Cutaneous T Cell lymphoma
Encyclopedia
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 cell
s. The malignant
T cells in the body initially migrate to the skin
, causing various lesion
s 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 tumor
s before metastasizing
to other parts of the body.
A WHO
-EORTC
classification has been developed.
FDA approved treatments are :
Other (off label) Treatments include:
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.
, 2% of cases are cutaneous T cell lymphomas.
There is some evidence of a relationship with human T-lymphotropic virus
.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
of the immune system
Immune system
An immune system is a system of biological structures and processes within an organism that protects against disease by identifying and killing pathogens and tumor cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own...
. Unlike most non-Hodgkin's lymphomas (which are generally B-cell related), CTCL is caused by a mutation of T cell
T cell
T cells or T lymphocytes belong to a group of white blood cells known as lymphocytes, and play a central role in cell-mediated immunity. They can be distinguished from other lymphocytes, such as B cells and natural killer cells , by the presence of a T cell receptor on the cell surface. They are...
s. The malignant
Malignant
Malignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
T cells in the body initially migrate to the skin
Skin
-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
, causing various lesion
Lesion
A lesion is any abnormality in the tissue of an organism , usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.- Types :...
s to appear. These lesions change shape as the disease progresses, typically beginning as what appears to be a rash
Rash
A rash is a change of the skin which affects its color, appearance or texture. A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, cracked or blistered, swell and may be painful. The causes, and...
which can be very itchy and eventually forming plaques and tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
s before metastasizing
Metastasis
Metastasis, or metastatic disease , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
to other parts of the body.
Classification
Cutaneous T-cell lymphomas may be divided into the following types:- Mycosis fungoidesMycosis fungoides-External links:* * *...
- Pagetoid reticulosisPagetoid reticulosisPagetoid reticulosis is a cutaneous condition, an uncommon lymphoproliferative disorder, sometimes considered a form of mycosis fungoides....
- Sézary syndrome
- Granulomatous slack skinGranulomatous slack skinGranulomatous slack skin is a rare cutaneous condition, a variant of lymphoma that typically presents in middle aged adults.It is a form of cutaneous T-cell lymphoma.It is a variant of mycosis fungoides....
- Lymphomatoid papulosisLymphomatoid papulosisLymphomatoid papulosis is a rare skin disorder. The overall prevalence rate of lymphomatoid papulosis is estimated at 1.2 to 1.9 cases per 1,000,000 population.This rare condition has only been studied in depth since 1968.-Presentation:...
- Pityriasis lichenoides chronicaPityriasis lichenoides chronicaPityriasis lichenoides chronica is an uncommon, idiopathic, acquired dermatosis, characterized by evolving groups of erythematous, scaly papules that may persist for months.- Causes :Pityriasis...
- Pityriasis lichenoides et varioliformis acutaPityriasis lichenoides et varioliformis acutaPityriasis lichenoides et varioliformis acuta is a disease of the immune system. It is the more severe version of Pityriasis lichenoides chronica...
- CD30+ cutaneous T-cell lymphomaCD30+ cutaneous T-cell lymphomaCD30+ cutaneous T-cell lymphoma is a cutaneous condition characterized by solitary of localized skin lesions that have a tendency to ulcerate.- See also :* Cutaneous T-cell lymphoma...
- Secondary cutaneous CD30+ large cell lymphomaSecondary cutaneous CD30+ large cell lymphomaSecondary cutaneous CD30+ large-cell lymphoma is a cutaneous condition that may arise in cases of mycosis fungoides, and in patients with lymphomatoid papulosis.- See also :* Cutaneous T-cell lymphoma* CD30+ cutaneous T-cell lymphoma* Skin lesion...
- Non-mycosis fungoides CD30− cutaneous large T-cell lymphomaNon-mycosis fungoides CD30− cutaneous large T-cell lymphomaNon-mycosis fungoides CD30− cutaneous large T-cell lymphoma is a cutaneous condition that usually presents as solitary or generalized plaques, nodules, or tumors of short duration.- See also :* Cutaneous T-cell lymphoma* CD30+ cutaneous T-cell lymphoma...
- Pleomorphic T-cell lymphomaPleomorphic T-cell lymphomaPleomorphic T-cell lymphoma is a cutaneous condition characterized by a 5-year survival rate of 62%.- See also :* Cutaneous T-cell lymphoma...
- Lennert lymphomaLennert lymphomaLennert lymphoma is a systemic T-cell lymphoma that presents with cutaneous skin lesions roughly 10% of the time.It is also known as "lymphoepithelioid variant of peripheral T-cell lymphoma"....
- Subcutaneous T-cell lymphomaSubcutaneous T-cell lymphomaSubcutaneous T-cell lymphoma is a cutaneous condition that most commonly presents in young adults, and is characterized by subcutaneous nodules.-Classification:...
- Angiocentric lymphomaAngiocentric lymphomaAngiocentric lymphoma is a cutaneous condition which in Korea, is reported to be the most common form of cutaneous lymphoma after mycosis fungoides.Extranodal NK-T-cell lymphoma is a type of lymphoma.It is called "extranodal" to...
- Blastic NK-cell lymphomaBlastic NK-cell lymphomaBlastic NK-cell lymphoma is a cutaneous condition characterized by multiple, rapidly expanding plaques....
A WHO
Who
Who may refer to:* Who , an English-language pronoun* who , a Unix command* Who?, one of the Five Ws in journalism- Art and entertainment :* Who? , a 1958 novel by Algis Budrys...
