MCID: CTN015
MIFTS: 45

Cutaneous T Cell Lymphoma malady

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

Aliases & Classifications for Cutaneous T Cell Lymphoma

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Aliases & Descriptions for Cutaneous T Cell Lymphoma:

Name: Cutaneous T Cell Lymphoma 8 10
Primary Cutaneous T-Cell Lymphoma 42
Lymphoma, T-Cell, Cutaneous 61
 
Cutaneous T-Cell Lymphoma 42
Lymphoma T-Cell Cutaneous 44
Ctcl 42


Classifications:



External Ids:

Disease Ontology8 DOID:0060061

Summaries for Cutaneous T Cell Lymphoma

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NIH Rare Diseases:42 Cutaneous t-cell lymphomas (ctcls) are a group of disorders characterized by an abnormal accumulation of cancerous t-cells (a type of white blood cells) in the skin resulting in an itchy, red rash that can thicken or form a tumor. ctcls belong to a larger group of disorders known asnon-hodgkin's lymphomas. the most common types are mycosis fungoides and sézary syndrome. in some cases, cancerous t-cells may spread to the lymph nodes and eventually to other body tissues and organs, potentially resulting in life-threatening complications. the specific signs and symptoms vary from person to person. the exact cause of these conditions is unknown. last updated: 9/27/2011

MalaCards based summary: Cutaneous T Cell Lymphoma, also known as primary cutaneous t-cell lymphoma, is related to mycosis fungoides and chronic lymphocytic leukemia. An important gene associated with Cutaneous T Cell Lymphoma is IFNA2 (interferon, alpha 2), and among its related pathways are Immunoregulatory interactions between a Lymphoid and a non-Lymphoid cell and Hematopoietic cell lineage. The drugs bexarotene and denileukin and the compounds mizoribine and pentostatin have been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and lymph node.

Disease Ontology:8 A non-hodgkin's lymphoma that is caused by a mutation of t cells.

Related Diseases for Cutaneous T Cell Lymphoma

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Diseases in the Cutaneous T Cell Lymphoma family:

Primary Cutaneous Gamma/delta-Positive T-Cell Lymphoma

Diseases related to Cutaneous T Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 117)
idRelated DiseaseScoreTop Affiliating Genes
1mycosis fungoides30.9PLS3, IL7, IL2RA, IFNA2, CCL17
2chronic lymphocytic leukemia30.6IL2RA, IL7
3bullous pemphigoid30.5CCL17, IL7
4hodgkin lymphoma30.5CCL17, IL2RA, IL7
5sezary's disease30.4IFNA2, IL2RA, IL7, PLS3
6hepatitis c30.4IFNA2, IL7, KIR2DL3
7ulcerative colitis30.2CCL17, IL2RA, IL7
8thrombocytopenia30.2IL7, IFNA2
9psoriasis30.2CCL17, IL2RA, IL7, KIR2DL3
10rheumatoid arthritis29.8KIR2DL1, IL7, IL2RA, ANG
11melanoma29.7KIR2DL1, IL7, IFNA2, ANG
12dermatitis10.8
13lymphomatoid papulosis10.7
14leukemia10.6
15atopic dermatitis10.5
16parapsoriasis10.5
17t-cell leukemia10.5
18pityriasis lichenoides10.5
19adult t-cell leukemia10.5
20hydroa vacciniforme10.5
21follicular mucinosis10.4
22pneumonia10.4
23chronic graft versus host disease10.4
24pityriasis lichenoides chronica10.4
25chronic actinic dermatitis10.4
26b-cell lymphomas10.3
27hematopoietic stem cell transplantation10.3
28peripheral t-cell lymphoma10.3
29pyoderma gangrenosum10.3
30capillary leak syndrome10.3
31dermatitis herpetiformis10.3
32fasciitis10.3
33pyoderma10.3
34vasculitis10.3
35xanthomatosis10.3
36eosinophilic fasciitis10.3
37systemic mastocytosis10.3IFNA2, IL2RA
38splenomegaly10.3IL2RA, IFNA2
39adrenal hyperplasia, congenital, due to 11-beta-hydroxylase deficiency10.3IL2RA, IFNA2
40mastocytosis10.3IL2RA, IFNA2
41hcl-v10.3IFNA2, IL2RA
42hepatitis b10.2IL2RA, IFNA2
43uveitis10.2IL2RA, IFNA2
44severe combined immunodeficiency, x-linked10.2IL7, IL2RA
45wolf-hirschhorn syndrome10.2
46aspergillosis10.2
47epidermodysplasia verruciformis10.2
48lung cancer10.2
49alopecia universalis10.2
50atopy10.2

