T-PLL
MCID: TCL005
MIFTS: 44

T-Cell Prolymphocytic Leukemia (T-PLL) malady

Cancer, Blood, Immune categories

Summaries for T-Cell Prolymphocytic Leukemia

Sources:
64Wikipedia, 33MalaCards
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Wikipedia:64 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 t-cell leukemia and nijmegen breakage syndrome. An important gene associated with T-Cell Prolymphocytic Leukemia is CD19 (CD19 molecule), and among its related pathways are Apoptosis Pathway and PLK3 signaling events. The compounds neocarzinostatin and ricin have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, lymph node and spleen, and related mouse phenotypes are tumorigenesis and normal.

Aliases & Classifications for T-Cell Prolymphocytic Leukemia

Sources:
49Orphanet, 61UMLS, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

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


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 61
t-pll 49


External Ids:

MESH via Orphanet36 D015461
ICD10 via Orphanet26 C91.3
SNOMED-CT via Orphanet58 128821006, 277567002
UMLS via Orphanet62 C2363142
ICD1025 C91.6

Related Diseases for T-Cell Prolymphocytic Leukemia

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 99)
idRelated DiseaseScoreTop Affiliating Genes
1t-cell leukemia31.4ZAP70, ATM, MTCP1, NFKB1, CD7, TCL1A
2nijmegen breakage syndrome30.4ATM, CHEK2
3ataxia telangiectasia30.4ATM, MTCP1, CHEK2, TCL1A, TP53
4acute leukemia30.4ATM, ANPEP, ETV6, CD7, SMARCB1
5macroglobulinemia30.1CD19, CD5
6macroglobulinemia, waldenstrom, somatic30.1MS4A1, ZAP70
7acute lymphocytic leukemia30.1ZAP70, ANPEP, ETV6, CD5, CD19, CD38
8b cell prolymphocytic leukemia10.8
9leukemia/lymphoma, t-cell10.5
10kaposi's sarcoma10.4
11t-cell prolymphocytic leukemia, somatic10.4
12lymphoma, diffuse large cell10.4
13acute liver failure10.3
14diffuse large b-cell lymphoma10.3
15n syndrome10.3
16vasculitis10.3
17large granular lymphocyte leukemia10.3
18guillain-barr� syndrome10.3
19ataxia10.3
20pemphigus10.3
21malignant histiocytosis10.3
22megaloblastic anemia10.3
23leukemia, t-cell, chronic10.3
24pemphigus foliaceus10.3
25graft versus host disease10.2
26richter's syndrome10.2
27splenic manifestation of prolymphocytic leukemia10.2
28central nervous system leukemia10.2
29mantle cell lymphoma10.2
30acute t cell leukemia10.1
31leukostasis10.1
32lymphosarcoma10.1
33adult syndrome10.1
34adult lymphoma10.1
35thrombocytopenia10.1
36acute graft versus host disease10.1
37cold agglutinin disease10.1
38chronic graft versus host disease10.1
39waldenstrom macroglobulinemia10.1
40hematopoietic stem cell transplantation10.1
41arthritis10.0ANPEP
42prolymphocytic leukemia10.0MTCP1
43hepatitis10.0ANPEP
44hepatitis c10.0HLA-S
45amyloidosis10.0IGKC
46laryngeal squamous cell carcinoma10.0TP53
47deficiency anemia10.0CD5, CD19
48gastrointestinal system disease10.0CD19
49esophageal cancer10.0TP53
50severe combined immunodeficiency10.0CD38, ZAP70

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



Diseases related to t-cell prolymphocytic leukemia

Clinical Features for T-Cell Prolymphocytic Leukemia

Drugs & Therapeutics for T-Cell Prolymphocytic Leukemia

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for T-Cell Prolymphocytic Leukemia

Drug clinical trials:

Search ClinicalTrials for T-Cell Prolymphocytic Leukemia

Search NIH Clinical Center for T-Cell Prolymphocytic Leukemia

Search CenterWatch for T-Cell Prolymphocytic Leukemia

Genetic Tests for T-Cell Prolymphocytic Leukemia

Anatomical Context for T-Cell Prolymphocytic Leukemia

Sources:
33MalaCards
See all sources

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

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

Animal Models for T-Cell Prolymphocytic Leukemia or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to T-Cell Prolymphocytic Leukemia:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.6CHEK2, ETV6, CD19, CDKN1B, SMARCB1
2MP:00028738.3TP53, CD52, CD19, CD5, ETV6, NFKB1
3MP:00053846.6ETV6, IGKC, NFKB1, CHEK2, ATM, ZAP70
4MP:00053976.5IGKC, NFKB1, ANPEP, CHEK2, ATM, ZAP70
5MP:00053876.4TP53, ZAP70, ATM, CHEK2, ANPEP, NFKB1