-EORTC
EORTC
The European Organisation for Research and Treatment of Cancer or EORTC is an international non-profit organisation that develops, coordinates and stimulates cancer laboratory and clinical research in Europe...
classification has been developed.
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 :
- (1999) Denileukin diftitoxDenileukin diftitoxDenileukin diftitox is an antineoplastic agent, an engineered protein combining Interleukin-2 and Diphtheria toxin. This can bind to Interleukin-2 receptors and introduce the diphtheria toxin into cells that express those receptors, killing the cells...
(Ontak) - (2000) BexaroteneBexaroteneBexarotene is an oral antineoplastic agent indicated by the FDA for cutaneous T cell lymphoma. It has been used off-label for lung cancer, breast cancer, and Kaposi's sarcoma.-Mechanism:...
(Targretin) a retinoidRetinoidThe retinoids are a class of chemical compounds that are related chemically to vitamin A. Retinoids are used in medicine, primarily due to the way they regulate epithelial cell growth.... - (2006) VorinostatVorinostatVorinostat or suberoylanilide hydroxamic acid is a member of a larger class of compounds that inhibit histone deacetylases...
(Zolinza) a hydroxymate histone deacetylase (HDAC) inhibitor - (2009) RomidepsinRomidepsinRomidepsin , codenamed FK228 and FR901228, is an anticancer agent undergoing clinical trials as a treatment for cutaneous T-cell lymphoma , peripheral T-cell lymphoma, and a variety of other cancers...
(Istodax) a cyclic peptide histone deacetylase (HDAC) inhibitor
Other (off label) Treatments include:
- Topical and oral corticosteroidCorticosteroidCorticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...
s - BexaroteneBexaroteneBexarotene is an oral antineoplastic agent indicated by the FDA for cutaneous T cell lymphoma. It has been used off-label for lung cancer, breast cancer, and Kaposi's sarcoma.-Mechanism:...
(Targretin) gel and capsules - CarmustineCarmustineCarmustine or BCNU is a mustard gas-related β-chloro-nitrosourea compound used as an alkylating agent in chemotherapy...
(BCNU, a nitrosourea) - Mechlorethamine (Nitrogen Mustard)
- Phototherapy (Broad & Narrow Band UVB or PUVA)
- Local & Total Skin Electron Beam Radiation
- Conventional Radiation Therapy
- PhotopheresisPhotopheresisIn medicine, photopheresis or extracorporeal photopheresis is a form of apheresis and photodynamic therapy in which blood is treated with photoactivable drugs which are then activated with ultraviolet light....
- InterferonInterferonInterferons are proteins made and released by host cells in response to the presence of pathogens—such as viruses, bacteria, or parasites—or tumor cells. They allow communication between cells to trigger the protective defenses of the immune system that eradicate pathogens or tumors.IFNs belong to...
s - AlemtuzumabAlemtuzumabAlemtuzumab is a monoclonal antibody used in the treatment of chronic lymphocytic leukemia , cutaneous T-cell lymphoma and T-cell lymphoma...
(Campath-1H) - MethotrexateMethotrexateMethotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
- PentostatinPentostatinPentostatin is an anticancer chemotherapeutic drug.-Mechanism:It is classified as a purine analog, which is a type of antimetabolite....
and other purine analogues (FludarabineFludarabineFludarabine or fludarabine phosphate is a chemotherapy drug used in the treatment of hematological malignancies. It has been unofficially and casually referred to as "AIDS in a bottle" amongst healthcare professionals due to its significant immunosuppresive activity.-Indications:Fludarabine is...
, 2-deoxychloroadenosine) - Liposomal doxorubicinDoxorubicinDoxorubicin INN is a drug used in cancer chemotherapy. It is an anthracycline antibiotic, closely related to the natural product daunomycin, and like all anthracyclines, it works by intercalating DNA....
(Doxil) - Gemcitabine (Gemzar)
- CyclophosphamideCyclophosphamideCyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
- Bone marrow / stem cells
- Allogenic transplantation
- ForodesineForodesineForodesine is a transition-state analog inhibitor of purine nucleoside phosphorylase under development for the treatment of relapsed B-cell chronic lymphocytic leukemia...
(Inhibits Purine Nucleoside phosphorylase)
Other drugs are under investigation (for example panobinostat
Panobinostat
Panobinostat is an experimental drug developed by Novartis for the treatment of various cancers. It is a hydroxamic acid and acts as a non-selective HDAC inhibitor.-Clinical trials:...
).
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 cellLymphoproliferative disorders
Lymphoproliferative disorders refer to several conditions in which lymphocytes are produced in excessive quantities. They typically occur in patients who have compromised immune systems...
, 2% of cases are cutaneous T cell lymphomas.
There is some evidence of a relationship with human T-lymphotropic virus
Human T-lymphotropic virus
Human T-cell Lymphotropic Virus Type 1 , also called the Adult T-cell lymphoma virus type 1, a virus that has been seriously implicated in several kinds of diseases including HTLV-I-associated myelopathy, Strongyloides stercoralis hyper-infection, and a virus cancer link for leukemia...
.
External links
- Information on cutaneous T-cell lymphomas from Lymphoma Information Network
- cutaneous T-cell lymphoma from H. Lee Moffitt Cancer Center & Research InstituteH. Lee Moffitt Cancer Center & Research InstituteH. Lee Moffitt Cancer Center & Research Institute opened on October 26, 1986, on the Tampa campus of the University of South Florida. The current President/CEO is Dr. William S...
- The Cutaneous Lymphoma Foundation