Graphical network of the top 20 diseases related to Cutaneous T Cell Lymphoma:



Diseases related to cutaneous t cell lymphoma

Symptoms for Cutaneous T Cell Lymphoma

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Drugs & Therapeutics for Cutaneous T Cell Lymphoma

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FDA approved drugs:

id Drug Name Active Ingredient(s)13 Pharmaceutical Company Approval Date
1
Istodax13 38 ROMIDEPSIN Gloucester Pharmaceuticals Approved November 2009
FDA Label: Istodax
Malady that Drug Treats: cutaneous T-cell lymphoma
Indications and Usage:13 ISTODAX is a histone deacetylase (HDAC) inhibitor indicated for:; Treatment of cutaneous T-cell lymphoma (CTCL) in patients who have; received at least one prior systemic therapy (1).; Treatment of peripheral T-cell lymphoma (PTCL) in patients who have; received at least one prior therapy (1).; These indications are based on response rate. Clinical benefit such as; improvement in overall survival has not been demonstrated (1).
DrugBank Targets:11 -
Mechanism of Action:13 
Target: histone deacetylase (HDAC)
Action: inhibitor
FDA: Romidepsin is a histone deacetylase (HDAC) inhibitor. HDACs catalyze the removal of acetyl groups from acetylated lysine residues in histones, resulting in the; modulation of gene expression. HDACs also deacetylate non-histone proteins, such as transcription factors. In vitro, romidepsin causes the accumulation of acetylated; histones, and induces cell cycle arrest and apoptosis of some cancer cell lines with IC50 values in the nanomolar range. The mechanism of the antineoplastic effect of; romidepsin observed in nonclinical and clinical studies has not been fully characterized.
2
UVADEX13 METHOXSALEN Therakos Approved February 1999
FDA Label: UVADEX
Malady that Drug Treats: skin manifestations of cutaneous T-cell lymphoma (CTCL)
Indications and Usage:13 UVADEX"! (methoxsalen) Sterile Solution is indicated for extracorporeal administration with; the UVAR"! XTS"! or THERAKOS"! CELLEX"! Photopheresis System in the palliative; treatment of the skin manifestations of Cutaneous T-Cell Lymphoma (CTCL) that is; unresponsive to other forms of treatment. ;
DrugBank Targets:11 1. DNA
Mechanism of Action:13 
Target: DNA
Action: inhibitor of DNA synthesis, cell division, and epidermal turnover
FDA: The exact mechanism of action of methoxsalen is not known. The best-known biochemical; reaction of methoxsalen is with DNA. Methoxsalen, upon photoactivation, conjugates and forms; covalent bonds with DNA which leads to the formation of both monofunctional (addition to a; single strand of DNA) and bifunctional adducts (crosslinking of psoralen to both strands of; DNA). Reactions with proteins have also been described. The formation of photoadducts results; in inhibition of DNA synthesis, cell division and epidermal turnover.; For the palliative treatment of Cutaneous T-Cell Lymphoma, Photopheresis consists of removing; a portion of the patient's blood and separating the red blood cells from the white cell layer (buffy; coat) by centrifugation. The red cells are returned to the patient and the UVADEX"! Sterile; Solution is then injected into the instrument and mixed with the buffy coat. The instrument then; irradiates this drug-cell mixture with ultraviolet light (UVA light, 320-400 nm) and returns the; treated cells to the patient. See the appropriate Operator's Manual for details of this process.; Although extracorporeal phototherapy exposes less than 10% of the total body burden of; malignant cells to methoxsalen plus light, some patients achieve a complete response. Animal; studies suggest that the photopheresis may activate an immune-mediated response against the; malignant T-cells.; Use of the UVAR"! and UVAR"! XTS"! Systems after oral administration of methoxsalen; were previously approved for the treatment of Cutaneous T-Cell Lymphoma. Interpatient; variability in peak plasma concentration after an oral dose of methoxsalen ranges from 6 to 15; fold. UVADEX"! is injected directly into the separated buffy coat in the instrument in an; attempt to diminish this interpatient variability and to improve the exposure of the cells to the; drug.; Methoxsalen is reversibly bound to serum albumin and is also preferentially taken up by; epidermal cells. Methoxsalen is rapidly metabolized in humans, with approximately 95% of the; drug excreted as metabolites in the urine within 24 hours.; Systemic administration of methoxsalen followed by UVA exposure leads to cell injury. The; most obvious manifestation of this injury after skin exposure is delayed erythema, which may; not begin for several hours and peaks at 48-72 hours. The inflammation is followed over several; days to weeks, by repair which is manifested by increased melanization of the epidermis and; thickening of the stratum corneum.; The total dose of methoxsalen delivered in UVADEX"! is substantially lower (approximately; 200 times) than that used with oral administration. More than 80% of blood samples collected; 30 minutes after reinfusion of the photoactivated buffy coat had methoxsalen levels below; detection limits of the assay (<10 ng/ml), and the mean plasma methoxsalen concentration was; approximately 25 ng/ml.
3
Valchlor13 MECHLORETHAMINE HYDROCHLORIDE Ceptaris Therapeutics Approved August 2013
FDA Label: Valchlor
Malady that Drug Treats: Stage IA/IB mycosisfungoides-type cutaneous T-cell lymphoma
Indications and Usage:13 VALCHLOR is an alkylating drug indicated for the topical treatment; of Stage IA and IB mycosis fungoides type cutaneous T cell; lymphoma in patients who have received prior skin directed therapy; (1).  
DrugBank Targets:11 1. DNA
Mechanism of Action:13 
Target: rapidly-proliferating cells
Action: inhibitor
FDA: Mechlorethamine, also known as nitrogen mustard, is an alkylating agent which inhibits rapidly proliferating cells.

