Cutaneous Lymphoma

At Siteman Cancer Center, patients can be seen in our multidisciplinary cutaneous lymphoma clinic, which is one of the largest in the country. Our dermatologists, oncologists and radiation oncologists have long experience diagnosing and treating patients with these rare diseases.

Cutaneous Lymphoma is a subset of non-Hodgkin lymphoma in which white blood cells in the skin become malignant and can cause rash or tumors. Classification is based on white blood cell type: B-lymphocytes (B-cell) or T-lymphocytes (T-cell).

Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma (CTCL) is the most common type of cutaneous lymphoma, representing about 80 percent of cases. It typically presents with red, scaly patches or thickened plaques of skin that often mimic eczema or chronic dermatitis. Progression from limited skin involvement is variable and may be accompanied by tumor formation, ulceration and exfoliation, combined with itching and infections. Advanced stages are defined by involvement of lymph nodes, peripheral blood and internal organs.

Most CTCLs typically fall into the category of chronic lymphomas, which means they are treatable but not curable. The majority of patients have early-stage disease which is generally not life threatening.

Are there different types of T-cell lymphoma?

They are two main types of cutaneous T-cell lymphoma:

  • Mycosis fungoides: This is the most common form of T-cell lymphoma. Mycosis fungoides occurs when T-cells become cancerous. Patients can have disease in the blood and lymph nodes in addition to lymphoma in the skin.
  • Sézary Syndrome: This is a variant of mycosis fungoides in which there are a large number of T-cells in the blood (Sézary cells). Patients will have rash and can have involvement in lymph nodes.

Cutaneous B-cell lymphoma

Cutaneous B-cell lymphoma (CBCL) is a less common version of cutaneous lymphomas, making up about 20-25 percent of all cutaneous lymphomas.  CBCLs are B-cell non-Hodgkin lymphomas which start in skin-based B-cells. When a skin biopsy shows B-cell lymphoma, it is very important to make sure that the skin is the only organ involved and that it is not a systemic lymphoma presenting in the skin. The most common forms of CBCL are slow-growing variations that respond well to mild treatments.

How is cutaneous lymphoma diagnosed?

To achieve a definitive diagnosis and differentiate cutaneous lymphoma from other skin disorders, some of the tests that may be performed include:

  • Physical exam:Your doctor will examine your skin for patchy, scaly areas or thickened plaques of skin. You will also be examined for signs that disease has spread to your lymph nodes or other organs.
  • Skin biopsy: Your doctor may remove a skin sample to be examined under a microscope. Tests on the sample can reveal how the immune system is functioning and can identify gene mutations that may help in diagnosing cutaneous lymphoma.
  • TCR (T-cell receptor) gene rearrangement studies: This molecular test can be useful in diagnosis, staging and follow-up in cutaneous T-cell lymphoma.
  • Blood flow cytometry and immunohistochemistry: Your doctor may take a sample of cells and treat them with antibodies that stick to certain proteins on cells. For blood flow cytometry, a special machine is used to see if antibodies stuck to the cells. For immunohistochemistry, the cells are examined under a microscope. These innovative tests help diagnose and classify lymphoma, and evaluate the risk of recurrence.
  • Lymph node biopsy: If a skin biopsy doesn’t provide enough information, your doctor may perform a lymph node biopsy to either confirm a diagnosis or determine if cutaneous lymphoma has spread to the lymph nodes.

Standard treatments for cutaneous lymphoma

There are many treatments available for patients with cutaneous lymphoma. Determining which treatments are best for you depends on the extent of your cancer.

Skin-directed treatments

Surgery: While surgery is not typically used as a standalone treatment for cutaneous lymphoma, it may be useful in certain situations. Surgery may be used to biopsy a skin sample or lymph node, or it may be used to treat lymphomas when there are only one or a few skin lesions that can be completely removed.

Radiation therapy: The type of radiation most often used to treat cutaneous lymphoma is electron beam radiation. This type of radiation only penetrates as far as the skin, meaning that other organs and tissues are spared. Radiation can be very helpful in treating localized areas of lymphoma involvement.  If mycosis fungoides or Sézary syndrome cover a large area of skin, electron beam therapy may be given to the entire body (total skin electron beam therapy).

Phototherapy (UV light therapy): Phototherapy uses UV light to kill cancer cells in the skin. This treatment is particularly beneficial for patients whose lesions are thinner.

Other topical therapy: There are a variety of treatments that may be applied to the skin to treat cutaneous lymphoma. These include corticosteroids, chemotherapy drugs, retinoids or creams that stimulate an immune response.

Systemic treatments

Systemic (whole-body) treatments are most useful for advanced or fast-growing cutaneous lymphoma. A systemic treatment may be combined with a skin-directed treatment or with another systemic treatment.

Photopheresis: In this procedure, a patient’s blood is drawn into a special machine that separates out the lymphocytes (including the cancerous cells). The lymphocytes are treated with a light-sensitizing drug and UV light before being mixed back in with the rest of the blood. After this process, the blood is infused back into the patient.

Targeted therapies: Targeted therapies involve the use of drugs to target specific parts of lymphoma cells, or to boost the body’s immune system to attack lymphoma cells.

Systemic retinoids: Retinoids are drugs derived from vitamin A. Topical treatment may be used when only a few small skin lesions are present. For more widespread lymphomas, retinoids are often taken in pill form. 

Systemic chemotherapy: Systemic chemo is typically used for more advanced cases of lymphoma, when other treatment options are no longer working. It can also be helpful if the lymphoma has spread to the lymph nodes or other parts of the body.

High-dose chemotherapy with stem cell transplant (SCT): When standard treatments are no longer working, doctors may turn to stem cell transplants. SCT may involve transplanting the blood-forming stem cells of another person (allogeneic stem cell transplant) or the patient’s own stem cells (autologous stem cell transplant). SCT allows for higher doses of chemotherapy to be given because the transplant of blood-forming stem cells restores the bone marrow.

In treating cutaneous lymphomas, unlike most other cancers, physicians often use the same treatment repeatedly, such as light therapy and radiation. What worked once, often will work again. Patients with early-stage disease can often achieve long-lasting remissions with only topical therapies. In CTCL, since malignant T-cells are thought to spend the majority of their time in the skin and are dependent upon the skin for survival, therapies aimed at the skin are likely to be effective for a long time. For more in-depth information on the disease, staging and treatment, check the National Cancer Institute pages.