| C84.0, C84.1|
| 202.1, 202.2|
| Hematology and oncology|
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 malignant 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 metastasizing to other parts of the body.
Cutaneous T cell lymphoma Wikipedia
Cutaneous T-cell lymphomas may be divided into the following types:
Granulomatous slack skin
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
Subcutaneous T-cell lymphoma
Blastic NK-cell lymphoma
A WHO-EORTC classification has been developed.
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 are:(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
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
Pentostatin and other purine analogues (Fludarabine, 2-deoxychloroadenosine)
Liposomal doxorubicin (Doxil)
Bone marrow / stem cells
Forodesine (Inhibits Purine Nucleoside phosphorylase)
Other drugs are under investigation (for example: panobinostat, Resimmune, resiquimod and synthetic hypericin).
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.
Of all cancers involving the same class of blood cell, 2% of cases are cutaneous T cell lymphomas.
There is some evidence of a relationship with human T-lymphotropic virus.