Cutaneous T-cell lymphoma (CTCL) is a class of

non-Hodgkin lymphoma Non-Hodgkin lymphoma (NHL), also known as non-Hodgkin's lymphoma, is a group of blood cancers that includes all types of lymphomas except Hodgkin lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredness. ...

, which is a type of

cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...

of the

immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splinte ...

. Unlike most non-Hodgkin lymphomas (which are generally

B-cell B cells, also known as B lymphocytes, are a type of white blood cell of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted o ...

-related), CTCL is caused by a mutation of

T cell A T cell is a type of lymphocyte. T cells are one of the important white blood cells of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes by the presence of a T-cell r ...

s. The

cancerous Cancer is a group of diseases involving Cell growth#Disorders, abnormal cell growth with the potential to Invasion (cancer), invade or Metastasis, spread to other parts of the body. These contrast with benign tumors, which do not spread. Poss ...

T cells in the body initially migrate to the

skin Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other cuticle, animal coverings, such as the arthropod exoskeleton, have diffe ...

, causing various

lesion A lesion is any damage or abnormal change in the tissue of an organism, usually caused by disease or trauma. ''Lesion'' is derived from the Latin "injury". Lesions may occur in plants as well as animals. Types There is no designated classifi ...

s to appear. These lesions change shape as the disease progresses, typically beginning as what appears to be a

rash A rash is a change of the human 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, cr ...

which can be very

itch Itch (also known as pruritus) is a sensation that causes the desire or reflex to scratch. Itch has resisted many attempts to be classified as any one type of sensory experience. Itch has many similarities to pain, and while both are unpleasant ...

y and eventually forming plaques and

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

s before spreading to other parts of the body.

Signs and symptoms

The presentation depends if it is

mycosis fungoides Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesion ...

or Sézary syndrome, the most common, though not the only types. Among the symptoms for the aforementioned types are:

enlarged lymph nodes Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In cl ...

, an enlarged liver and spleen, and non-specific

dermatitis Dermatitis is inflammation of the skin, typically characterized by itchiness, redness and a rash. In cases of short duration, there may be small blisters, while in long-term cases the skin may become thickened. The area of skin involved can v ...

Cause

The cause of CTCL is unknown.

Diagnosis

A point-based algorithm for the diagnosis for early forms of cutaneous T-cell lymphoma was proposed by the International Society for Cutaneous Lymphomas in 2005.

Classification

Cutaneous T-cell lymphoma may be divided into the several subtypes.

Mycosis fungoides Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesion ...

is the most common form of CTCL and is responsible for half of all cases. A

WHO Who or WHO may refer to: * Who (pronoun), an interrogative or relative pronoun * Who?, one of the Five Ws in journalism * World Health Organization Arts and entertainment Fictional characters * Who, a creature in the Dr. Seuss book '' Horton He ...

EORTC The European Organisation for Research and Treatment of Cancer (EORTC) is a unique pan-European non-profit clinical cancer research organisation established in 1962 operating as an international association under Belgium law. It develops, conduc ...

classification has been developed.

Treatment

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 include the following: * (1999)

Denileukin diftitox Denileukin diftitox (trade name Ontak) was an antineoplastic agent, an engineered protein combining interleukin-2 and diphtheria toxin. Denileukin diftitox could bind to interleukin-2 receptors and introduce the diphtheria toxin into cells that ...

(Ontak) * (2000)

Bexarotene Bexarotene, sold under the brand Targretin, is an antineoplastic (anti-cancer) agent used for the treatment of cutaneous T cell lymphoma (CTCL). It is a third-generation retinoid. It was approved by the U.S. Food and Drug Administration (FDA) ...

(Targretin) a retinoid * (2006)

Vorinostat Vorinostat (rINN) also known as Suberoylanilide hydroxamic acid ( suberoyl+anilide+ hydroxamic acid abbreviated as SAHA) is a member of a larger class of compounds that inhibit histone deacetylases (HDAC). Histone deacetylase inhibitors (HDI) ...

(Zolinza) a hydroxymate histone deacetylase (HDAC) inhibitor * (2009)

Romidepsin Romidepsin, also known as Istodax, is an anticancer agent used in cutaneous T-cell lymphoma (CTCL) and other peripheral T-cell lymphomas (PTCLs). Romidepsin is a natural product obtained from the bacterium ''Chromobacterium violaceum'', and wo ...

(Istodax) a cyclic peptide histone deacetylase (HDAC) inhibitor * (2018) Poteligeo (mogamulizumab-kpkc) Histone deacetylase (HDAC) inhibitors are shown to have antiproliferative and cytotoxic properties against CTCL.Other (off label) treatments include: In 2010, the U.S. Food and Drug Administration granted orphan drug designation for

naloxone Naloxone, sold under the brand names Narcan (4 mg) and Kloxxado (8 mg) among others, is a medication used to reverse or reduce the effects of opioids. It is commonly used to counter decreased breathing in opioid overdose. Effects begin within ...

lotion as a treatment for pruritus in cutaneous T-cell lymphoma to a pharmaceutical company called Elorac.Elorac, Inc. Announces Orphan Drug Designation for Novel Topical Treatment for Pruritus in Cutaneous T-cell Lymphoma (CTCL)
website

Epidemiology

Of all cancers involving lymphocytes, 2% of cases are cutaneous T cell lymphomas. CTCL is more common in men and in

African-American African Americans (also referred to as Black Americans and Afro-Americans) are an Race and ethnicity in the United States, ethnic group consisting of Americans with partial or total ancestry from sub-Saharan Africa. The term "African American ...

people. The incidence of CTCL in men is 1.6 times higher than in women. There is some evidence of a relationship with

human T-lymphotropic virus The human T-lymphotropic virus, human T-cell lymphotropic virus, or human T-cell leukemia-lymphoma virus (HTLV) family of viruses are a group of human retroviruses that are known to cause a type of cancer called adult T-cell leukemia/lymphoma and ...

(HTLV) with the adult T-cell leukemia/lymphoma subtype. No definitive link between any viral infection or environmental factor has been definitely shown with other CTCL subtypes.

References

External links

* {{DEFAULTSORT:Cutaneous T-Cell Lymphoma Lymphoid-related cutaneous conditions Lymphoma Rare cancers