Cutaneous T-cell lymphoma

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

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

Cutaneous T cell lymphoma (CTCL) is a class of non-Hodgkin's lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin's 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:^{[1]:727–740}

• 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.^[2][3]

Treatment

There is no cure for CTCL, but there are a variety of treatment options available and most 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 :^[4]

• (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 & Total Skin Electron Beam Radiation
• 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).

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.^[5]

Epidemiology

Of all cancers involving the same class of blood cell, 2% of cases are cutaneous T cell lymphomas.^[6]

There is some evidence of a relationship with human T-lymphotropic virus.^[7]

See also

• Cutaneous B-cell lymphoma
• List of cutaneous conditions

References

1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. 
2. ^ Willemze, R.; Jaffe, ES.; Burg, G.; Cerroni, L.; Berti, E.; Swerdlow, SH.; Ralfkiaer, E.; Chimenti, S. et al. (May 2005). "WHO-EORTC classification for cutaneous lymphomas.". Blood 105 (10): 3768–85. doi:10.1182/blood-2004-09-3502. PMID 15692063. 
3. ^ Khamaysi, Z.; Ben-Arieh, Y.; Izhak, OB.; Epelbaum, R.; Dann, EJ.; Bergman, R. (Feb 2008). "The applicability of the new WHO-EORTC classification of primary cutaneous lymphomas to a single referral center.". Am J Dermatopathol 30 (1): 37–44. doi:10.1097/DAD.0b013e31815f9841. PMID 18212543. 
4. ^ http://www.istockanalyst.com/article/viewnewspaged/articleid/3622028/pageid/1
5. ^ Elorac, Inc. Announces Orphan Drug Designation for Novel Topical Treatment for Pruritus in Cutaneous T-cell Lymphoma (CTCL) website
6. ^ Turgeon, Mary Louise (2005). Clinical hematology: theory and procedures. Hagerstown, MD: Lippincott Williams & Wilkins. p. 283. ISBN 0-7817-5007-5. "Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001.)" 
7. ^ Nicot C (March 2005). "Current views in HTLV-I-associated adult T-cell leukemia/lymphoma". Am. J. Hematol. 78 (3): 232–9. doi:10.1002/ajh.20307. PMID 15726602. 

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