Cutaneous T-cell lymphoma
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.
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 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 :
- (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
- 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)
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.
- ^ 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.
- ^ http://www.istockanalyst.com/article/viewnewspaged/articleid/3622028/pageid/1
- ^ 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.
- 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
Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)By development/
markerALL (Precursor B acute lymphoblastic leukemia/lymphoma)CD5+CD22+see immunoproliferative immunoglobulin disordersBy infectionBy development/
CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma · Pleomorphic T-cell lymphoma · Lymphomatoid papulosis type BCD30+: CD30+ cutaneous T-cell lymphoma · Secondary cutaneous CD30+ large cell lymphoma · Lymphomatoid papulosis type AOther peripheralBy infectionHTLV-1 (Adult T-cell leukemia/lymphoma)NK cell/
(most CD56)Aggressive NK-cell leukemia · Blastic NK cell lymphomaT or NKLymphoid+myeloid
Cutaneous lymphoid hyperplasiaCutaneous lymphoid hyperplasia with bandlike and perivascular patterns · Cutaneous lymphoid hyperplasia with nodular pattern · Jessner lymphocytic infiltrate of the skin
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