T-PLL
MCID: TCL005
MIFTS: 49

T-Cell Prolymphocytic Leukemia (T-PLL) malady

Cancer diseases, Blood diseases, Rare diseases, Immune diseases categories

Summaries for T-Cell Prolymphocytic Leukemia

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Wikipedia:64 T-cell-prolymphocytic leukemia (T-PLL) is a mature T-cell leukemia with aggressive behavior and... more...

MalaCards based summary: T-Cell Prolymphocytic Leukemia, also known as leukemia, lymphocytic, chronic, t-cell, 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 bone, and related mouse phenotypes are tumorigenesis and hematopoietic system.

Aliases & Classifications for T-Cell Prolymphocytic Leukemia

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Sources:
48Orphanet, 61UMLS, 35MESH via Orphanet, 27ICD10 via Orphanet, 62UMLS via Orphanet, 26ICD10
See all sources

T-Cell Prolymphocytic Leukemia, Aliases & Descriptions:

Name: T-Cell Prolymphocytic Leukemia 48
Leukemia, Lymphocytic, Chronic, T-Cell 61
T-Cell Chronic Lymphocytic Leukemia 48
 
Leukemia, Prolymphocytic, T-Cell 61
T-Pll 48


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
t-cell chronic lymphocytic leukemia:
Age of onset: Adulthood


External Ids:

MESH via Orphanet35 D015461
ICD10 via Orphanet27 C91.3
UMLS via Orphanet62 C2363142
ICD1026 C91.6

Related Diseases for T-Cell Prolymphocytic Leukemia

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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 47)
idRelated DiseaseScoreTop Affiliating Genes
1prolymphocytic leukemia32.8MTCP1
2leukemia31.2CD52, MTCP1, TCL1A, CHEK2, ATM, CDKN1B
3t-cell leukemia30.9TCL1A, MTCP1, ATM
4nijmegen breakage syndrome30.8ATM, CHEK2
5hodgkin's lymphoma30.8MS4A1, TCL1A, ATM
6chronic lymphocytic leukemia30.5CD52, MS4A1, TCL1A, ATM
7b-cell lymphomas30.5MS4A1, ATM, TCL1A, CDKN1B
8ataxia telangiectasia30.4CHEK2, ATM, TCL1A, MTCP1
9breast cancer30.0CDKN1B, CHEK2, VEGFA, ATM
10diffuse large b-cell lymphoma10.5
11t-cell large granular lymphocyte leukemia10.5
12sarcoma10.5
13large granular lymphocyte leukemia10.5
14t-cell prolymphocytic leukemia, somatic10.5
15acute liver failure10.3
16anterior scleritis10.3
17panuveitis10.3
18scleritis10.3
19thyroiditis10.3
20vasculitis10.3
21ataxia10.3
22endotheliitis10.3
23pemphigus foliaceus10.3
24megaloblastic anemia10.3
25pemphigus10.3
26malignant histiocytosis10.3
27histiocytosis10.3
28peripheral t-cell lymphoma10.3TCL1A, MS4A1
29bilateral breast cancer10.3CHEK2, ATM
30li-fraumeni syndrome10.3CHEK2, ATM
31burkitt's lymphoma10.1TCL1A, CHEK2, ATM
32follicular lymphoma10.0CDKN1B, MS4A1, TCL1A
33lung cancer10.0VEGFA, ATM, CHEK2
34hypersensitivity reaction type ii disease10.0HLA-S, MS4A1, CD52
35ipex syndrome9.9ATM, HLA-S, SMARCB1, MS4A1
36hematologic cancer9.9ATM, CDKN1B, VEGFA
37esophageal cancer9.9CHEK2, CDKN1B
38acute lymphoblastic leukemia, childhood9.8CDKN1B, ATM
39colorectal cancer9.8ATM, VEGFA, CDKN1B
40stomach cancer9.8CHEK2, CDKN1B, VEGFA
41non-hodgkin lymphoma9.7MS4A1, CDKN1B, ATM, TCL1A, CD52
42ovarian cancer9.7CHEK2, CDKN1B, VEGFA, ATM
43retinoblastoma9.7SMARCB1, CDKN1B, CHEK2
44colonic benign neoplasm9.7CHEK2, CDKN1B, VEGFA
45melanoma9.6CDKN1B, ATM, VEGFA, CHEK2
46prostate cancer9.6ATM, VEGFA, CHEK2, CDKN1B
47glioblastoma9.4CDKN1B, ATM, SMARCB1, VEGFA, CHEK2