Publications for T-Cell Prolymphocytic Leukemia

Sources:
51PubMed
See all sources

Articles related to T-Cell Prolymphocytic Leukemia:

(show top 50)    (show all 51)
idTitleAuthorsYear
1
Smudge cells following treatment with pentostatin in a patient with B-cell prolymphocytic leukemia. (24049774)
2013
2
Reply to "A combination treatment approach and cord blood stem cell transplant for blastic plasmacytoid dendritic cell neoplasm" and to "A low-grade B-cell lymphoma with prolymphocytic/paraimmunoblastic proliferation and IRF4 rearrangement" dendritic cell leukemia. (23449597)
2013
3
Cerebriform variant type of T cell prolymphocytic leukemia with complex karyotype including an additional segment at 1p36.1. (23054642)
2012
4
TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients. (22189846)
2012
5
Epstein-Barr virus-negative classical Hodgkin's lymphoma in a patient with T-cell prolymphocytic leukemia treated with fludarabine. (22706535)
2012
6
Hematopoietic stem cell transplantation in T-prolymphocytic leukemia: a retrospective study from the European Group for Blood and Marrow Transplantation and the Royal Marsden Consortium. (22116553)
2012
7
CD30-positive EBV-associated diffuse large B-cell lymphoma occurring after immunosuppressive therapy for T-cell prolymphocytic leukemia. (21454193)
2011
8
Alemtuzumab therapy in T-cell prolymphocytic leukemia: comparing efficacy in a series treated intravenously and a study piloting the subcutaneous route. (21948296)
2011
9
A case of T cell prolymphocytic leukemia involving blast transformation. (21505955)
2011
10
Allogeneic hematopoietic cell transplant for prolymphocytic leukemia. (19961946)
2010
11
T cell-prolymphocytic leukemia detected in a patient of breast cancer at the time of recurrence: a case report. (20076807)
2010
12
Epstein-barr virus-positive diffuse large B-cell lymphoma during therapy with alemtuzumab for T-cell prolymphocytic leukemia. (19917859)
2010
13
Recurrent loss, but lack of mutations, of the SMARCB1 tumor suppressor gene in T-cell prolymphocytic leukemia with TCL1A-TCRAD juxtaposition. (19480937)
2009
14
Translocations t(X;14)(q28;q11) and t(Y;14)(q12;q11) in T-cell prolymphocytic leukemia. (18294235)
2009
15
High TCL1 expression and intact T-cell receptor signaling define a hyperproliferative subset of T-cell prolymphocytic leukemia. (17890451)
2008
16
Rhodococcus equi infection after alemtuzumab therapy for T-cell prolymphocytic leukemia. (18258054)
2007
17
Short telomeres and high telomerase activity in T-cell prolymphocytic leukemia. (17898784)
2007
18
Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia. (16997373)
2007
19
Successful treatment of T-cell prolymphocytic leukemia with full-intensity conditioning followed by matched unrelated donor allogeneic stem cell transplantation. (18185486)
2007
20
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
21
Small cell variant type of T-prolymphocytic leukemia with a four-year indolent course preceding acute exacerbation. (16840216)
2006
22
T-cell prolymphocytic leukemia: an aggressive T cell malignancy with frequent cutaneous tropism. (16908353)
2006
23
Mismatched unrelated cord blood transplantation in a patient with T-cell prolymphocytic leukemia. (15690068)
2005
24
Allogeneic peripheral stem-cell transplantation with reduced-intensity conditioning regimen in refractory primary B-cell prolymphocytic leukemia: a long-term follow-up. (15880130)
2005
25
Translocation (8;14)(q24;q32) as the sole cytogenetic abnormality in B-cell prolymphocytic leukemia. (15066324)
2004
26
Successful treatment of B-cell prolymphocytic leukemia with monoclonal anti-CD20 antibody. (15060751)
2004
27
High levels of chromosomal imbalances in typical and small-cell variants of T-cell prolymphocytic leukemia. (14580769)
2003
28
Level of CD 20-expression and efficacy of rituximab treatment in patients with resistant or relapsing B-cell prolymphocytic leukemia and B-cell chronic lymphocytic leukemia. (11908720)
2002
29
Campath-1H treatment of T-cell prolymphocytic leukemia in patients for whom at least one prior chemotherapy regimen has failed. (11773171)
2002
30
Allogeneic peripheral blood stem cell transplantation with reduced intensity conditioning in primary refractory prolymphocytic leukemia: graft-versus-leukemia effect without graft-versus-host disease. (11803359)
2001
31
Treatment of T-prolymphocytic leukemia with nonmyeloablative allogeneic stem cell transplantation. (11168523)
2001
32
Concurrent Hodgkin's disease (mixed cellularity type) and T-cell chronic lymphocytic leukemia/prolymphocytic leukemia. (11372737)
2001
33
Prolymphocytic leukemia or prolymphocytic transformation of mantle cell lymphoma. (11710698)
2001
34
Successful autologous stem cell transplantation in aggressive prolymphocytic leukemia. (10706770)
2000
35
Human T-lymphotropic virus type I provirus and T-cell prolymphocytic leukemia. (10830745)
2000
36
Transgenic mice expressing MTCP1: an animal model for T-cell prolymphocytic leukemia]. (10669814)
2000
37
T-cell prolymphocytic leukemia with a novel translocation (6;11)(q21;q23). (10347553)
1999
38
Crystal structure of p14TCL1, an oncogene product involved in T-cell prolymphocytic leukemia, reveals a novel beta-barrel topology. (9519406)
1998
39
14q11 abnormality and trisomy 8q are not common in Japanese T-cell prolymphocytic leukemia. (9846013)
1998
40
Transgenic mice for MTCP1 develop T-cell prolymphocytic leukemia. (9657733)
1998
41
5' region and exon 7 mutations of the TP53 gene in two cases of B-cell prolymphocytic leukemia. (9844609)
1998
42
Treatment of T-cell prolymphocytic leukemia with human CD52 antibody. (9215839)
1997
43
The human T-cell lymphotropic viruses types I/II are not involved in T prolymphocytic leukemia and large granular lymphocytic leukemia. (9264385)
1997
44
Perforin-positive leukemic cell infiltration in the aortic tissue of a patient with T-cell prolymphocytic leukemia. (8971584)
1996
45
A gene on chromosome Xq28 associated with T-cell prolymphocytic leukemia in two patients with ataxia telangiectasia. (8152252)
1994
46
Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia. (1913594)
1991
47
Further characterization of prolymphocytic leukemia cells as a tumor of activated B cells. (2194392)
1990
48
Recurrent chromosome changes at 3p21 in Epstein-Barr virus-transformed cells derived from human prolymphocytic leukemia. (3019524)
1986
49
Prolymphocytic leukemia of B cell type: rearranged immunoglobulin (Ig) genes with defective Ig production. (2990609)
1985
50
Alkaline phosphatase and tartrate resistant acid phosphatase activity in cells of prolymphocytic leukemia. (642404)
1978

