Cutaneous T cell lymphoma
|Cutaneous T cell lymphoma|
|Micrograph showing cutaneous T-cell lymphoma. H&E stain.|
|Classification and external resources|
|Specialty||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 lymphomas may be divided into the following types::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
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
- Alemtuzumab (Campath-1H)
- Pentostatin and other purine analogues (Fludarabine, 2-deoxychloroadenosine)
- Liposomal doxorubicin (Doxil)
- Gemcitabine (Gemzar)
- 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 a topical treatment for pruritus in cutaneous T-cell lymphoma to a pharmaceutical company called Elorac.
- James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- 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.
- 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.
- Elorac, Inc. Announces Orphan Drug Designation for Novel Topical Treatment for Pruritus in Cutaneous T-cell Lymphoma (CTCL) website
- 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.)
- 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.