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 58

Characteristics:

Orphanet epidemiological data:

58
primary cutaneous t-cell lymphoma
Prevalence: 1-9/100000 (Europe),1-5/10000 (Europe);

Classifications:

Orphanet: 58  
Rare skin diseases
Rare haematological diseases


External Ids:

MESH via Orphanet 44 D016410
ICD10 via Orphanet 33 C84.8
UMLS via Orphanet 72 C0079773
Orphanet 58 ORPHA171901

Summaries for Primary Cutaneous T-Cell Lymphoma

MalaCards based summary : Primary Cutaneous T-Cell Lymphoma is related to mycosis fungoides and lymphoma. An important gene associated with Primary Cutaneous T-Cell Lymphoma is JUNB (JunB Proto-Oncogene, AP-1 Transcription Factor Subunit), 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 b 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 71)
# Related Disease Score Top Affiliating Genes
1 mycosis fungoides 32.3 TNFRSF8 PLS3 IL2RA
2 lymphoma 30.6 TNFRSF8 PDCD1 MS4A1 IL2RA
3 sezary's disease 30.2 TNFRSF8 PLS3 IL2RA
4 anaplastic large cell lymphoma 30.2 TNFRSF8 JUNB JUN IL2RA
5 peripheral t-cell lymphoma 30.0 TNFRSF8 PDCD1 MS4A1
6 adult t-cell leukemia 29.7 TNFRSF8 JUN IL2RA
7 angioimmunoblastic t-cell lymphoma 29.6 TNFRSF8 PDCD1 IL2RA
8 t-cell leukemia 29.4 TERT JUNB JUN IL2RA
9 lymphoma, hodgkin, classic 29.3 TNFRSF8 MS4A1 JUNB IL2RA
10 lymphoma, non-hodgkin, familial 28.4 TNFRSF8 TFRC MS4A1 JUNB IL2RA
11 leukemia, acute myeloid 28.2 TNFRSF8 TFRC TERT JUN
12 aggressive primary cutaneous t-cell lymphoma 12.8
13 indolent primary cutaneous t-cell lymphoma 12.6
14 cutaneous t cell lymphoma 10.9
15 t-cell lymphoma, subcutaneous panniculitis-like 10.4
16 lymphomatoid papulosis 10.4
17 panniculitis 10.3
18 b-cell lymphoma 10.3
19 primary cutaneous lymphoma 10.3
20 lymphoproliferative syndrome 10.2
21 skin disease 10.2
22 primary cutaneous anaplastic large cell lymphoma 10.2
23 b cell prolymphocytic leukemia 10.2 MS4A1 IL2RA
24 prolymphocytic leukemia 10.2 MS4A1 IL2RA
25 dermatitis, atopic 10.1
26 parapsoriasis 10.1
27 hypereosinophilic syndrome 10.1
28 b-cell expansion with nfkb and t-cell anergy 10.1 MS4A1 IL2RA
29 marek disease 10.1 TNFRSF8 JUN
30 pityriasis lichenoides 10.1 TNFRSF8 MS4A1
31 t-cell adult acute lymphocytic leukemia 10.0 TNFRSF8 IL2RA
32 nodular lymphocyte predominant hodgkin lymphoma 10.0 TNFRSF8 PDCD1
33 hypercholesterolemia, familial, 1 10.0
34 hypertriglyceridemia, familial 10.0
35 ichthyosis vulgaris 10.0
36 small cell cancer of the lung 10.0
37 autoimmune lymphoproliferative syndrome 10.0
38 graft-versus-host disease 10.0
39 hypophosphatemia 10.0
40 diffuse large b-cell lymphoma 10.0
41 capillary leak syndrome 10.0
42 ichthyosis 10.0
43 hyperuricemia 10.0
44 dermatitis 10.0
45 mastocytosis 10.0
46 hypokalemia 10.0
47 peripheral nervous system disease 10.0
48 lymphopenia 10.0
49 neuropathy 10.0
50 lichen planus 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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.81 IL2RA JUN JUNB MS4A1 PDCD1 PLS3
2 immune system MP:0005387 9.61 IL2RA JUN JUNB MS4A1 PDCD1 PLS3
3 skeleton MP:0005390 9.1 IL2RA JUN JUNB PDCD1 PLS3 TERT

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
4 Hypo-pigmented Skin Lesions in Children : Dermoscopic Evaluation Not yet recruiting NCT04089475

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:

40
T Cells, Skin, B Cells, Bone, Nk Cells, Bone Marrow, Thymus

Publications for Primary Cutaneous T-Cell Lymphoma

Articles related to Primary Cutaneous T-Cell Lymphoma:

(show top 50) (show all 212)
# Title Authors PMID Year
1
Frequent and Persistent PLCG1 Mutations in Sézary Cells Directly Enhance PLCγ1 Activity and Stimulate NFκB, AP-1, and NFAT Signaling. 61
31376383 2020
2
Mycosis Fungoides, Lymphomatoid Papulosis and Hodgkin's Lymphoma in the Same Patient: Apropos of a Possible Monoclonal Origin. 61
32029943 2020
3
[LINEAR MYCOSIS FUNGOIDES IN A BLASCHKOID DISTRIBUTION]. 61
31930803 2020
4
Interferon alfa-2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement. 61
30565216 2019
5
Two cases of phenotypic switch of primary cutaneous T cell lymphoma after treatment with an aggressive course and review of the literature. 61
31218405 2019
6
High-throughput Sequencing of Subcutaneous Panniculitis-like T-Cell Lymphoma Reveals Candidate Pathogenic Mutations. 61
31702703 2019
7
Prevalence and spectrum of T-cell lymphoproliferative disorders in patients with Hypereosinophilia: A reference laboratory experience. 61
31778950 2019
8
Primary Cutaneous Peripheral T-Cell Lymphoma of the Hand: Case Report of a Rare Diagnosis and Review of the Literature. 61
31616124 2019
9
T-Cell-Rich Recurrence of Primary Cutaneous Follicle Center Lymphoma After Systemic Rituximab: A Diagnostic Pitfall. 61
31592859 2019
10
Mycosis Fungoides: A 10-year Turkish Experience. 61
31542058 2019
11
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. 61
31253572 2019
12
Effective remission of chidamide on treatment of advanced mycosis fungoides: An unusual case report. 61
31012254 2019
13
Sézary Syndrome, recent biomarkers and new drugs. 61
30525758 2019
14
Two Different Extranodal Lymphomas in an HIV+ Patient: A Case Report and Review of the Literature. 61
31662919 2019
15
Sézary Syndrome Presenting With Renal Involvement. 61
29941220 2018
16
Molecular pathogenesis of cutaneous lymphomas. 61
29862563 2018
17
Methotrexate in the treatment of mycosis fungoides - a multicenter observational study in 79 patients. 61
29917213 2018
18
Allogeneic hematopoietic stem cell transplantation in Primary Cutaneous T Cell Lymphoma. 61
29442161 2018
19
Total Skin Electron Beam Therapy with Rotary Dual Technique as Palliative Treatment for Mycosis Fungoides. 61
29695554 2018
20
Synchronous Occurrence of Mycosis Fungoides, Diffuse Large B Cell Lymphoma and Acute Myeloid Leukemia. 61
29321428 2018
21
Survival outcomes of primary cutaneous T-cell lymphoma in HIV-infected patients: a national population-based study. 61
29330308 2018
22
Nodal Involvement by CD30+ Cutaneous Lymphoproliferative Disorders and Its Challenging Differentiation From Classical Hodgkin Lymphoma. 61
29257929 2018
23
Second primary cutaneous T-cell lymphoma in patients with non-Hodgkin lymphoma: a chicken-or-the-egg issue. 61
29047113 2018
24
Histone deacetylase inhibitors downregulate CCR4 expression and decrease mogamulizumab efficacy in CCR4-positive mature T-cell lymphomas. 61
29025909 2018
25
Eruption of lymphocyte recovery with atypical lymphocytes mimicking a primary cutaneous T-cell lymphoma: a series of 12 patients. 61
29107664 2018
26
Low-dose total skin electron beam therapy for cutaneous lymphoma : Minimal risk of acute toxicities. 61
28785772 2017
27
A rare localised nasal CD30+ primary cutaneous T-cell lymphoma following liver transplantation. 61
29103006 2017
28
Immunohistochemistry reveals an increased proportion of MYC-positive cells in subcutaneous panniculitis-like T-cell lymphoma compared with lupus panniculitis. 61
28800143 2017
29
Brentuximab vedotin therapy for CD30-positive cutaneous T-cell lymphoma: a targeted approach to management. 61
28805086 2017
30
Immunohistopathological Study of c-FLIP Protein in Mycosis Fungoides 61
28952283 2017
31
Primary cutaneous T-cell lymphoma: experience from the Peruvian National Cancer Institute. 61
29166501 2017
32
[Role of stem cell transplantation in treatment of primary cutaneous T‑cell lymphoma]. 61
28785925 2017
33
Mycosis Fungoides: Experience in a Pediatric Hospital. 61
28279399 2017
34
Clinicopathologic Variants of Mycosis Fungoides. 61
27871620 2017
35
CD4-Positive T-Cell Large Granular Lymphocytosis Mimicking Sezary Syndrome in a Patient With Mycosis Fungoides. 61
28441377 2017
36
Primary Cutaneous T-cell Lymphoma with Aberrant Expression of CD20. 61
27840885 2017
37
Management Strategies for Mycosis Fungoides in India. 61
28400632 2017
38
Differential NFATc1 Expression in Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoma and Other Forms of Cutaneous T-Cell Lymphoma and Pseudolymphoma. 61
27097342 2017
39
Expression of SOX18 in Mycosis Fungoides. 61
27230943 2017
40
Total Skin Electron Beam Therapy as Part of Multimodal Treatment Strategies for Primary Cutaneous T-Cell Lymphoma. 61
28448985 2017
41
Fatal Case of Primary Cutaneous Aggressive T-Cell Lymphoma Switching From a CD4+ to a CD8+ Phenotype: Progressive Disease With Bexarotene and Romidepsin Treatment. 61
27322928 2016
42
Primary Cutaneous T Cell Lymphoma (Gamma Delta subtype). 61
28064300 2016
43
Genomic imbalances and microRNA transcriptional profiles in patients with mycosis fungoides. 61
27473081 2016
44
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. 61
27741114 2016
45
Expression of CD31 in Mycosis Fungoides. 61
27630298 2016
46
CD8-positive Mycosis Fungoides Masquerading as Pyoderma Gangrenosum. 61
27688458 2016
47
Alemtuzumab in refractory Sézary syndrome. 61
27828640 2016
48
MicroRNA-16 mediates the regulation of a senescence-apoptosis switch in cutaneous T-cell and other non-Hodgkin lymphomas. 61
26640145 2016
49
Mycosis Fungoides: A Retrospective Study of 44 Swedish Cases. 61
26778803 2016
50
How I treat mycosis fungoides and Sézary syndrome. 61
27151889 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 19)
# Super pathways Score Top Affiliating Genes
1 12.18 TNFRSF8 PDCD1 JUNB IL2RA
2 12.01 TERT JUN IL2RA
3 11.73 TERT JUNB JUN
4 11.58 TFRC MS4A1 IL2RA
5
Show member pathways
11.56 JUNB JUN
6
Show member pathways
11.38 JUNB JUN IL2RA
7 11.35 TNFRSF8 PDCD1
8 11.32 JUNB JUN
9 11.27 JUNB JUN
10 11.25 TFRC TERT JUN
11 11.24 JUNB JUN
12 11.19 JUNB JUN
13
Show member pathways
11.12 JUNB JUN IL2RA
14 11.1 JUNB JUN
15 11.04 JUNB JUN
16
Show member pathways
10.94 JUN IL2RA
17 10.9 JUN IL2RA
18 10.86 TNFRSF8 PDCD1 MS4A1
19
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:

# Name GO ID Score Top Affiliating Genes
1 humoral immune response GO:0006959 9.48 PDCD1 MS4A1
2 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.46 TERT JUN
3 cellular response to hormone stimulus GO:0032870 9.43 JUNB JUN
4 response to cAMP GO:0051591 9.4 JUNB JUN
5 positive regulation of cell differentiation GO:0045597 9.37 JUNB JUN
6 response to radiation GO:0009314 9.32 JUNB JUN
7 osteoclast differentiation GO:0030316 9.26 TFRC JUNB
8 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.16 TERT JUN
9 negative regulation of immune response GO:0050777 8.96 PDCD1 IL2RA
10 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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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