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

32
T cells, Bone marrow, Bone, Spleen, Liver, Skin, Lymph node, B cells, Eye, Brain, Heart, Thyroid, Breast, Endothelial

Animal Models for T-Cell Prolymphocytic Leukemia or affiliated genes

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MGI Mouse Phenotypes related to T-Cell Prolymphocytic Leukemia:

36
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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Articles related to T-Cell Prolymphocytic Leukemia:

(show top 50)    (show all 116)
idTitleAuthorsYear
1
T-cell prolymphocytic leukemia in a 63-year-old female with a pre-existing T-cell large granular lymphocytic leukemia: Metachronous T-cell leukemias with discordant subset restrictions (CD4 versus CD8) and distinct clonal identities. (25238936)
2014
2
Identification of a novel SEPT9-ABL1 fusion gene in a patient with T-cell prolymphocytic leukemia. (25068103)
2014
3
Immunophenotypic characterization of T-cell prolymphocytic leukemia. (24124154)
2013
4
Recurrent JAK1 and JAK3 somatic mutations in T-cell prolymphocytic leukemia. (24048415)
2013
5
Cutaneous presentation of T-cell prolymphocytic leukemia. (23513557)
2013
6
Sequential chemoimmunotherapy of fludarabine, mitoxantrone, and cyclophosphamide induction followed by alemtuzumab consolidation is effective in T-cell prolymphocytic leukemia. (23512246)
2013
7
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
8
T-cell prolymphocytic leukemia in Japan: is it a variant? (22527853)
2012
9
Pediatric T-cell prolymphocytic leukemia with an isolated 12(p13) deletion and aberrant CD117 expression. (23211022)
2012
10
"T-cell prolymphocytic leukemia (T-PLL), a heterogeneous disease exemplified by two cases and the important role of cytogenetics: a multidisciplinary approach". (23211026)
2012
11
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
12
Cost-effectiveness of alemtuzumab for T-cell prolymphocytic leukemia. (22980700)
2012
13
Case Study Interpretation--Houston: Case 4. T-cell prolymphocytic leukemia. (21638771)
2011
14
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
15
T-cell prolymphocytic leukemia: A rare disease in an elderly female. (21129072)
2010
16
The evolving management of a rare lymphoproliferative disorder-T-cell prolymphocytic leukemia. (19714590)
2009
17
Recurrent loss, but lack of mutations, of the SMARCB1 tumor suppressor gene in T-cell prolymphocytic leukemia with TCL1A-TCRAD juxtaposition. (19480937)
2009
18
Translocations t(X;14)(q28;q11) and t(Y;14)(q12;q11) in T-cell prolymphocytic leukemia. (18294235)
2009
19
T-cell prolymphocytic leukemia. (19275513)
2009
20
High TCL1 expression and intact T-cell receptor signaling define a hyperproliferative subset of T-cell prolymphocytic leukemia. (17890451)
2008
21
Detection of Epstein-Barr virus in T-cell prolymphocytic leukemia cells in vitro. (18790666)
2008
22
Rhodococcus equi infection after alemtuzumab therapy for T-cell prolymphocytic leukemia. (18258054)
2007
23
Short telomeres and high telomerase activity in T-cell prolymphocytic leukemia. (17898784)
2007
24
Clinically and genetically atypical T-cell prolymphocytic leukemia underlines the relevance of a multidisciplinary diagnostic approach. (17405754)
2007
25
Successful treatment of T-cell prolymphocytic leukemia with full-intensity conditioning followed by matched unrelated donor allogeneic stem cell transplantation. (18185486)
2007
26
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
27
Bortezomib induces apoptosis in T-cell prolymphocytic leukemia (T-PLL). (17990182)
2007
28
T-cell prolymphocytic leukemia. (16645226)
2006
29
Small cell variant of T-cell prolymphocytic leukemia exhibiting suppressor phenotype. (16966296)
2006
30
Periorbital edema as the initial presentation of T-cell prolymphocytic leukemia. (16714934)
2006
31
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
32
T-cell prolymphocytic leukemia involving extramedullary sites. (15716243)
2005
33
Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review. (15958859)
2005
34
T- cell prolymphocytic leukemia - a rare case. (16141512)
2005
35
Small cell variant of T-cell prolymphocytic leukemia with a gammadelta immunophenotype. (15717692)
2005
36
Mismatched unrelated cord blood transplantation in a patient with T-cell prolymphocytic leukemia. (15690068)
2005
37
T-cell prolymphocytic leukemia: update and focus on alemtuzumab (Campath-1H). (12623420)
2003
38
Indolent T-cell prolymphocytic leukemia: a case report and a review of the literature. (14508807)
2003
39
Campath-1H treatment of T-cell prolymphocytic leukemia in patients for whom at least one prior chemotherapy regimen has failed. (11773171)
2002
40
CD3-negative, CD20-positive T-cell prolymphocytic leukemia: case report and review of the literature. (12447967)
2002
41
Recurrent molecular deletion of the 12p13 region, centromeric to ETV6/TEL, in T-cell prolymphocytic leukemia. (11920168)
2000
42
Improvement of quality of life after splenectomy in an HTLV-I carrier with T-cell prolymphocytic leukemia. (10609799)
1999
43
T-cell prolymphocytic leukemia with a novel translocation (6;11)(q21;q23). (10347553)
1999
44
Abnormalities of the short arm of chromosome 12 in T cell prolymphocytic leukemia. (9639428)
1998
45
IL-2-induced growth of CD8+ T cell prolymphocytic leukemia cells mediated by NF-kappaB induction and IL-2 receptor alpha expression. (9619918)
1998
46
Perforin-positive leukemic cell infiltration in the aortic tissue of a patient with T-cell prolymphocytic leukemia. (8971584)
1996
47
Complete remission in T-cell prolymphocytic leukemia with 2-chlorodeoxyadenosine. (7738636)
1995
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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Clinvar genetic disease variations for T-Cell Prolymphocytic Leukemia:

7
id Gene Name Type Significance SNP ID Assembly Location
1NM_000051.3(ATM): c.7636_7644delTCTAGAATT (p.Arg2547_Ser2549del)deletionPathogenicGRCh38Chr 11, 108331885: 108331893

Expression for genes affiliated with T-Cell Prolymphocytic Leukemia

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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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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 Pathway37
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 pathway37
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 pathway59
9.6VEGFA, SMARCB1
139.6CDKN1B, VEGFA
149.6CDKN1B, VEGFA
15
Show member pathways
9.5CHEK2, SMARCB1, ATM
16
Show member pathways
DNA damage response37
9.4CDKN1B, CHEK2, ATM
17
Show member pathways
Cell cycle37
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 Glioblastoma37
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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Compounds related to T-Cell Prolymphocytic Leukemia according to GeneCards/GeneDecks:

(show all 24)
idCompoundScoreTop Affiliating Genes
1neocarzinostatin4410.2CHEK2, ATM
2bpde4410.1CHEK2, ATM
3temozolomide44 1210.9VEGFA, CHEK2
4cetuximab44 50 1211.8VEGFA, CDKN1B
5topotecan44 1210.8VEGFA, ATM
6aphidicolin449.8CDKN1B, CHEK2, ATM
7ns 39844 6010.8CDKN1B, VEGFA
8erlotinib44 50 1211.7CDKN1B, VEGFA
9camptothecin44 60 1211.7CDKN1B, CHEK2, ATM
10etoposide44 50 60 1212.6CDKN1B, CHEK2, ATM
11trastuzumab44 50 1211.6CDKN1B, VEGFA
12fludarabine44 50 1211.6CD52, CDKN1B, MS4A1
13gemcitabine44 50 1211.6CDKN1B, CHEK2, VEGFA
14paraffin449.3ATM, VEGFA, MS4A1, CDKN1B
15wortmannin449.3CDKN1B, CHEK2, ATM
16n acetylcysteine449.3ATM, VEGFA, CHEK2, CDKN1B
17ly294002449.3ATM, VEGFA, CDKN1B
18flavopiridol44 1210.3CDKN1B, VEGFA
19rapamycin449.3ATM, VEGFA, CHEK2, CDKN1B
20doxorubicin44 50 1211.3CDKN1B, CHEK2, VEGFA, ATM
21genistein44 29 60 3 25 1214.3ATM, VEGFA, CHEK2, CDKN1B
22cisplatin44 50 60 1212.2ATM, VEGFA, CHEK2, CDKN1B
23cyclophosphamide44 50 1211.1CDKN1B, MS4A1, VEGFA, HLA-S
24threonine448.8CDKN1B, TCL1A, CHEK2, ATM

GO Terms for genes affiliated with T-Cell Prolymphocytic Leukemia

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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
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
29IUPHAR
30KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet