Cutaneous T-Cell Lymphoma Symptoms and Treatment

Cutaneous T-cell lymphoma (CTCL) includes several types of skin lymphoma—the most common are mycosis fungoides and Sezary syndrome. Most of the clinical features of CTCL involve skin lesions.

Lymphoma is cancer that arises from T-cells, which are a type of lymphocyte (white blood cell, WBC). Lymphoma typically involves the lymph nodes. A key characteristic of CTCL is that the cancer cells also migrate to the skin.

CTCL represents a rare type of Non-Hodgkin Lymphoma (NHL). CTCL accounts for only about 1 in 40 newly diagnosed NHL patients.

Doctor holding digital tablet, talking to patient undergoing medical treatment in hospital
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Risk Factors

CTCL affects men more commonly than women, and it's usually diagnosed in people who are in their fifties or sixties. Children are rarely affected. There has been a striking increase in the number of individuals affected by skin lymphoma over the last 3 decades.

Not much is known about the cause of CTCL. Unlike some other types of lymphomas, there is no association with known viruses. Research is underway to determine what causes CTCL.

Symptoms

The first symptoms of skin lymphoma include dry or scaly skin, red rashes, and itching. The rashes are more common in areas that are usually covered with clothes.

If you have CTCL, you may first notice red or dark patches on your skin. These symptoms are not specific for lymphoma, so it's common for people to be treated for more common skin conditions before lymphoma is suspected.

As the disease progresses, the red patches may become raised. These elevated patches are described as plaques. Plaques may eventually turn into nodular or bumpy tumors. In advanced disease, ulcers can develop on top of these lesions.

Most people with CTCL only have skin involvement, but the condition can also spread to the lymph nodes or other organs. About 10 percent of late-stage cases progress to develop serious complications.

Diagnosis

A skin biopsy is needed for a definitive diagnosis of CTCL. The biopsy sample is examined under a microscope, and lymphoma cells may be visualized if a portion of the cancer was sampled.

A number of other tests, including tests for lymphoma markers (immunohistochemistry) and genetic tests, can help define the specific type of lymphoma.

CT scans or other imaging tests may be done to determine the extent of the disease.

Treatment

Treatment of skin lymphoma is quite different from the treatment of other lymphomas. Your treatment strategy will depend on the extent of skin involvement, the type of skin lesions, and the involvement of nodes or other body organs.

Many types of treatment are used:

  • Chemotherapy agents applied to the skin
  • Total skin electron beam therapy (a type of radiation treatment)
  • Psoralen and ultraviolet A rays
  • Ultraviolet B rays
  • Bexarotene (both as a gel as well as tablets)
  • Denileukin Difitox
  • Interferon alpha
  • Chemotherapy by injections or pills

Most recently, the U.S. Food and Drug Administration (FDA) approved mechlorethamine gel (Valchlor) for topical skin treatment of mycosis fungoides, the most common type of CTCL. Before its approval, mechlorethamine was only approved for intravenous (IV, in a vein) treatment.

A Word From Verywell

A European expert consensus group met and published their recommendations on treatment for various different kinds and cases of CTCL, noting the skin-directed therapies are still the most appropriate option for early-stage mycosis fungoides and that most patients with mycosis fungoides can look forward to a normal life expectancy. Unfortunately, the prognosis is still not as good for patients with advanced disease, they noted. Although, for a highly selected subset of patients, prolonged survival can be achieved with allogeneic stem cell transplantation (alloSCT).

Still, a huge amount of progress has been made in the last decade in terms of the understanding of how mycosis fungicides and Sezary syndrome develops, so there is hope that this will lead to treatment advances as well.

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Article Sources
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  1. Cutaneous lymphoma foundation. What is cutaneous lymphoma?

Additional Reading
  • Trautinger F, Eder J, Assaf C, et al. European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2017. Eur J Cancer. 2017;77:57-74.