Cutaneous T cell lymphoma

"CTCL" redirects here. For the book by Loren Pope, see Colleges That Change Lives.

Cutaneous T cell lymphoma
200px Micrograph showing cutaneous T-cell lymphoma. H&E stain.
Classification and external resources
Specialty	Hematology and oncology
ICD-10	C84.0, C84.1
ICD-9-CM	202.1, 202.2
ICD-O	M9700/3, M9701/3
DiseasesDB	8595
eMedicine	med/3486
Patient UK	Cutaneous T cell lymphoma
MeSH	D016410

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.

Classification

Cutaneous T-cell lymphomas may be divided into the following types:

• Mycosis fungoides
• 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

A WHO-EORTC classification has been developed.

Treatment

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
• 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)

Other drugs are under investigation (for example: panobinostat, Resimmune and resiquimod).

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 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.

See also

• Cutaneous B-cell lymphoma
• List of cutaneous conditions

External links

• DermNet dermal-infiltrative/cutaneous-t-cell-lymphoma

• Information on cutaneous T-cell lymphomas from Lymphoma Information Network
• cutaneous T-cell lymphoma from H. Lee Moffitt Cancer Center & Research Institute
• The Cutaneous Lymphoma Foundation
• Skin Research Center lab Hopital St Louis, Paris (France) Dir. Dr. A. Bensussan