Cutaneous T-cell lymphoma is a blanket term for a group of blood cancers that target white blood cells and produce rashes on the skin. They are linked together because they all start in the white blood cells known as T cells.
The two most common types of cutaneous T-cell lymphoma are myocosis fungoides and Sezary syndrome.
Although CTCLs can occur at any age, the risk increases with age. Most people receive a diagnosis between the ages of 50 and 60, and males are twice as likely as females to develop CTCL.
This article will explain what cutaneous T-cell lymphoma is, before describing the symptoms, outlook, diagnosis, and treatment options of its most common types.
White blood cells, or lymphocytes, play an important role in fighting infections and travel all around the body in the lymphatic system. CTCLs occur when something disrupts the production of T cells, a large percentage of which are in the skin, and the cells become damaged. These damaged cells continue to multiply at an uncontrolled rate and build up in the skin.
The two most common types of CTCL are mycosis fungoides (MF) and Sezary syndrome (SS). Together, these make up about 75% of CTCL diagnoses. MF is the most common, and is a slow-growing, or indolent form that can remain just as a rash for years. SS tends to be more aggressive and spreads more quickly beyond the skin into the blood, lymph nodes, and sometimes organs.
Other, rarer types include:
- lymphomatoid papulosis
- pagetoid reticulosis, which is also known as Woringer-Kolopp disease
- primary cutaneous anaplastic large cell lymphomas
- subcutaneous panniculitic T-cell lymphoma
The symptoms of CTCL mainly affect the skin.
Damaged T cells build up in the skin, forming dry patches and skin rashes. Sometimes, so many damaged cells build up that they form raised patches, or plaques. These skin rashes can be extremely itchy.
With MF, the rash usually develops on an area of skin that is not often exposed to sunlight, such as the buttocks, top of the thigh, feet, or chest area. As this is a slow-growing cancer, it can remain at this stage for years or decades. If it does spread, a person may develop light spots on their skin, raised plaques, and tumors.
Rashes with SS tend to be widespread and peel. SS rashes are typically intensely itchy and can cause flushed skin.
Other symptoms of SS include:
- hair loss
- nail disorders
- thickened skin on the palms of the hands and soles of the feet
- changes to the eyelids, such as drooping of the lower lid
A person should see a doctor if they notice any of these symptoms.
In about 10% of people with MF, the cancerous cells spread, and may affect major organs in the body, including the liver, spleen, or gastrointestinal system.
SS is more aggressive than MF and spreads more quickly.
Symptoms will vary depending on where the cells have spread. This is why it is vital to contact a doctor if a person develops a rash that could be due to CTCL.
Not all symptoms of CTCL are physical.
If the symptoms of CTCL are negatively impacting a person’s mental health, they should talk to a doctor. Researchers are exploring ways to incorporate this into treatment plans.
While there is no cure for CTCL, treatment can help people live without symptoms for many years.
MF is an indolent form of cancer and doctors diagnose it at an early stage about 70% of the time.
SS is a more aggressive cancer and spreads rapidly. This means the outlook varies more dramatically from person to person, depending on where the cancer has spread. Researchers have estimated that the median survival for SS is 2–4 years from diagnosis. However, survival is improving as researchers discover new forms of treatment.
The outlook for treatment depends on how far advanced the condition is when a person has received a diagnosis, and whether or not it has spread. Doctors use the same four-point
- T for tumor: How much of the skin is affected by the lymphoma?
- N for nodes: How much of the lymphoma is in the the lymph nodes?
- M for metastasis: Has the lymphoma spread to other organs?
- B for blood: Are there lymphoma cells in the blood?
Doctors diagnose CTCL by thoroughly examining the skin, then running an array of tests, including:
Treatment plans for CTCL can differ depending on what symptoms a person has, their general health, and the stage of the cancer.
Some treatments target the skin and are called
Skin-directed treatments include creams or lotions that are applied topically. These may contain steroids, retinoids, or chemotherapy drugs.
Ultraviolet light treatments, including Psoralen and ultraviolet A (PUVA) and narrowband ultraviolet B can also be effective. Trials have shown an
If the CTCL has spread beyond the skin, people may need whole-body, or
- oral retinoids, such as Targretin (bexarotene)
- biologics or immunotherapy
- extracorporeal photopheresis
- stem cell transplants
Doctors may prescribe any one of these treatments on their own or in combination.
CTCL is an umbrella term for a group of cancers that target the white blood cells. This condition can cause itchy or painful rashes, among other symptoms. The two most common types of cutaneous T-cell lymphoma are myocosis fungoides and Sezary syndrome.
Treating CTCLs depends on how far the cancer has spread and if it has affected any other organs. CLCLs are not curable, but treatment can slow their progress and improve a person’s quality of life.