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 tiredn ...

, which is a type of

cancer 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. Po ...

of the

immune system The immune system is a network of biological systems that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to bacteria, as well as Tumor immunology, cancer cells, Parasitic worm, parasitic ...

. Unlike most non-Hodgkin lymphomas (which are generally

B-cell B cells, also known as B lymphocytes, are a type 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 or inserted into the plasm ...

-related), CTCL is caused by a mutation of

T cell T cells (also known as T lymphocytes) are an important part 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 receptor (TCR) on their cell ...

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. Po ...

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 animal coverings, such as the arthropod exoskeleton, have different ...

, causing various

lesion A lesion is any damage or abnormal change in the tissue of an organism, usually caused by injury or diseases. The term ''Lesion'' is derived from the Latin meaning "injury". Lesions may occur in both plants and animals. Types There is no de ...

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 skin that 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, cracke ...

which can be very

itch An itch (also known as pruritus) is a sensation that causes a strong desire or reflex to scratch. Itches have resisted many attempts to be classified as any one type of sensory experience. Itches have many similarities to pain, and while both ...

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 lesio ...

or Sézary syndrome, the most common, though not the only types. Among the symptoms for the aforementioned types are: enlarged lymph nodes, an enlarged liver and spleen, and non-specific

dermatitis Dermatitis is a term used for different types of skin inflammation, 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 ...

Cause

The cause of CTCL remains largely unknown, but several external risk factors have been proposed as potential triggers and promoters of the disease. These include the use of

hydrochlorothiazide Hydrochlorothiazide, sold under the brand name Hydrodiuril among others, is a diuretic medication used to treat hypertension and swelling due to fluid build-up. Other uses include treating diabetes insipidus and renal tubular acidosis and t ...

diuretics, therapy-induced immunosuppression, and possible infections by a range of viral (e.g., HTLV-1,

HTLV-2 A virus closely related to HTLV-I, human T-lymphotropic virus 2 (HTLV-II) shares approximately 70% genomic homology (structural similarity) with HTLV-I. It was discovered by Robert Gallo and colleagues. HTLV-2 is prevalent in Africa and amon ...

HIV The human immunodeficiency viruses (HIV) are two species of '' Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the im ...

, Epstein-Barr virus, ''

Cytomegalovirus ''Cytomegalovirus'' (CMV) (from ''cyto-'' 'cell' via Greek - 'container' + 'big, megalo-' + -''virus'' via Latin 'poison') is a genus of viruses in the order '' Herpesvirales'', in the family '' Herpesviridae'', in the subfamily '' Betaherp ...

'',

HHV-6 Human herpesvirus 6 (HHV-6) is the common collective name for human herpesvirus 6A (HHV-6A) and human herpesvirus 6B (HHV-6B). These closely related viruses are two of the nine known herpesviruses that have humans as their primary host. HHV-6 ...

HHV-7 Human herpesvirus 7 (HHV-7) is one of nine known members of the ''Herpesviridae'' family that infects humans. HHV-7 is a member of ''Betaherpesvirinae'', a subfamily of the ''Herpesviridae'' that also includes HHV-6 and ''Cytomegalovirus'' (HHV ...

, HHV-8 (KSHV), and Polyomaviruses such as

Merkel cell polyomavirus Merkel cell polyomavirus (MCV or MCPyV) was first described in January 2008 in Pittsburgh, Pennsylvania. It was the first example of a human viral pathogen discovered using unbiased metagenomic next-generation sequencing with a technique called ...

) and bacterial or fungal pathogens (including ''

Staphylococcus aureus ''Staphylococcus aureus'' is a Gram-positive spherically shaped bacterium, a member of the Bacillota, and is a usual member of the microbiota of the body, frequently found in the upper respiratory tract and on the skin. It is often posi ...

'', ''

Mycobacterium leprae ''Mycobacterium leprae'' (also known as the leprosy bacillus or Hansen's bacillus) is one of the two species of bacteria that cause Hansen's disease (leprosy), a chronic but curable infectious disease that damages the peripheral nerves and ta ...

'', ''

Chlamydophila pneumoniae ''Chlamydia'' is a genus of pathogenic Gram-negative bacteria that are obligate intracellular parasites. ''Chlamydia'' infections are the most common bacterial sexually transmitted diseases in humans and are the leading cause of infectious blin ...

'', and

dermatophytes Dermatophyte (from Greek '' derma'' "skin" ( GEN ''dermatos'') and ''phyton'' "plant") is a common label for a group of fungus of '' Arthrodermataceae'' that commonly causes skin disease in animals and humans. Traditionally, these anamorphic ( ...

). The level of evidence varies among the different factors.

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 lesio ...

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

WHO The World Health Organization (WHO) is a specialized agency of the United Nations which coordinates responses to international public health issues and emergencies. It is headquartered in Geneva, Switzerland, and has 6 regional offices and 15 ...

- EORTC 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, sold under the brand name Ontak among others, is an anti-cancer medication used for the treatment of adults with T-cell lymphoma. It is an IL2-receptor-directed cytotoxin. It is an engineered protein combining interleukin-2 ...

(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 The retinoids are a class of chemical compounds that are natural derivatives of vitamin A or are chemically related to it. Synthetic retinoids are utilized in cosmetic formulations, clinical dermatology, and the treatment of some forms of cancer ...

* (2006)

Vorinostat Vorinostat (International Nonproprietary Name, rINN), also known as suberoylanilide hydroxamic acid (suberic acid, suberoyl+aniline, anilide+hydroxamic acid abbreviated as SAHA), is a member of a larger class of compounds that inhibit histone de ...

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

Romidepsin Romidepsin, sold under the brand name 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 ...

(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 name Narcan among others, is an opioid antagonist, a medication used to reverse or reduce the effects of opioids. For example, it is used to restore breathing after an opioid overdose. Effects begin within two ...

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 known as Black Americans and formerly also called Afro-Americans, are an American racial and ethnic group that consists of Americans who have total or partial ancestry from any of the Black racial groups of Africa. ...

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 primate T-lymphotropic viruses (PTLVs) are a group of retroviruses that infect primates, using their lymphocytes to reproduce. The ones that infect humans are known as human T-lymphotropic virus (HTLV), and the ones that infect Old World monk ...

(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