MCID: PRM301
MIFTS: 35

Primary Cutaneous T-Cell Lymphoma

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Primary Cutaneous T-Cell Lymphoma

MalaCards integrated aliases for Primary Cutaneous T-Cell Lymphoma:

Name: Primary Cutaneous T-Cell Lymphoma 59

Characteristics:

Orphanet epidemiological data:

59
primary cutaneous t-cell lymphoma
Prevalence: 1-9/100000 (Europe),1-5/10000 (Europe);

Classifications:



External Ids:

MESH via Orphanet 45 D016410
ICD10 via Orphanet 34 C84.8
UMLS via Orphanet 73 C0079773
Orphanet 59 ORPHA171901

Summaries for Primary Cutaneous T-Cell Lymphoma

MalaCards based summary : Primary Cutaneous T-Cell Lymphoma is related to sezary's disease and t-cell leukemia. An important gene associated with Primary Cutaneous T-Cell Lymphoma is IL2RA (Interleukin 2 Receptor Subunit Alpha), and among its related pathways/superpathways are NF-kappaB Signaling and Human T-cell leukemia virus 1 infection. The drug Bexarotene has been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and nk cells, and related phenotypes are Synthetic lethal with MLN4924 (a NAE inhibitor) and Synthetic lethal with MLN4924 (a NAE inhibitor)

Related Diseases for Primary Cutaneous T-Cell Lymphoma

Diseases in the Cutaneous T Cell Lymphoma family:

Primary Cutaneous B-Cell Lymphoma Primary Cutaneous Gamma/delta-Positive T-Cell Lymphoma
Primary Cutaneous T-Cell Lymphoma

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

(show top 50) (show all 62)
# Related Disease Score Top Affiliating Genes
1 sezary's disease 30.1 TNFRSF8 PLS3 IL2RA
2 t-cell leukemia 29.0 TERT JUN IL2RA
3 lymphoma, non-hodgkin, familial 28.4 TNFRSF8 TFRC MS4A1 IL2RA
4 aggressive primary cutaneous t-cell lymphoma 12.8
5 indolent primary cutaneous t-cell lymphoma 12.6
6 mycosis fungoides 11.9
7 cutaneous t cell lymphoma 10.9
8 lymphoma 10.9
9 anaplastic large cell lymphoma 10.5
10 t-cell lymphoma, subcutaneous panniculitis-like 10.4
11 lymphomatoid papulosis 10.4
12 peripheral t-cell lymphoma 10.3
13 panniculitis 10.3
14 b-cell lymphoma 10.3
15 primary cutaneous lymphoma 10.3
16 lymphoproliferative syndrome 10.2
17 skin disease 10.2
18 primary cutaneous anaplastic large cell lymphoma 10.2
19 hematopoietic stem cell transplantation 10.2
20 dermatitis, atopic 10.1
21 parapsoriasis 10.1
22 hypereosinophilic syndrome 10.1
23 leukemia, t-cell, chronic 10.1
24 b cell prolymphocytic leukemia 10.1 MS4A1 IL2RA
25 marek disease 10.0 TNFRSF8 JUN
26 pityriasis lichenoides 10.0 TNFRSF8 MS4A1
27 hypercholesterolemia, familial, 1 10.0
28 hypertriglyceridemia, familial 10.0
29 ichthyosis vulgaris 10.0
30 small cell cancer of the lung 10.0
31 lymphoma, hodgkin, classic 10.0
32 leukemia, acute myeloid 10.0
33 autoimmune lymphoproliferative syndrome 10.0
34 graft-versus-host disease 10.0
35 hypophosphatemia 10.0
36 adult t-cell leukemia 10.0
37 diffuse large b-cell lymphoma 10.0
38 angioimmunoblastic t-cell lymphoma 10.0
39 capillary leak syndrome 10.0
40 ichthyosis 10.0
41 hyperuricemia 10.0
42 dermatitis 10.0
43 mastocytosis 10.0
44 hypokalemia 10.0
45 peripheral nervous system disease 10.0
46 lymphopenia 10.0
47 neuropathy 10.0
48 lichen planus 10.0
49 pulmonary embolism 10.0
50 hypoglycemia 10.0

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



Diseases related to Primary Cutaneous T-Cell Lymphoma

Symptoms & Phenotypes for Primary Cutaneous T-Cell Lymphoma

GenomeRNAi Phenotypes related to Primary Cutaneous T-Cell Lymphoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.62 IL2RA JUN JUNB PDCD1 TERT
2 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.62 IL2RA JUN JUNB PDCD1 TERT

MGI Mouse Phenotypes related to Primary Cutaneous T-Cell Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.81 IL2RA JUN JUNB MS4A1 PDCD1 PLS3
2 homeostasis/metabolism MP:0005376 9.5 IL2RA JUN JUNB PDCD1 PLS3 TERT
3 immune system MP:0005387 9.28 IL2RA JUN JUNB MS4A1 PDCD1 PLS3

Drugs & Therapeutics for Primary Cutaneous T-Cell Lymphoma

Drugs for Primary Cutaneous T-Cell Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bexarotene Approved, Investigational Phase 2 153559-49-0 82146

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase II Prospective Non-Randomized Clinical Trial of Dose-Adjusted Schedule of Vorinostat in Patients With Primary Cutaneous T-Cell Lymphoma Who Did Not Receive Prior Systemic Therapy or Have Been Treated With Single Agent Targretin Terminated NCT00958074 Phase 2 vorinostat
2 A Phase II, Multi-center, Open-label, Randomized Study of Vorinostat Plus Lenalidomide and Dexamethasone or Lenalidomide Plus Dexamethasone in Multiple Myeloma Patients Who Experience Biochemical Relapse During Lenalidomide Maintenance Therapy Withdrawn NCT01501370 Phase 2 Vorinostat;Lenalidomide;Dexamethasone;Lenalidomide;Dexamethasone
3 Detection of Somatic Mutations on Cell-free Circulating DNA in Potentially Aggressive Cutaneous Lymphomas Unknown status NCT02883517

Search NIH Clinical Center for Primary Cutaneous T-Cell Lymphoma

Genetic Tests for Primary Cutaneous T-Cell Lymphoma

Anatomical Context for Primary Cutaneous T-Cell Lymphoma

MalaCards organs/tissues related to Primary Cutaneous T-Cell Lymphoma:

41
T Cells, Skin, Nk Cells, B Cells, Bone, Bone Marrow, Thymus

Publications for Primary Cutaneous T-Cell Lymphoma

Articles related to Primary Cutaneous T-Cell Lymphoma:

(show top 50) (show all 204)
# Title Authors PMID Year
1
IPH4102, a first-in-class anti-KIR3DL2 monoclonal antibody, in patients with relapsed or refractory cutaneous T-cell lymphoma: an international, first-in-human, open-label, phase 1 trial. 38
31253572 2019
2
Effective remission of chidamide on treatment of advanced mycosis fungoides: An unusual case report. 38
31012254 2019
3
Frequent and persistent PLCG1 mutations in Sézary cells directly enhance PLCγ1 activity and stimulate NFκB, AP-1 and NFAT signaling. Short title: Gain-of-function PLCG1 mutations in Sézary Syndrome. 38
31376383 2019
4
Two cases of phenotypic switch of primary cutaneous T cell lymphoma after treatment with an aggressive course and review of the literature. 38
31218405 2019
5
Sézary Syndrome, recent biomarkers and new drugs. 38
30525758 2019
6
Sézary Syndrome Presenting With Renal Involvement. 38
29941220 2018
7
Interferon alfa-2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement. 38
30565216 2018
8
Molecular pathogenesis of cutaneous lymphomas. 38
29862563 2018
9
Methotrexate in the treatment of mycosis fungoides - a multicenter observational study in 79 patients. 38
29917213 2018
10
Allogeneic hematopoietic stem cell transplantation in Primary Cutaneous T Cell Lymphoma. 38
29442161 2018
11
Total Skin Electron Beam Therapy with Rotary Dual Technique as Palliative Treatment for Mycosis Fungoides. 38
29695554 2018
12
Synchronous Occurrence of Mycosis Fungoides, Diffuse Large B Cell Lymphoma and Acute Myeloid Leukemia. 38
29321428 2018
13
Survival outcomes of primary cutaneous T-cell lymphoma in HIV-infected patients: a national population-based study. 38
29330308 2018
14
Nodal Involvement by CD30+ Cutaneous Lymphoproliferative Disorders and Its Challenging Differentiation From Classical Hodgkin Lymphoma. 38
29257929 2018
15
Second primary cutaneous T-cell lymphoma in patients with non-Hodgkin lymphoma: a chicken-or-the-egg issue. 38
29047113 2018
16
Eruption of lymphocyte recovery with atypical lymphocytes mimicking a primary cutaneous T-cell lymphoma: a series of 12 patients. 38
29107664 2018
17
Histone deacetylase inhibitors downregulate CCR4 expression and decrease mogamulizumab efficacy in CCR4-positive mature T-cell lymphomas. 38
29025909 2018
18
Low-dose total skin electron beam therapy for cutaneous lymphoma : Minimal risk of acute toxicities. 38
28785772 2017
19
A rare localised nasal CD30+ primary cutaneous T-cell lymphoma following liver transplantation. 38
29103006 2017
20
Immunohistochemistry reveals an increased proportion of MYC-positive cells in subcutaneous panniculitis-like T-cell lymphoma compared with lupus panniculitis. 38
28800143 2017
21
Brentuximab vedotin therapy for CD30-positive cutaneous T-cell lymphoma: a targeted approach to management. 38
28805086 2017
22
Immunohistopathological Study of c-FLIP Protein in Mycosis Fungoides 38
28952283 2017
23
Primary cutaneous T-cell lymphoma: experience from the Peruvian National Cancer Institute. 38
29166501 2017
24
[Role of stem cell transplantation in treatment of primary cutaneous T‑cell lymphoma]. 38
28785925 2017
25
Mycosis Fungoides: Experience in a Pediatric Hospital. 38
28279399 2017
26
Clinicopathologic Variants of Mycosis Fungoides. 38
27871620 2017
27
CD4-Positive T-Cell Large Granular Lymphocytosis Mimicking Sezary Syndrome in a Patient With Mycosis Fungoides. 38
28441377 2017
28
Primary Cutaneous T-cell Lymphoma with Aberrant Expression of CD20. 38
27840885 2017
29
Management Strategies for Mycosis Fungoides in India. 38
28400632 2017
30
Differential NFATc1 Expression in Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoma and Other Forms of Cutaneous T-Cell Lymphoma and Pseudolymphoma. 38
27097342 2017
31
Expression of SOX18 in Mycosis Fungoides. 38
27230943 2017
32
Total Skin Electron Beam Therapy as Part of Multimodal Treatment Strategies for Primary Cutaneous T-Cell Lymphoma. 38
28448985 2017
33
Fatal Case of Primary Cutaneous Aggressive T-Cell Lymphoma Switching From a CD4+ to a CD8+ Phenotype: Progressive Disease With Bexarotene and Romidepsin Treatment. 38
27322928 2016
34
Primary Cutaneous T Cell Lymphoma (Gamma Delta subtype). 38
28064300 2016
35
Mycosis fungoides patient accompanied actinic keratosis, actinic keratosis with squamous cell carcinoma transformation, and porokeratosis after NBUVB therapy - 1st case report and review of the literature. 38
27741114 2016
36
Genomic imbalances and microRNA transcriptional profiles in patients with mycosis fungoides. 38
27473081 2016
37
CD8-positive Mycosis Fungoides Masquerading as Pyoderma Gangrenosum. 38
27688458 2016
38
Expression of CD31 in Mycosis Fungoides. 38
27630298 2016
39
Alemtuzumab in refractory Sézary syndrome. 38
27828640 2016
40
MicroRNA-16 mediates the regulation of a senescence-apoptosis switch in cutaneous T-cell and other non-Hodgkin lymphomas. 38
26640145 2016
41
How I treat mycosis fungoides and Sézary syndrome. 38
27151889 2016
42
Mycosis Fungoides: A Retrospective Study of 44 Swedish Cases. 38
26778803 2016
43
Independent Loss of Methylthioadenosine Phosphorylase (MTAP) in Primary Cutaneous T-Cell Lymphoma. 38
26872600 2016
44
Expression of CD164 on Malignant T cells in Sézary Syndrome. 38
26524186 2016
45
HPyV6, HPyV7 and TSPyV DNA sequences detection in skin disease patients and healthy subjects. 38
25809796 2016
46
Multiple cutaneous lymphoproliferative disorders showing a retained tumor clone by T-cell receptor gene rearrangement analysis: a case series of four patients and review of the literature. 38
26266670 2016
47
Primary cutaneous follicular helper T-cell lymphoma. 38
26282465 2016
48
Molecular genetic and cytogenetic analysis of a primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma. 38
26676804 2016
49
Primary Cutaneous T-cell Lymphoma With Coexpression of T-Cell Receptors αβ and γδ. 38
26258878 2016
50
Cutaneous CD8+ Cytotoxic T-Cell Lymphoma Infiltrates: Clinicopathological Correlation and Outcome of 35 Cases. 38
28261650 2016

