Cutaneous T-cell Lymphoma: Symptoms and Treatments

Learn everything there is to know about T-cell cutaneous lymphoma and its potential symptoms, treatments and diagnosis.

What is skin lymphoma?


What is skin lymphoma?

Skin or cutaneous lymphoma is a type of cancer that starts in lymphocytes, which are a kind of leukocytes or white blood cells. These cells in our blood are part of the immune system of our body, and their purpose is to fight potential infections. In cutaneous lymphoma, lymphocytes develop certain abnormalities that deprogram them from their initial purpose and reprogram them to attack skin cells. The exact causes for this process in all types of lymphoma are unknown.

In general, lymphocytes are divided in type B and type T. More specifically, T-cell cutaneous lymphoma (CTCL) is a type of cancer that starts in T lymphocytes. There are multiple subtypes of Tcell cutaneous lymphoma according to the subtype of T lymphocyte that origins the lymphoma.

Although it is a rare type of cancer, it comprises 75% of all cases of cutaneous lymphoma. Of all the subtypes of T-cell cutaneous lymphoma, the most common are mycosis fungoides and Sézary syndrome. There are approximately 1,000 new cases of skin lymphoma each year in the United States. Approximately 16,000-20,000 Americans have the classic presentation of CTCL known as mycosis fungoides. One estimate placed the incidence of mycosis fungoides at one case per 1,000,000 people in the United States.

The incidence of CTCL increases significantly with age, with a median age at diagnosis in the mid-50s and a fourfold increase in incidence appreciated in patients older than age 70 years. CTCL is twice as common in men than women and more common in blacks than whites.

What causes cutaneous lymphoma?

One theory about the cause of CTCL is that it is related to some type of viral infection resulting in a chronically heightened immune state. Other theories include genetic changes and chemical exposure, but the actual causes remain unknown.

How to detect cutaneous lymphoma?

For the diagnosis of cutaneous lymphoma, it is important to pay attention to the signs and symptoms that may occur. Signs and symptoms of T-cell cutaneous lymphoma include:

Patches associated with a cutaneous lymphoma and that weren't there before may appear in the skin:

  • Round, raised patches that may be rough or scaly and cause itching.
  • White patches, or with less pigment than the surrounding skin.
  • Swellings or lumps in the skin that may crack or not.
  • Thickening of the skin in the palms of the hands and the soles of the feet. Generalized reddening and inflammation of the skin or erythroderma.

On the other hand, hair loss or enlargement of the lymph nodes can also be signs associated with Tcell cutaneous lymphoma.

Mycosis fungoides

Mycosis fungoides is the most common form of T-cell cutaneous lymphoma. It accounts for approximately 70 percent of cutaneous T-cell lymphomas. It affects men twice more often than women, with an annual incidence between 1/350,000 and 1/110,000 cases. In the United States, there are an estimated 3.6 cases per million people each year .Its clinical progression is usually slow, with visible patches or macules in the skin only in the early stages of the disease. As the disease progresses, these patches become plaques and/or tumors. Occasionally, these lesions may spread to areas of the skin that were previously healthy. In general, nearly three quarters of patients never develop generalized mycosis fungoides, remaining as a local disease. The prognosis depends on the type and extension of the condition, but it is usually favorable.

Sézary syndrome

The Sézary syndrome is considered an aggressive form of T-cell cutaneous lymphoma. It is mainly characterized by reddening and inflammation of the skin (or erythroderma) throughout the body, lymph node disorder (lymphadenopathy) and the presence of circulating atypical lymphocytes (Sézary cells). Sézary syndrome is a rare condition. It is the second most common form of cutaneous T-cell lymphoma after mycosis fungoides, accounting for approximately 3 to 5 percent of cases of cutaneous T-cell lymphoma.

It is more common in men, in many cases over 50 years old, and progresses fast. Besides erythroderma, patients usually suffer alopecia and general fatigue. Since it advances fast, based on initial presentation and evolution of each patient, the prognosis is usually bad.

How to prevent CTCL?

Because it is not definitively known what causes CTCL there are no known prevention measures.

How to treat CTCL?

While early stage CTCL is potentially curable, for most patients this is a chronic cancer. It can progress over many years and most patients live with CTCL for an extended period of time.

While early (patch/plaque) stage disease may respond well to topical therapy alone, more advanced cases may require a combination of topical, phototherapy, radiotherapy and systemic therapies to be successful. Advanced cases of CTCL may be treated with chemotherapy.

References

1. Bagot, Martine; Stadler, Rudolf. Chapter 119: Cutaneous Lymphoma. Access Medicine [Internet]. 2020 March. Available at: https://accessmedicine.mhmedical.com/content.aspx?sectionid=210435990&bookid=2570 &jumpsectionid=210436087&Resultclick=2

2. Geskin, Larisa. Chapter 103: Cutaneous T-Cell Lymphoma (Mycosis Fungoides and SéZary Syndrome). Access Medicine [Internet]. 2020 March. Available at: https://accessmedicine.mhmedical.com/content.aspx?sectionid=108075592&bookid=1581 &Resultclick=2

3. Lymphoma Research Foundation. Getting the facts. Cutaneous T-Cell Lymphoma. 2017 [Revised March 2020]. Available at: https://lymphoma.org/wp-content/uploads/2017/06/LRF_FACTSHEET_Cutaneous_TCell_Lymphoma_CTCL.pdf

4. OncoLink Team. All About Cutaneous T Cell Lymphoma (CTCL). OncoLink [Internet]. May 2018 [Revised March 2020]. Available at: https://www.oncolink.org/tipos-de-cancer/linfomas/linfoma-cutaneo-de-celulas-t/all-about-cutaneous-t-cell-lymphoma-ctcl

5. Genetic Home Reference. Sézary Syndrome. 2017 October [Revised March 2020]. Available at: https://ghr.nlm.nih.gov/condition/sezary-syndrome#statistics

6. Genetic Home Reference. Mycosis Fungoides. 2017 February [Revised March 2020]. Available at: https://ghr.nlm.nih.gov/condition/mycosis-fungoides#statistics

7. National organization of rare disorders. Cutaneous T-Cell Lymphomas. 2013 March [Revised March 2020]. Available at: https://rarediseases.org/rare-diseases/cutaneous-t-cell-lymphomas/