Cutaneous T Cell Lymphoma - Mycosis Fungoides - Sezary Syndrome

Overview and treatment for cutaneous t cell non-Hodgkin's lymphomas.

Cutaneous T Cell Lymphoma - Mycosis Fungoides - Sezary Syndrome

by Dr. C.H. Weaver M.D. Medically reviewed 10/2018

Cutaneous T-cell lymphoma (CTCL) is a general term for T-cell lymphomas that involve the skin. The disease affects men more often than women and usually occurs in people in their 50s and 60s. Symptoms can include dry skin, itching, a red rash, and CTCL can involve the blood, lymph nodes, and other internal organs. There are many subtypes of CTCLs including the following:

Mycosis Fungoides (MF) is the most common type of CTCL. The majority of patients with MF experience skin symptoms. Skin symptoms can appear as patches, plaques, tumors, or erythroderma (reddening of the skin). Patches are usually flat and scaly, and look like a rash; plaques are thicker, raised, and usually itchy. These lesions are often mistaken for eczema, psoriasis, or dermatitis. It is possible to have more than one type of lesion. Patients with erythrodermic MF have diffuse scaly red skin eruptions that can be very itchy. A skin biopsy is essential for diagnosis. This disease is usually indolent and develops slowly. Early stage MF might not progress to later stages at all in some patients or progress rapidly in others, with the cancer spreading to the lymph nodes, blood, and/or internal organs.

Sézary Syndrome is characterized by a generalized erythroderma and the presence of a lymphoma cells circulating in the blood. Extensive thin, red, itchy rashes typically appear on the skin and patches and tumors may appear. Severe itching and skin infections are common, and individuals may also experience changes in their nails, hair, or eyelids, or have enlarged lymph nodes.

CD30-positive lymphoproliferative disorders. Some of the rare CTCL subtypes are very aggressive. The skin symptoms and appearance, and kind of treatment used, varies depending on the subtypes of CTCL. MF and SS

Treatment of CTCL

The clinical stage is the primary factor for determining treatment however treatment is tailored for each patient, and may be adjusted frequently depending on the treatment response and side effects.

· For MF, treatment is either directed at the skin only for early stages of disease. Patients with advanced-stage MF require systemic therapy.

· Sézary syndrome always has lymphoma cells circulating in the blood therefore it requires systemic treatment and cannot usually be treated with skin- directed therapies alone.

Skin-Directed Therapies

These therapies include topical corticosteroids, chemotherapy, retinoids, or imiquimod, local or total skin radiation therapy, and ultraviolet light.

· Targretin (bexarotene gel)

· Valchor (mechlorethamine gel)

Systemic Treatment

Systemic therapy reaches lymphoma cells everywhere in the body and is used for advanced-stage MF, Sezary syndrome and in individuals with early stage disease in whom skin-directed therapies did not work.

· Acitretin (Soriatane)

· Liposomal doxorubicin

· Bexarotene capsules (Targretin)

· Methotrexate tablets

· Brentuximab vedotin (Adcetris)

· Pralatrexate (Folotyn)

· Extracorporeal photopheresis

· Romidepsin (Istodax)

· Gemcitabine (Gemzar)

· Vorinostat (Zolinza)

· Interferons (alpha or gamma)

· Extracorporeal photopheresis (ECP)

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