Drug clinical trials:

Search ClinicalTrials for Cutaneous T Cell Lymphoma

Search NIH Clinical Center for Cutaneous T Cell Lymphoma

Inferred drug relations via UMLS61/NDF-RT40:

Genetic Tests for Cutaneous T Cell Lymphoma

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Anatomical Context for Cutaneous T Cell Lymphoma

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MalaCards organs/tissues related to Cutaneous T Cell Lymphoma:

31
T cells, Skin, Lymph node, B cells, Bone, Neutrophil, Bone marrow, Testis, Monocytes, Heart, Colon, Liver, Lung, Testes, Nk cells, Endothelial

Animal Models for Cutaneous T Cell Lymphoma or affiliated genes

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Publications for Cutaneous T Cell Lymphoma

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Articles related to Cutaneous T Cell Lymphoma:

(show top 50)    (show all 740)
idTitleAuthorsYear
1
Romidepsin in peripheral and cutaneous T-cell lymphoma: mechanistic implications from clinical and correlative data. (25891346)
2015
2
Resimmune-an anti-CD3I/ recombinant immunotoxin induces durable remissions in cutaneous T cell lymphoma patients. (25795722)
2015
3
Cutaneous T-cell lymphoma with nasal necrosis. (25798408)
2015
4
Phase II evaluation of VDC-1101 in canine cutaneous T-cell lymphoma. (25274443)
2014
5
Novel therapies for cutaneous T-cell lymphoma: what does the future hold? (24397291)
2014
6
Romidepsin for cutaneous T-cell lymphoma. (24295412)
2013
7
Serum visfatin levels in patients with atopic dermatitis and cutaneous T-cell lymphoma. (24001451)
2013
8
Expression of miR-155 and miR-126 in situ in cutaneous T-cell lymphoma. (24033365)
2013
9
Canine inflamed nonepitheliotropic cutaneous T-cell lymphoma: a diagnostic conundrum. (23331699)
2013
10
Association of nerve growth factor, chemokine (C-C motif) ligands and immunoglobulin E with pruritus in cutaneous T-cell lymphoma. (22948508)
2013
11
Comparison of pegylated interferon I+-2b plus psoralen PUVA versus standard interferon I+-2a plus PUVA in patients with cutaneous T-cell lymphoma. (22168776)
2012
12
Romidepsin: a new drug for the treatment of cutaneous T-cell lymphoma. (22459529)
2012
13
Integrated positron-emission tomography and computed tomography manifestations of cutaneous T-cell lymphoma. (23247490)
2012
14
Low herpesvirus entry mediator (HVEM) expression on dermal fibroblasts contributes to a Th2-dominant microenvironment in advanced cutaneous T-cell lymphoma. (22297640)
2012
15
Management of cutaneous T cell lymphoma: new and emerging targets and treatment options. (22457602)
2012
16
Galectin-1 inhibits the viability, proliferation, and Th1 cytokine production of nonmalignant T cells in patients with leukemic cutaneous T-cell lymphoma. (22383798)
2012
17
Photopheresis in the treatment of cutaneous T-cell lymphoma: current status. (22157241)
2012
18
The histone deacetylase inhibitor, romidepsin, suppresses cellular immune functions of cutaneous T-cell lymphoma patients. (22367792)
2012
19
IL-22, but not IL-17, dominant environment in cutaneous T-cell lymphoma. (22048239)
2011
20
Romidepsin in the treatment of cutaneous T-cell lymphoma. (22287862)
2011
21
The role of cytokine signaling in the pathogenesis of cutaneous T-cell lymphoma. (22236880)
2011
22
Absence of TCR loci chromosomal translocations in cutaneous T-cell lymphomas. (21872828)
2011
23
Matrix metalloproteinase-2 promoter genotype as a marker of cutaneous T-cell lymphoma early stage. (20625418)
2010
24
Posttransplant cutaneous T-cell lymphoma: case reports and review of the association of calcineurin inhibitor use with posttransplant lymphoproliferative disease risk. (20479299)
2010
25
Phase I trial of a Toll-like receptor 9 agonist, PF-3512676 (CPG 7909), in patients with treatment-refractory, cutaneous T-cell lymphoma. (20888065)
