Cutaneous T-Cell Lymphoma (CTCL)

What It Is:

Cutaneous T-cell lymphoma (CTCL), is primarily a disease of the skin. CTCL may also involve the blood, lymph nodes, and other internal organs. Symptoms include:

  • Dry skin
  • Itchy skin
  • Rash
  • Enlarged lymph nodes 

Most patients with CTCL experience only skin symptoms with no severe complications. However, a small portion of patients (approximately 10%) progress to advanced stages in which the disease spreads to lymph nodes and internal organs.

What it looks like:

CTCL describes several different disorders with varying symptoms, outcomes, and treatment options. Diagnosis of CTCL requires a medical history, physical exam, and skin biopsy. A physician will examine lymph nodes, and may order various blood tests. Advanced CTCL may require additional imaging tests to determine if it has spread to the lymph nodes or rarely, other organs. These tests include a computerized tomography (CT) scan, a positron emission tomography (PET) scan, and/or a magnetic resonance imaging (MRI) scan.

The two most common types are mycosis fungoides and Sézary syndrome:

Mycosis fungoides is the most common type of CTCL. This disease is different for each person affected, but primarily involves symptoms on the skin which can appear as:

  • Patches- Usually flat, scaly, look like a rash
  • Plaques- Thick, raised, usually itchy lesions that are often mistaken for eczema, psoriasis, or dermatitis
  • Tumors- Raised bumps, may or may not be ulcerated

It is possible to have more than one type of lesion. Mycosis fungoides is difficult to diagnose in the early stages because the symptoms and skin biopsy findings may look like other skin conditions.

Sezary syndrome is an advanced form of mycosis fungoides. It is characterized by the presence of lymphoma cells in the blood. Thin, red, itchy rashes usually cover over 80 percent of the body. In some patients, patches and tumors appear. Patients may also experience changes in the nails, hair, or eyelids, or have enlarged lymph nodes.

How We Treat it:

Treating CTCL depends on the extent of skin involvement, the type of skin lesion, and whether the cancer has spread to the lymph nodes or other internal organs.

For mycosis fungoides, treatment is usually focused on the skin. Skin-directed therapies are useful for patch and limited plaque disease and include topical treatments such as corticosteroids, retinoids, or imiquimod (which activates immune cells), mechlorethamine gel (Valchlor), topical chemotherapy, local radiation, methotrexate, photopheresis, or ultraviolet light (phototherapy).

Because Sézary syndrome affects the entire body (not just skin), it is usually not treated with skin-directed therapies alone. Multiple treatments may be prescribed in combination to treat the entire disease. Common treatments include radiation, chemotherapy, and/or therapies such as:

  • Bortezomib (Velcade)
  • Denileukin diftitox (Ontak)
  • Pralatrexate (Folotyn)
  • Romidepsin (Istodax)
  • Vorinostat (Zolinza)

Combination chemotherapy regimens are usually only used when patients have not responded well to several single-agent therapies.

(Source: Lymphoma Research Foundation)