Variations for Primary Cutaneous T-Cell Lymphoma

Expression for Primary Cutaneous T-Cell Lymphoma

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

Pathways for Primary Cutaneous T-Cell Lymphoma

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

(show all 18)
# Super pathways Score Top Affiliating Genes
1 12.22 TNFRSF8 PDCD1 JUNB IL2RA
2 12.04 TERT JUN IL2RA
3 11.76 TERT JUNB JUN
4 11.6 TFRC MS4A1 IL2RA
5
Show member pathways
11.43 JUNB JUN IL2RA
6 11.34 TNFRSF8 PDCD1
7 11.31 JUNB JUN
8 11.31 TFRC TERT JUN
9
Show member pathways
11.28 TERT JUNB JUN IL2RA
10 11.27 JUNB JUN
11 11.23 JUNB JUN
12
Show member pathways
11.22 JUNB JUN IL2RA
13 11.18 JUNB JUN
14 11.06 TNFRSF8 PDCD1 MS4A1
15 11.04 JUNB JUN
16
Show member pathways
10.96 JUN IL2RA
17 10.92 JUN IL2RA
18
Show member pathways
10.25 TERT JUNB JUN IL2RA

GO Terms for Primary Cutaneous T-Cell Lymphoma

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

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 9.26 TFRC PDCD1 MS4A1 IL2RA
2 transcription factor AP-1 complex GO:0035976 8.62 JUNB JUN

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of apoptotic process GO:0043065 9.67 TNFRSF8 PDCD1 JUN
2 positive regulation of T cell proliferation GO:0042102 9.51 TFRC IL2RA
3 response to cytokine GO:0034097 9.48 JUNB JUN
4 humoral immune response GO:0006959 9.46 PDCD1 MS4A1
5 response to cAMP GO:0051591 9.43 JUNB JUN
6 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.4 TERT JUN
7 cellular response to hormone stimulus GO:0032870 9.37 JUNB JUN
8 positive regulation of cell differentiation GO:0045597 9.32 JUNB JUN
9 response to radiation GO:0009314 9.26 JUNB JUN
10 osteoclast differentiation GO:0030316 9.16 TFRC JUNB
11 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 8.96 TERT JUN
12 cellular process GO:0009987 8.62 JUNB JUN

Sources for Primary Cutaneous T-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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