Genetic Variations for T-Cell Prolymphocytic Leukemia

Expression for genes affiliated with T-Cell Prolymphocytic Leukemia

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
60Tocris Bioscience, 38NCBI BioSystems Database, 54Reactome, 12EMD Millipore, 52QIAGEN, 30KEGG, 50PharmGKB
See all sources

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

(show all 40)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.2ATM
210.0CHEK2, TP53
3
Hide members
10.0ATM, CHEK2
4
DNA damage Role of Brca1 and Brca2 in DNA repair
Hide members
9.8ATM, CHEK2, TP53
5
Hide members
9.8ATM, CHEK2, TP53
69.8ATM, CHEK2, TP53
79.8TP53, CHEK2, ATM
89.8TP53, CHEK2, ATM
9
Cell cycle Role of 14-3-3 proteins in cell cycle regulation
Hide members
9.8TP53, CHEK2, ATM
109.8ATM, CDKN1B, TP53
11
Hide members
9.8TP53, CDKN1B, ATM
12
Hide members
9.8CHEK2, NFKB1, TP53
139.7NFKB1, ATM, ZAP70
14
Transcription P53 signaling pathway
Hide members
9.7TP53, NFKB1, ATM
15
G-protein signaling Ras family GTPases in kinase cascades (scheme)
Hide members
9.7NFKB1, CDKN1B, TP53
16
Hide members
9.7TP53, CDKN1B, NFKB1
17
Hide members
9.7TP53, CDKN1B, NFKB1
18
Hide members
9.7TP53, CDKN1B, NFKB1
199.7TP53, CDKN1B, NFKB1
209.7CDKN1B, NFKB1, ATM
21
Immune response NFAT in immune response
Hide members
9.7IGKC, NFKB1, ZAP70
22
Hide members
9.5ATM, CHEK2, CDKN1B, TP53
23
Hide members
9.5ATM, CHEK2, CDKN1B, TP53
249.5TP53, SMARCB1, CHEK2, ATM
259.5ATM, CHEK2, NFKB1, TP53
269.5ATM, CHEK2, NFKB1, TP53
279.5ATM, CHEK2, NFKB1, TP53
28
Hide members
9.5ZAP70, NFKB1, IGKC, CD19
29
Hide members
9.5ATM, NFKB1, CDKN1B, TP53
30
Hide members
9.5ATM, NFKB1, CDKN1B, TP53
319.5TP53, CDKN1B, NFKB1, ATM
32
Hide members
9.4NFKB1, IGKC, CD19, CDKN1B
33
Hide members
9.4NFKB1, IGKC, CD19, CDKN1B
349.4NFKB1, CD19, CD38, CDKN1B, TP53
35
Hide members
9.3TP53, NFKB1, CHEK2, ATM, ZAP70
369.3TP53, CDKN1B, NFKB1, CHEK2, ATM
37
Hide members
9.1TP53, CDKN1B, NFKB1, CHEK2, ATM, ZAP70
389.0MS4A1, CD7, CD38, CD19, CD5, ANPEP
398.9ATM, NFKB1, ETV6, CDKN1B, TP53
408.8MTCP1, NFKB1, CD19, CDKN1B, TCL1A, TP53

