Primary Cutaneous Lymphoma

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

TNFRSF8, NCAM1, ACVRL1, SLPI, SMUG1, MALT1, TOX, TRGC1, TRG, STAT1, NFKB2, NPM1, ALK, JAK1, ERBB3, TSC22D3, CD44, CASP1, TARP

Drugs

A lentiviral vector pseudotyped by the Indiana serotype of the vesicular stomatitis virus G protein encoding an antigen derived from the Tax, HBZ, p12I and p30II HTLV-1 proteins, A lentiviral vector pseudotyped by the New-Jersey serotype of the vesicular stomatitis virus G protein encoding an antigen derived from the Tax, HBZ, p12I and p30II HTLV-1 proteins, A-dmDT390-bisFv(UCHT1), Adenovirus-Interferon gamma - coding DNA sequence, Alisertib, Allogeneic multi-virus specific T lymphocytes targeting BK virus, cytomegalovirus, human herpesvirus-6, Epstein Barr virus and adenovirus, Autologous Epstein-Barr virus specific T cells derived from peripheral blood mononuclear cells, expanded ex vivo-, Belinostat ( BELEODAQ ), Brentuximab vedotin ( ADCETRIS ), Chlormethine ( Ledaga ), Darinaparsin, Denileukin diftitox, Fenretinide, Forodesine hydrochloride, Human monoclonal antibody against CD4, Humanised IgG1 monoclonal antibody against human KIR3DL2, Meclorethamine ( VALCHLOR ), Miltefosine ( IMPAVIDO ), Mogamulizumab ( POTELIGEO ), N-(2-aminophenyl)-4-(1-[(1,3-dimethyl-1H-pyrazol-4-yl)methyl]piperidin)benzamide, Naloxone hydrochloride dihydrate, Panobinostat ( FARYDAK ), Pralatrexate ( FOLOTYN ), Recombinant anti-CD3-bi-single-chain-Fv-diphtheria toxin fusion protein, Resiquimod, Rituximab ( BLITZIMA, MABTHERA, TRUXIMA, RITEMVIA, RITUXAN, RITUZENA, RIXATHON, RIXIMYO, Ruxience ), Romidepsin ( ISTODAX ), Sintilimab, Suberolylanilide hydroxamic acid, Synthetic hypericin, Vorinostat ( ZOLINZA ), Zanolimumab, haematopoietic stem cells and blood progenitors umbilical cord-derived expanded with (1R, 4R)-N1-(2-benzyl-7-(2-methyl-2H-tetrazol-5-yl)-9H-pyrimido[4,5-b]indol-4-yl)cyclohexane-1,4-diamine dihydrobromide dihydrate, oligonucleotide inhibitor of microRNA miR-155-5p

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Cutaneous lymphoma is a heterogeneous entity with respect to its clinical and pathological features, evolutive profile, prognosis, molecular aetiology and response to therapy. These specifications have been taken into account in recent classifications, which have placed particular importance on the prognostic implications of these different entities.

Clinical description

Cutaneous T-cell lymphomas can be subdivided into forms with a good prognosis (Mycosis fungoides and CD30+ cutaneous T-cell lymphoma), forms with a poor prognosis (Sezary syndrome and peripheral T-cell lymphoma) and forms with a variable prognosis (pleomorphic small/medium-sized T-cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, cutaneous T/NK cell lymphomas (referred to as nasal-type lesions), epidermotropic CD8+ T-cell lymphomas and the TCRgamma-delta phenotype). The group of cutaneous B-cell lymphomas includes marginal zone B-cell lymphomas and primary cutaneous follicular lymphomas (both of which have a good prognosis), cutaneous large B-cell lymphomas of the leg (with a variable prognosis), and blastic NK lymphomas (associated with a poor prognosis).

Management and treatment

While treatment is effective and the prognosis is favourable for low grade forms, recent therapeutic innovations relying on the use of biological tools are likely to improve the prognosis for the most severe forms, such as those already existing in the form of anti-CD20 monoclonal antibodies (rituximab) for disseminated cutaneous B-cell lymphomas, and anti-CD52 monoclonal antibody (alemtuzumab) for refractory forms of cutaneous T-cell lymphoma. Deciphering the key molecular mechanisms underlying these malignancies should create new therapeutic perspectives in the close future.