Cutaneous T-cell lymphoma
From Wikipedia, the free encyclopedia
| Cutaneous T cell lymphoma | |
|---|---|
| Classification and external resources | |
| 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 are pushed to the surface of the skin in a biological process used to rid the body of offending material, causing various lesions to appear on the skin. These lesions change shape as the disease progresses, typically beginning as what appears to be a rash and eventually forming plaques and tumors before metastasizing to other parts of the body.
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[edit] Classification
Cutaneous T-cell lymphomas may be divided into the following types:[1]:727–740
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- 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
[edit] Treatment
There is no cure for CTCL, but there are a variety of treatment options available and some CTCL patients are able to live somewhat normal lives with this cancer, although symptoms can be debilitating and painful, even in earlier stages.
FDA approved treatments are :[2]
- (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 (Nitrogen Mustard)
- Mechlorethamine
- 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
Other drugs are under investigation (for example panobinostat).
[edit] Epidemiology
Of all cancers involving the same class of blood cell, 2% of cases are cutaneous T cell lymphomas.[3]
There is some evidence of a relationship with human T-lymphotropic virus.[4]
[edit] See also
[edit] References
- ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ^ http://www.istockanalyst.com/article/viewnewspaged/articleid/3622028/pageid/1
- ^ 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:. PMID 15726602. http://dx.doi.org/10.1002/ajh.20307.
[edit] External links
- 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
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