphocytic leukemia with a novel translocation (6;11)(q21;q23). (10347553)
1999
43
Abnormalities of the short arm of chromosome 12 in T cell prolymphocytic leukemia. (9639428)
1998
44
IL-2-induced growth of CD8+ T cell prolymphocytic leukemia cells mediated by NF-kappaB induction and IL-2 receptor alpha expression. (9619918)
1998
45
Perforin-positive leukemic cell infiltration in the aortic tissue of a patient with T-cell prolymphocytic leukemia. (8971584)
1996
46
Complete remission in T-cell prolymphocytic leukemia with 2-chlorodeoxyadenosine. (7738636)
1995
47
Bone marrow histological patterns in B-cell and T-cell prolymphocytic leukemia. (2777734)
1989
48
Functional and phenotypic analysis of a T cell prolymphocytic leukemia. (3159942)
1985
49
Cytogenetic studies in a case of T-cell prolymphocytic leukemia. (6609763)
1984
50
T-cell prolymphocytic leukemia with a suppressor phenotype. (6223570)
1983

Variations for T-Cell Prolymphocytic Leukemia

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1 National Center for Biotechnology Information (Clinvar)
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Clinvar genetic disease variations for T-Cell Prolymphocytic Leukemia:

1
id Gene Name Type Significance SNP ID Assembly Location
1NM_000051.3(ATM): c.7636_7644delTCTAGAATT (p.Arg2547_Ser2549del)deletionPathogenic/card/t_cell_prolymphocytic_leukemiaGRCh38Chr 11, 108331885: 108331893

Expression for genes affiliated with T-Cell Prolymphocytic 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 Prolymphocytic Leukemia

Search GEO for disease gene expression data for T-Cell Prolymphocytic Leukemia.

Pathways for genes affiliated with T-Cell Prolymphocytic Leukemia

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50PathCards, 60Thomson Reuters, 53QIAGEN, 38NCBI BioSystems Database, 55Reactome, 57SinoBiological, 51PharmGKB, 30KEGG, 5Cell Signaling Technology
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Pathways related to T-Cell Prolymphocytic Leukemia according to GeneCards/GeneDecks:

(show all 25)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8CHEK2, ATM
2
Show member pathways
ATM Signaling Pathway38
9.8ATM, CHEK2
39.8CHEK2, ATM
49.8CHEK2, ATM
5
Show member pathways
9.8ATM, CHEK2
6
Show member pathways
9.8ATM, CHEK2
7
Show member pathways
p53 pathway38
9.8CHEK2, ATM
89.8CDKN1B, ATM
99.8CDKN1B, ATM
109.7VEGFA, ATM
119.7SMARCB1, CDKN1B
12
Show member pathways
Development Ligand dependent activation of the ESR1 AP 1 pathway60
9.6VEGFA, SMARCB1
139.6CDKN1B, VEGFA
149.6CDKN1B, VEGFA
15
Show member pathways
9.5CHEK2, SMARCB1, ATM
16
Show member pathways
DNA damage response38
9.4CDKN1B, CHEK2, ATM
17
Show member pathways
Cell cycle38
9.4ATM, CHEK2, CDKN1B
18
Show member pathways
9.4CDKN1B, CHEK2, ATM
19
Show member pathways
9.4CDKN1B, CHEK2, ATM
209.4CHEK2, VEGFA, ATM
21
Show member pathways
9.3CDKN1B, VEGFA, ATM
229.3CDKN1B, VEGFA, ATM
23
Show member pathways
Signaling Pathways in Glioblastoma38
9.3ATM, VEGFA, CDKN1B
249.0CDKN1B, CHEK2, VEGFA, ATM
258.3VEGFA, TCL1A, MTCP1, CDKN1B

Compounds for genes affiliated with T-Cell Prolymphocytic Leukemia

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

(show all 24)
idCompoundScoreTop Affiliating Genes
1neocarzinostatin4510.2CHEK2, ATM
2bpde4510.1CHEK2, ATM
3temozolomide45 1110.9VEGFA, CHEK2
4cetuximab45 51 1111.8VEGFA, CDKN1B
5topotecan45 1110.8VEGFA, ATM
6aphidicolin459.8CDKN1B, CHEK2, ATM
7ns 39845 6110.8CDKN1B, VEGFA
8erlotinib45 51 1111.7CDKN1B, VEGFA
9camptothecin45 61 1111.7CDKN1B, CHEK2, ATM
10etoposide45 51 61 1112.6CDKN1B, CHEK2, ATM
11trastuzumab45 51 1111.6CDKN1B, VEGFA
12fludarabine45 51 1111.6CD52, CDKN1B, MS4A1
13gemcitabine45 51 1111.6CDKN1B, CHEK2, VEGFA
14paraffin459.3ATM, VEGFA, MS4A1, CDKN1B
15wortmannin459.3CDKN1B, CHEK2, ATM
16n acetylcysteine459.3ATM, VEGFA, CHEK2, CDKN1B
17ly294002459.3ATM, VEGFA, CDKN1B
18flavopiridol45 1110.3CDKN1B, VEGFA
19rapamycin459.3ATM, VEGFA, CHEK2, CDKN1B
20doxorubicin45 51 1111.3CDKN1B, CHEK2, VEGFA, ATM
21genistein45 29 61 3 24 1114.3ATM, VEGFA, CHEK2, CDKN1B
22cisplatin45 51 61 1112.2ATM, VEGFA, CHEK2, CDKN1B
23cyclophosphamide45 51 1111.1CDKN1B, MS4A1, VEGFA, HLA-S
24threonine458.8CDKN1B, TCL1A, CHEK2, ATM

GO Terms for genes affiliated with T-Cell Prolymphocytic Leukemia

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

idNameGO IDScoreTop Affiliating Genes
1chromosome, telomeric regionGO:0007819.5CHEK2, ATM
2nucleoplasmGO:0056549.0CDKN1B, CHEK2, SMARCB1, ATM

Biological processes related to T-Cell Prolymphocytic Leukemia according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1DNA damage induced protein phosphorylationGO:0069759.9CHEK2, ATM
2replicative senescenceGO:0903999.9CHEK2, ATM
3DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrestGO:0069779.8CDKN1B, ATM
4response to ionizing radiationGO:0102129.8BRCC3, ATM
5double-strand break repairGO:0063029.7CHEK2, BRCC3, ATM
6intrinsic apoptotic signaling pathway in response to DNA damageGO:0086309.5CHEK2, ATM
7response to hypoxiaGO:0016669.3ATM, VEGFA, CDKN1B

Products for genes affiliated with T-Cell Prolymphocytic Leukemia

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Sources for T-Cell Prolymphocytic 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