2010
26
Primary cutaneous T-cell lymphoma following organ transplantation in a 16-year-old boy. (19250431)
2009
27
Primary cutaneous T-cell lymphoma of unspecified type with cytotoxic phenotype: clinicopathological analysis of 27 patients. (19018763)
2009
28
Extracorporeal methoxsalen: new drug. Cutaneous T cell lymphoma: for rare patients, but better assessment needed. (19388212)
2009
29
Inhibition of constitutively activated nuclear factor-kappaB induces reactive oxygen species- and iron-dependent cell death in cutaneous T-cell lymphoma. (19258503)
2009
30
SAczary syndrome is a unique cutaneous T-cell lymphoma as identified by an expanded gene signature including diagnostic marker molecules CDO1 and DNM3. (18033314)
2008
31
U.K. consensus statement on the use of extracorporeal photopheresis for treatment of cutaneous T-cell lymphoma and chronic graft-versus-host disease. (18241274)
2008
32
Inhibition of IkappaB kinase subunit 2 in cutaneous T-cell lymphoma down-regulates nuclear factor-kappaB constitutive activation, induces cell death, and potentiates the apoptotic response to antineoplastic chemotherapeutic agents. (18245554)
2008
33
T-cell receptor-gamma gene analysis in evolving to advancing cutaneous T-cell lymphoma. (17680965)
2007
34
Cutaneous T-cell lymphoma. (16720231)
2006
35
Treatment of cutaneous T-cell lymphoma with retinoids. (17014481)
2006
36
Future perspectives in the treatment of cutaneous T-cell lymphoma (CTCL). (16516674)
2006
37
Activation of neutrophils in cutaneous T-cell lymphoma. (16322281)
2005
38
CCR10 is expressed in cutaneous T-cell lymphoma. (15700309)
2005
39
Cutaneous T-cell lymphoma: malignant proliferation of T-regulatory cells. (15514008)
2005
40
Serological immunomarkers in cutaneous T cell lymphoma. (15539892)
2004
41
T cell receptor gamma-chain gene polymerase chain reaction to diagnose central nervous system involvement by cutaneous T cell lymphoma. (11986404)
2002
42
Interleukin-7 and interleukin-15 regulate the expression of the bcl-2 and c-myb genes in cutaneous T-cell lymphoma cells. (11675351)
2001
43
Functional inhibitory receptors expressed by a cutaneous T cell lymphoma-specific cytolytic clonal T cell population. (11121132)
2000
44
Activity of pentostatin (Nipent) in cutaneous T-cell lymphoma: single-agent and combination studies. (10877054)
2000
45
Constitutive and interleukin-7/interleukin-15 stimulated DNA binding of Myc, Jun, and novel Myc-like proteins in cutaneous T-cell lymphoma cells. (9864169)
1999
46
Immunophenotyping and gene rearrangement analysis provide additional criteria to differentiate between cutaneous T-cell lymphomas and pseudo-T-cell lymphomas. (9176388)
1997
47
A dominant inhibitory mutant of the type II transforming growth factor beta receptor in the malignant progression of a cutaneous T-cell lymphoma. (8668164)
1996
48
Interferon-inducible protein-10 and the pathogenesis of cutaneous T-cell lymphomas. (9049966)
1996
49
Chronic actinic dermatitis. An immunohistochemical study of its T-cell antigenic profile, with comparison to cutaneous T-cell lymphoma. (7802165)
1994
50
Interferon alpha and etretinate combination treatment of cutaneous T-cell lymphoma. (2258637)
1990

Variations for Cutaneous T Cell Lymphoma

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Cosmic variations for Cutaneous T Cell Lymphoma:

6
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Conf
1532KRAShaematopoietic and lymphoid tissue,skin,lymphoid neoplasm,cutaneous T cell lymphoma1

Expression for genes affiliated with Cutaneous T Cell Lymphoma

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Search GEO for disease gene expression data for Cutaneous T Cell Lymphoma.

Pathways for genes affiliated with Cutaneous T Cell Lymphoma

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Pathways related to Cutaneous T Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 12)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8KIR2DL1, KIR2DL3
29.6IL2RA, IL7
3
Show member pathways
9.6IL7, IL2RA
4
Show member pathways
Immune response CD16 signaling in NK cells59
9.4KIR2DL1, KIR2DL3, IFNA2
59.3IL7, IL2RA, IFNA2
6
Show member pathways
9.3IFNA2, IL2RA, IL7
7
Show member pathways
Type III interferon signaling36
9.3IL7, IL2RA, IFNA2
8
Show member pathways
9.0KIR2DL1, KIR2DL3, IL2RA, IFNA2
9
Show member pathways
8.8CCL17, IL2RA, IL7
10
Show member pathways
8.8CCL17, IL2RA, IL7
11
Show member pathways
8.5CCL17, IFNA2, IL2RA, IL7
12
Show member pathways
8.5CCL17, IFNA2, IL2RA, IL7

Compounds for genes affiliated with Cutaneous T Cell Lymphoma

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Compounds related to Cutaneous T Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 38)
idCompoundScoreTop Affiliating Genes
1mizoribine4410.2IFNA2, IL2RA
2pentostatin44 60 28 1113.2IFNA2, IL2RA
3hydroxychloroquine44 1111.1IL2RA, IFNA2
4megestrol acetate4410.1IL2RA, IFNA2
5levamisole44 1111.1IFNA2, IL2RA
6lamivudine44 50 1112.1IFNA2, IL2RA
7con a4410.1IFNA2, IL2RA
8diethyl dithiocarbamate4410.1IL2RA, IFNA2
92,5-oligoadenylate4410.1IFNA2, IL2RA
10infliximab44 50 1112.0IL2RA, IFNA2
11erythromycin44 1 50 1113.0IL2RA, IFNA2
123-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide4410.0IFNA2, IL7
13c-d4t4410.0IL7, IL2RA
14ifn-alpha4410.0IFNA2, IL2RA
15basiliximab44 1111.0IL7, IL2RA
16betacarotene4410.0IFNA2, IL2RA
17okt34410.0IL2RA, IL7
18neopterin4410.0IL2RA, IFNA2
19rituximab44 50 1111.9IFNA2, IL2RA
20bleomycin44 1110.9IL2RA, IFNA2
21thalidomide44 50 60 1112.8IL2RA, IFNA2
22vitamin-e449.7IFNA2, IL2RA
23vincristine44 50 1111.7IL2RA, IFNA2
24cyclophosphamide44 50 1111.7IL2RA, IFNA2, IL7
25azathioprine44 1 50 1112.6IL2RA, IFNA2
26histamine44 28 2411.6IFNA2, IL2RA, IL7
27doxorubicin44 50 1111.6IL7, IL2RA, IFNA2
28neomycin449.5ANG, IL7
29thymidine44 2410.5IL7, IL2RA, IFNA2
30ccl444 2810.5IL7, CCL17
31alpha tocopherol449.4IFNA2, IL2RA
32allergens449.4CCL17, IL7
33il 10449.2CCL17, IL7, IL2RA
34tacrolimus44 50 1111.2CCL17, IL7, IL2RA
35rantes449.2CCL17, IL2RA, IL7
36cyclosporin a44 28 6011.1IL2RA, IL7, CCL17
37vegf449.0IL2RA, IFNA2, IL7, ANG
38tyrosine448.4IFNA2, ANG, IL2RA, IL7, KIR2DL3, KIR2DL1

GO Terms for genes affiliated with Cutaneous T Cell Lymphoma

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Cellular components related to Cutaneous T Cell Lymphoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00056158.3IL7, IFNA2, CCL17, ANG

Biological processes related to Cutaneous T Cell Lymphoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1regulation of immune responseGO:00507769.8KIR2DL1, KIR2DL3
2positive regulation of T cell differentiationGO:00455829.5IL2RA, IL7
3cell-cell signalingGO:00072678.6IL7, IFNA2, CCL17
4immune responseGO:00069558.5KIR2DL1, KIR2DL3, IL2RA, CCL17

Sources for Cutaneous T Cell Lymphoma

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2CDC
12ExPASy
13FDA
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet