Cutaneous T Cell Lymphoma

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Retrieved

2021-01-18

Source

Wikipedia

Trials

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Genes

CTLA4, CD28, TP53, FAS, CDKN2A, STAT5B, PLCG1, MCL1, NFKB2, IRF4, MIR22, ZEB1, BAX, TNFAIP3, CTCF, PRKCQ, RHOA, ATM, ARID1A, FGFR3, CARD11, DNMT3A, BRAF, TRBV20OR9-2, HDAC9, IL15, TNFRSF1B, TNFRSF8, BCL2, STAT3, CCR4, IL10, IL2, JAK3, IL4, MIR155, IFNG, IL7, KIR3DL2, STAT5A, TOX, IL17A, NOTCH1, IL5, HDAC1, CCR6, FOXP3, SOAT1, CDKN2B, CXCR4, CCR10, DNER, NAV3, ITGAM, CTAGE1, IFNA1, ISG20, TSPYL2, IFNA13, IL16, MDM2, IL2RA, JUNB, ITGB2, JAK1, MIR150, CD164, CTNNB1, TNF, XPR1, IL31, CCL20, MIR203A, MIR205, RARA, TRBV16, STAT6, SYCP1, TRB, CTDSP1, MUC1, TRG, TRGC1, TGFB1, SCPEP1, THBS1, SOCS3, CFLAR, VEGFA, AHI1, MYC, STAT4, PTPN6, PIK3CG, PIK3CD, TRBC1, MAPK3, PIK3CB, PIK3CA, PAK1, SAMHD1, BRD4, HDAC6, OPRM1, ERVW-1, IL22, NRAS, AKT1, SATB1, NFKB1, TRBV7-9, RAD23B, TP63, THEMIS, CD40LG, IL1A, FOS, IL1B, CBL, CCR3, GATA3, PPP1R2C, IL6, CXCL8, TARP, IL17F, DPP4, FKBP4, CXCL10, CD47, EZH2, ATN1, IL13, H3P10, ANG, BIN1, RUNX3, MIR122, DLEU1, MIRLET7I, SUPT7L, NFKBIZ, CCL26, CIB1, MRPL28, POSTN, CD226, MALAT1, CCL27, SUB1, RAB40B, CKAP4, MIR126, SART3, MIR200B, ERVK-20, TNFSF10, TNFSF9, H3P42, H3P9, HDAC3, ARHGEF7, LINC02605, USP2, ERVK-32, ERVK-18, MIR4433A, MIR200C, MIR711, DEFB4B, KIR2DS2, IL32, STK17B, MIR337, MIR223, SIRT2, MIR214, MIR21, PSIP1, SIRT1, TPX2, KRT20, SPZ1, ASCC2, ARHGAP24, LBH, TXNDC5, ASCC1, HM13, HDAC7, GDE1, BCL11A, SOX18, CHFR, CXCL17, HDAC8, MYDGF, TIGAR, NLN, PREX1, MUL1, IL22RA1, IL21, SOCS1, BCL11B, IL25, IL20, HAVCR2, TSLP, TBX21, PDCD11, ARC, DICER1, CADM1, POT1, PRSS55, CBLL2, SND1, BBC3, CARMIL2, IL31RA, IL17B, H4-16, GTSF1, APOA5, FCRL3, GNG8, DROSHA, NXT1, PYCARD, CD274, IL19, OPN4, JAG1, RRM2, NCOA1, HSP90AA1, FLT4, FOSB, FOSL2, FTH1, GATA6, CXCR3, GSTM1, GTF2H4, HDAC2, HLA-C, HLA-DQB1, HLA-DRB1, HMGB1, NR4A1, ICAM3, ZNF195, IFNA2, IKBKB, IL2RG, IL3RA, IL9, TNFRSF9, IL18, IDO1, ITGB7, JAK2, JUN, JUND, KIT, KRAS, FLG, FOXO3, FKBP5, EPHA4, ANXA5, BIRC5, APOC3, FASLG, ASS1, CCND1, BCL6, BCL7A, BLK, CASP1, CASP3, CASP9, CCND2, CD5, MS4A1, CD22, CD80, CD40, CD44, CDK2, CDKN1B, CDKN1C, CHI3L1, CCR7, CSF2, CUX1, DECR1, DEFB4A, DNMT1, LAIR2, LCK, LGALS1, RAG1, RB1, REG1A, RELB, RPE65, S100A7, CCL17, CCL18, CCL21, CCL22, SDC4, SELL, SELPLG, PMEL, SST, STAT1, STAT2, AURKA, MAP3K7, CNTN2, PPP1R11, TDO2, THBS4, TP73, CRISP2, NR2C2, TRAF2, TWIST1, TNFSF4, UVRAG, RAG2, RAC1, LGALS9, PVR, LTA, MBL2, MDM4, MIA2, MGMT, CXCL9, MT1E, MTAP, NCAM1, NFATC1, NTS, NTSR1, OAS3, PAEP, PRKN, PCM1, PDGFRA, PGF, ABCB1, PIM1, PKM, PLK1, PLS3, PTEN, PTH, PTGS2, PTPN11, PTPRC, PTPRG, H3P40

Drugs

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Cutaneous T cell lymphoma (CTCL) is a class of non-Hodgkin lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin lymphomas (which are generally B cell related), CTCL is caused by a mutation of T cells. The cancerous T cells in the body initially migrate to the skin, causing various lesions to appear. These lesions change shape as the disease progresses, typically beginning as what appears to be a rash which can be very itchy and eventually forming plaques and tumors before spreading to other parts of the body.

Signs and symptoms

The presentation depends if it is mycosis fungoides or Sézary syndrome, the most common, though not the only types. Among the symptoms for the aforementioned types are: enlarged lymph nodes, an enlarged liver and spleen, and non-specific dermatitis.

Cause

The cause of CTCL is unknown.

Diagnosis

A point-based algorithm for the diagnosis for early forms of cutaneous T cell lymphoma was proposed by The International Society for Cutaneous Lymphomas in 2005.

Classification

Cutaneous T-cell lymphoma may be divided into the several subtypes.^:727–740 Mycosis fungoides is the most common form of CTCL and is responsible for half of all cases. A WHO-EORTC classification has been developed.

Pagetoid reticulosis
Sézary syndrome
Granulomatous slack skin
Lymphomatoid papulosis
Pityriasis lichenoides chronica
Pityriasis lichenoides et varioliformis acuta
CD30+ cutaneous T-cell lymphoma
Secondary cutaneous CD30+ large cell lymphoma
Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
Pleomorphic T-cell lymphoma
Lennert lymphoma
Subcutaneous T-cell lymphoma
Angiocentric lymphoma
Blastic NK-cell lymphoma

Treatment

Romidepsin

There is no cure for CTCL, but there are a variety of treatment options available and some CTCL patients are able to live normal lives with this cancer, although symptoms can be debilitating and painful, even in earlier stages. FDA approved treatments include the following:

(1999) Denileukin diftitox (Ontak)
(2000) Bexarotene (Targretin) a retinoid
(2006) Vorinostat (Zolinza) a hydroxymate histone deacetylase (HDAC) inhibitor
(2009) Romidepsin (Istodax) a cyclic peptide histone deacetylase (HDAC) inhibitor
(2018) Poteligeo (mogamulizumab-kpkc)

Histone deacetylase (HDAC) inhibitors are shown to have antiproliferative and cytotoxic properties against CTCL.Other (off label) treatments include:

Topical and oral corticosteroids
Bexarotene (Targretin) gel and capsules
Carmustine (BCNU, a nitrosourea)
Mechlorethamine (Nitrogen Mustard)
Phototherapy (Broad & Narrow Band UVB or PUVA)
Local and Total Skin Electron Therapy (TSET)
Conventional Radiation Therapy
Photopheresis
Interferons
Alemtuzumab (Campath-1H)
Methotrexate
Pentostatin and other purine analogues (Fludarabine, 2-deoxychloroadenosine)
Liposomal doxorubicin (Doxil)
Gemcitabine (Gemzar)
Cyclophosphamide
Bone marrow / stem cells
Allogenic transplantation
Forodesine (Inhibits Purine Nucleoside phosphorylase)

In 2010, the U.S. Food and Drug Administration granted orphan drug designation for a topical treatment for pruritus in cutaneous T-cell lymphoma to a pharmaceutical company called Elorac.

Epidemiology

Of all cancers involving lymphocytes, 2% of cases are cutaneous T cell lymphomas. CTCL is more common in men and in African-American people. The incidence of CTCL in men is 1.6 times higher than in women.

There is some evidence of a relationship with human T-lymphotropic virus (HTLV) with the adult T-cell leukemia/lymphoma subtype. No definitive link between any viral infection or environmental factor has been definitely shown with other CTCL subtypes.