Compounds for genes affiliated with T-Cell Prolymphocytic Leukemia

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 60Tocris Bioscience, 29IUPHAR, 24HMDB
See all sources

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

(show all 23)
idCompoundScoreTop Affiliating Genes
1neocarzinostatin4510.3ATM, CHEK2
2ricin4510.1CD5, CD19
3bpde4510.1TP53, CHEK2, ATM
4gemcitabine45 50 1111.9TP53, CDKN1B, CHEK2
5hydroxyurea45 50 1111.9ATM, CHEK2, TP53
6aphidicolin459.9ATM, CHEK2, CDKN1B, TP53
7camptothecin45 60 1111.8TP53, CDKN1B, CHEK2, ATM
8n acetylcysteine459.8ATM, CHEK2, CDKN1B, TP53
9rituximab45 50 1111.7ZAP70, CD38, CD52, MS4A1
10cytarabine45 50 1111.7TP53, CDKN1B, ATM
11cyclophosphamide45 50 1111.6HLA-S, ZAP70, CDKN1B, MS4A1, TP53
12etoposide45 50 60 1112.6ATM, CHEK2, NFKB1, CDKN1B, TP53
13phosphatidylinositol459.4ZAP70, ATM, CHEK2, CD19, CD38, CDKN1B
14paraffin459.4ATM, ANPEP, CD5, CDKN1B, MS4A1, TP53
15doxorubicin45 50 1111.4ATM, CHEK2, ANPEP, CD19, CDKN1B, TP53
16serine459.4TCL1A, CDKN1B, ANPEP, CHEK2
17oligonucleotide459.3TP53, CDKN1B, ANPEP, CHEK2, ZAP70
18fludarabine45 1110.3TP53, MS4A1, CD52, CDKN1B, CD38, ZAP70
19atp45 2910.2ZAP70, ATM, CHEK2, ANPEP, CD38, CDKN1B
20wortmannin459.1ATM, CHEK2, ANPEP, CDKN1B
21threonine459.1ZAP70, ATM, CHEK2, ANPEP, CDKN1B, TCL1A
22retinoic acid45 249.9CHEK2, ANPEP, ETV6, CD38, CDKN1B, TP53
23tyrosine458.4ZAP70, ATM, CHEK2, ANPEP, ETV6, CD5

GO Terms for genes affiliated with T-Cell Prolymphocytic Leukemia

Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:0098979.3MS4A1, CD19, CD5, ANPEP
2nucleoplasmGO:0056549.0CHEK2, NFKB1, CDKN1B, SMARCB1, TP53, ATM

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

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1DNA damage induced protein phosphorylationGO:00697510.1CHEK2, ATM
2replicative senescenceGO:09039910.0TP53, CHEK2, ATM
3DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrestGO:0069779.8ATM, CDKN1B, TP53
4double-strand break repairGO:0063029.7TP53, CHEK2, ATM, BRCC3
5mitotic cell cycle arrestGO:0718509.7CDKN1B, TP53
6Fc-epsilon receptor signaling pathwayGO:0380959.6NFKB1, IGKC, CD19, CDKN1B
7immune responseGO:0069559.3MS4A1, CD7, IGKC, ZAP70
8negative regulation of apoptotic processGO:0430669.2TP53, CDKN1B, CD38, NFKB1, ATM

Products for genes affiliated with T-Cell Prolymphocytic Leukemia

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for T-Cell Prolymphocytic Leukemia

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet