cutaneous t-cell lymphoma cutaneous t-cell lymphoma 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. cutaneous t-cell lymphomamicrograph showing cutaneous t-cell lymphoma. h&e stainspecialtyhematology and oncology 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 primary cutaneous anaplastic large-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 and narrow band uvb or puva) electron therapy 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 naloxone lotion as a 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.