General Information of Disease (ID: DIS62RB8)

Disease Name Mycosis fungoides
Synonyms
granuloma fungoides; classic mycosis fungoides; Alibert-Bazin syndrome; mycosis fungoides lymphoma; CTCL/ mycosis fungoides; cutaneous T-cell lymphoma/mycosis fungoides; mycosis fungoides; mycosis fungoides (morphologic abnormality); MF; mycosis fungoides of unspecified site; mycosis fungoides, unspecified site, extranodal and solid organ sites; mycosis fungoides NOS (morphologic abnormality); mycosis fungoides, Alibert-Bazin type
Disease Class 2B01: Mycosis fungoides
Definition
Classical mycosis fungoides is the most common type of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, and is characterized by slow progression from patches to more infiltrated plaques and eventually to tumors.
Disease Hierarchy
DISWWW80: Mycosis fungoides and variants
DISAYSZB: Cutaneous T-cell lymphoma
DIS62RB8: Mycosis fungoides
ICD Code
ICD-11
ICD-11: 2B01
ICD-10
ICD-10: C84.0
Expand ICD-11
'2B01
Expand ICD-10
'C84.0
Disease Identifiers
MONDO ID
MONDO_0009691
MESH ID
D009182
UMLS CUI
C0026948
OMIM ID
254400
MedGen ID
7771
Orphanet ID
2584
SNOMED CT ID
118618005

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 16 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Anidulafungin DM8QM5U Approved Small molecular drug [1]
Betamethasone DMAHJEF Approved Small molecular drug [2]
Caspofungin DMGQIPT Approved Small molecular drug [3]
Cortisone Acetate DMG8K57 Approved Small molecular drug [4]
Cyclophosphamide DM4O2Z7 Approved Small molecular drug [5]
Dexamethasone DMMWZET Approved Small molecular drug [6]
Hydrocortisone DMGEMB7 Approved Small molecular drug [7]
Isotretinoin DM4QTBN Approved Small molecular drug [8]
Mechlorethamine DM0CVXA Approved Small molecular drug [9]
Methotrexate DM2TEOL Approved Small molecular drug [10]
Methylprednisolone DM4BDON Approved Small molecular drug [11]
Mogamulizumab DMISH0Z Approved Monoclonal antibody [12]
Prednisolone DMQ8FR2 Approved Small molecular drug [13]
Prednisone DM2HG4X Approved Small molecular drug [14]
Triamcinolone DM98IXF Approved Small molecular drug [15]
Vinblastine DM5TVS3 Approved Small molecular drug [16]
------------------------------------------------------------------------------------
⏷ Show the Full List of 16 Drug(s)
This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
AFM13 DMN45IC Phase 2 Antibody [17]
TTI-621 DMDNPAV Phase 1 Recombinant protein [18]
------------------------------------------------------------------------------------

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 23 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
CXCL11 TTWG0RE Limited Biomarker [19]
IL16 TTW4R0B Limited Altered Expression [20]
KIR3DL2 TTQH3N0 Limited Altered Expression [21]
USP2 TTUEQ1W Disputed Altered Expression [22]
CCL21 TTLZK1U Strong Biomarker [23]
CCR4 TT7HQD0 Strong Biomarker [24]
CD28 TTQ13FT Strong FusionGene [25]
CD7 TTP6B8O Strong Biomarker [26]
CTLA4 TTI2S1D Strong FusionGene [25]
DPP4 TTDIGC1 Strong Altered Expression [27]
HLA-DQB2 TTL7VOU Strong Genetic Variation [28]
IL31 TT1RJXK Strong Biomarker [29]
IL32 TTD4G7L Strong Biomarker [30]
KLK5 TTULSEW Strong Altered Expression [31]
RNF34 TTEWDK1 Strong Genetic Variation [32]
SELPLG TTS5K8U Strong Biomarker [33]
TCIRG1 TTVRN05 Strong Altered Expression [34]
TCL1A TTUKRDV Strong Altered Expression [35]
TNFRSF1B TT63WSF Strong Genetic Variation [36]
TRB TT84HCW Strong Genetic Variation [37]
TRBC1 TT1DHW2 Strong Genetic Variation [37]
USP14 TTVSYP9 Strong Biomarker [38]
ZNF217 TTY3BRA Strong Biomarker [39]
------------------------------------------------------------------------------------
⏷ Show the Full List of 23 DTT(s)
This Disease Is Related to 37 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
CCL27 OTUZYC61 Limited Altered Expression [40]
CCR10 OT7ZWSSD Limited Biomarker [40]
GNG8 OT9YHSF1 Limited Genetic Variation [41]
HM13 OTGEO1LP Limited Biomarker [42]
KRT1 OTIOJWA4 Limited Altered Expression [43]
NFKBIZ OTU728KS Limited Altered Expression [44]
NKG7 OTXDD006 Limited Altered Expression [45]
TRIP13 OTFM3TI9 Limited Biomarker [44]
SOX18 OTPUMHWA Disputed Biomarker [46]
ACSBG1 OTM040MW Strong Genetic Variation [47]
BCL7A OTYFUJTP Strong Genetic Variation [48]
CADM1 OTRWG9QS Strong Altered Expression [49]
CDO1 OTLG1P77 Strong Altered Expression [50]
DEFB103B OT8RWY64 Strong Altered Expression [51]
DNM3 OTDMUGCR Strong Altered Expression [50]
DUSP22 OTEZ3U85 Strong Genetic Variation [52]
HBD OTRQG4WA Strong Altered Expression [51]
HELLS OTVVV668 Strong Biomarker [53]
HHEX OTLIUVYX Strong Biomarker [53]
ICAM3 OTTZ5A5D Strong Biomarker [54]
INTS1 OT7TY1M1 Strong Genetic Variation [55]
INTS2 OT2N5TCK Strong Biomarker [55]
MYBL2 OTZ3JX8Q Strong Biomarker [39]
MYCL OT1MFQ5U Strong Biomarker [39]
NAV3 OT97M1TR Strong Genetic Variation [36]
PLA2G4D OTKBAB0F Strong Altered Expression [56]
PWWP3A OTXQVL4U Strong Altered Expression [52]
QTRT1 OTC33MCV Strong Biomarker [38]
RAPH1 OTMQXW7S Strong Genetic Variation [47]
RHOF OTPA3F8Q Strong Genetic Variation [48]
SHARPIN OTU1J2KH Strong Altered Expression [57]
SS18 OTDKHN1E Strong Biomarker [58]
STAT4 OTAK3VFR Strong Altered Expression [59]
TCL1B OT4CSO39 Strong Altered Expression [35]
TOX OTE8BL5Z Strong Altered Expression [60]
TSPYL2 OTGGW2EF Strong Biomarker [61]
BCL11B OT8KKCVJ Definitive Altered Expression [62]
------------------------------------------------------------------------------------
⏷ Show the Full List of 37 DOT(s)

References

1 Anidulafungin FDA Label
2 Betamethasone FDA Label
3 Caspofungin FDA Label
4 Cortisone acetate FDA Label
5 Cyclophosphamide FDA Label
6 Dexamethasone FDA Label
7 Hydrocortisone FDA Label
8 Isotretinoin FDA Label
9 Mechlorethamine FDA Label
10 Methotrexate FDA Label
11 Methylprednisolone FDA Label
12 2018 FDA drug approvals.Nat Rev Drug Discov. 2019 Feb;18(2):85-89.
13 Prednisolone FDA Label
14 Prednisone FDA Label
15 Triamcinolone FDA Label
16 Vinblastine FDA Label
17 ClinicalTrials.gov (NCT04101331) Phase II Study to Assess AFM13 in Patients With R/R CD30-positive T-cell Lymphoma or Transformed Mycosis Fungoides (REDIRECT). U.S. National Institutes of Health.
18 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
19 Tumor-associated M2 macrophages in mycosis fungoides acquire immunomodulatory function by interferon alpha and interferon gamma.J Dermatol Sci. 2016 Sep;83(3):182-9. doi: 10.1016/j.jdermsci.2016.05.004. Epub 2016 Jun 8.
20 IL-15 and IL-16 overexpression in cutaneous T-cell lymphomas: stage-dependent increase in mycosis fungoides progression.Exp Dermatol. 2000 Aug;9(4):248-51. doi: 10.1034/j.1600-0625.2000.009004248.x.
21 Leukaemic variants of cutaneous T-cell lymphoma: Erythrodermic mycosis fungoides and Szary syndrome.Best Pract Res Clin Haematol. 2019 Sep;32(3):239-252. doi: 10.1016/j.beha.2019.06.004. Epub 2019 Jun 6.
22 Ubiquitin-specific protease 2 decreases p53-dependent apoptosis in cutaneous T-cell lymphoma.Oncotarget. 2016 Jul 26;7(30):48391-48400. doi: 10.18632/oncotarget.10268.
23 CCL21 Induces mTOR-dependent MALAT1 Expression, Leading to Cell Migration in Cutaneous T-Cell Lymphoma.In Vivo. 2019 May-Jun;33(3):793-800. doi: 10.21873/invivo.11541.
24 C-C chemokine receptor 4 expression in CD8+ cutaneous T-cell lymphomas and lymphoproliferative disorders, and its implications for diagnosis and treatment.Histopathology. 2020 Jan;76(2):222-232. doi: 10.1111/his.13960. Epub 2019 Nov 13.
25 Genomic analysis of mycosis fungoides and Szary syndrome identifies recurrent alterations in TNFR2.Nat Genet. 2015 Sep;47(9):1056-60. doi: 10.1038/ng.3370. Epub 2015 Aug 10.
26 Leu-8 and Leu-9 antigen phenotypes: immunologic criteria for the distinction of mycosis fungoides from cutaneous inflammation.J Am Acad Dermatol. 1986 Jun;14(6):1006-13. doi: 10.1016/s0190-9622(86)70124-2.
27 Usefulness of Flow Cytometry for the Detection of Cutaneous Localization in Malignant Hematologic Disorders.Cytometry B Clin Cytom. 2019 Jul;96(4):283-293. doi: 10.1002/cyto.b.21784. Epub 2019 May 3.
28 Familial mycosis fungoides: report of 6 kindreds and a study of the HLA system.J Am Acad Dermatol. 2005 Mar;52(3 Pt 1):393-402. doi: 10.1016/j.jaad.2003.12.052.
29 Analysis of the IL-31 pathway in Mycosis fungoides and Szary syndrome.Arch Dermatol Res. 2015 Aug;307(6):479-85. doi: 10.1007/s00403-014-1527-x. Epub 2014 Dec 7.
30 IL-32 induces indoleamine 2,3-dioxygenase(+)CD1c(+) dendritic cells and indoleamine 2,3-dioxygenase(+)CD163(+) macrophages: Relevance to mycosis fungoides progression.Oncoimmunology. 2016 May 5;6(2):e1181237. doi: 10.1080/2162402X.2016.1181237. eCollection 2017.
31 Mechanisms of itching in mycosis fungoides: grade of itching correlates with eosinophil infiltration and kallikrein 5 expression.Eur J Dermatol. 2019 Jun 1;29(3):268-273. doi: 10.1684/ejd.2019.3560.
32 Maintenance phase in psoralen-ultraviolet A phototherapy of early-stage mycosis fungoides. Acritically appraised topic.Br J Dermatol. 2017 Aug;177(2):406-410. doi: 10.1111/bjd.15302.
33 Expression of HECA-452 antigen correlates with disease relapse in mycosis fungoides.Anal Quant Cytol Histol. 2008 Aug;30(4):203-8.
34 Genetic Analysis of Giant Cell Lesions of the Maxillofacial and Axial/Appendicular Skeletons.J Oral Maxillofac Surg. 2017 Feb;75(2):298-308. doi: 10.1016/j.joms.2016.07.014. Epub 2016 Jul 26.
35 Regulation of TCL1 expression in B- and T-cell lymphomas and reactive lymphoid tissues.Cancer Res. 2000 Apr 15;60(8):2095-100.
36 Analysis of differential variable region of T cell receptor expression and NAV3/TNFRSF1B gene mutation in mycosis fungoides.Oncotarget. 2016 Apr 5;7(14):17986-90. doi: 10.18632/oncotarget.7673.
37 Radiotherapy Eradicates Malignant T Cells and Is Associated with Improved Survival in Early-Stage Mycosis Fungoides.Clin Cancer Res. 2020 Jan 15;26(2):408-418. doi: 10.1158/1078-0432.CCR-18-4147. Epub 2019 Oct 21.
38 K-Ras mutant fraction in A/J mouse lung increases as a function of benzo[a]pyrene dose.Environ Mol Mutagen. 2010 Mar;51(2):146-55. doi: 10.1002/em.20513.
39 Amplification and overexpression of JUNB is associated with primary cutaneous T-cell lymphomas.Blood. 2003 Feb 15;101(4):1513-9. doi: 10.1182/blood-2002-08-2434. Epub 2002 Oct 3.
40 Microarray analysis of gene expression by microdissected epidermis and dermis in mycosis fungoides and adult T-cell leukemia/lymphoma.Int J Oncol. 2014 Sep;45(3):1200-8. doi: 10.3892/ijo.2014.2524. Epub 2014 Jun 26.
41 Immunophenotyping and gene rearrangement analysis provide additional criteria to differentiate between cutaneous T-cell lymphomas and pseudo-T-cell lymphomas.Am J Pathol. 1997 Jun;150(6):1941-9.
42 Expression of cancer/testis antigens in cutaneous T cell lymphomas.Int J Cancer. 2002 Feb 10;97(5):668-70. doi: 10.1002/ijc.1643.
43 Differential expression of CRABP II, psoriasin and cytokeratin 1 mRNA in human skin diseases.Arch Dermatol Res. 1996 Jul;288(8):426-30. doi: 10.1007/BF02505229.
44 A meta-analysis of gene expression data identifies a molecular signature characteristic for tumor-stage mycosis fungoides.J Invest Dermatol. 2012 Aug;132(8):2050-9. doi: 10.1038/jid.2012.117. Epub 2012 Apr 19.
45 Th1 response and cytotoxicity genes are down-regulated in cutaneous T-cell lymphoma.Clin Cancer Res. 2006 Aug 15;12(16):4812-21. doi: 10.1158/1078-0432.CCR-06-0532.
46 Expression of SOX18 in Mycosis Fungoides.Acta Derm Venereol. 2017 Jan 4;97(1):17-23. doi: 10.2340/00015555-2466.
47 Nodal involvement by cutaneous CD30-positive T-cell lymphoma mimicking classical Hodgkin lymphoma.Am J Surg Pathol. 2012 May;36(5):716-25. doi: 10.1097/PAS.0b013e3182487158.
48 Array-based comparative genomic hybridization in early-stage mycosis fungoides: recurrent deletion of tumor suppressor genes BCL7A, SMAC/DIABLO, and RHOF.Genes Chromosomes Cancer. 2008 Dec;47(12):1067-75. doi: 10.1002/gcc.20601.
49 The expression of cell adhesion molecule 1 and its splicing variants in Szary cells and cell lines from cutaneous T-cell lymphoma.J Dermatol. 2019 Nov;46(11):967-977. doi: 10.1111/1346-8138.15078. Epub 2019 Sep 12.
50 Szary syndrome is a unique cutaneous T-cell lymphoma as identified by an expanded gene signature including diagnostic marker molecules CDO1 and DNM3.Leukemia. 2008 Feb;22(2):393-9. doi: 10.1038/sj.leu.2405044. Epub 2007 Nov 22.
51 Expression of human beta-defensins in patients with mycosis fungoides.Arch Dermatol Res. 2007 Jul;299(4):221-4. doi: 10.1007/s00403-007-0749-6. Epub 2007 Apr 6.
52 Mycosis fungoides in Taiwan shows a relatively high frequency of large cell transformation and CD56 expression.Pathology. 2018 Dec;50(7):718-724. doi: 10.1016/j.pathol.2018.08.008. Epub 2018 Oct 20.
53 Fine mapping of chromosome 10q deletions in mycosis fungoides and sezary syndrome: identification of two discrete regions of deletion at 10q23.33-24.1 and 10q24.33-25.1.Genes Chromosomes Cancer. 2005 Feb;42(2):184-92. doi: 10.1002/gcc.20115.
54 CD20 positive mycosis fungoides: a case report.J Cutan Pathol. 2008 Apr;35(4):398-403. doi: 10.1111/j.1600-0560.2007.00817.x. Epub 2008 Feb 4.
55 Genetically inspired prognostic scoring system (GIPSS) outperforms dynamic international prognostic scoring system (DIPSS) in myelofibrosis patients.Am J Hematol. 2019 Jan;94(1):87-92. doi: 10.1002/ajh.25335. Epub 2018 Nov 25.
56 Cloning of a gene for a novel epithelium-specific cytosolic phospholipase A2, cPLA2delta, induced in psoriatic skin.J Biol Chem. 2004 Mar 26;279(13):12890-7. doi: 10.1074/jbc.M305801200. Epub 2004 Jan 6.
57 SHARPIN overexpression promotes TAK1 expression and activates JNKs and NF-B pathway in Mycosis Fungoides.Exp Dermatol. 2019 Nov;28(11):1279-1288. doi: 10.1111/exd.14026. Epub 2019 Sep 10.
58 Primary cutaneous T-cell lymphomas do not show specific NAV3 gene deletion or translocation.J Invest Dermatol. 2008 Oct;128(10):2458-66. doi: 10.1038/jid.2008.113. Epub 2008 May 29.
59 Analysis of STAT4 expression in cutaneous T-cell lymphoma (CTCL) patients and patient-derived cell lines.Cell Cycle. 2014;13(18):2975-82. doi: 10.4161/15384101.2014.947759.
60 Molecular profiling of TOX-deficient neoplastic cells in cutaneous T cell lymphoma.Arch Dermatol Res. 2020 Sep;312(7):513-525. doi: 10.1007/s00403-019-02000-0. Epub 2019 Nov 1.
61 Primary cutaneous non-Hodgkin lymphoma: results of a retrospective analysis in the light of the recent ILROG guidelines.Tumori. 2018 Oct;104(5):394-400. doi: 10.5301/tj.5000606. Epub 2018 May 8.
62 BCL11B-Mediated Epigenetic Repression Is a Crucial Target for Histone Deacetylase Inhibitors in Cutaneous T-Cell Lymphoma.J Invest Dermatol. 2017 Jul;137(7):1523-1532. doi: 10.1016/j.jid.2017.02.980. Epub 2017 Mar 10.