Cutaneous T-cell lymphoma
Cutaneous T-cell lymphoma (CTCL) is a group of lymphoproliferative disorders characterized by localization of neoplastic T lymphocytes to the skin. Collectively, CTCL is classified as a type of non-Hodgkin lymphoma (NHL). The two most common types are mycosis fungoides and Sézary syndrome. Mycosis fungoides is the most common type of CTCL. The disease looks different in each patient, with skin symptoms that can appear as patches, plaques, or tumors. Patches are usually flat, possibly scaly, and look like a rash; plaques are thicker, raised, usually itchy lesions that are often mistaken for eczema, psoriasis, or dermatitis; and tumors are raised bumps, which may or may not ulcerate. Sezary syndrome is an advanced, variant form of mycosis fungoides, which is characterized by the presence of lymphoma cells in the blood. Extensive thin, red, itchy rashes usually cover over 80 percent of the body. Of all primary cutaneous lymphomas, 65% are of the T-cell type. The most common immunophenotype is CD4 positive. There is no common pathophysiology for these diseases, as the term cutaneous T-cell lymphoma encompasses a wide variety of disorders.
Treatment selection for 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 either directed at the skin or the entire body (systemic). Because Sézary syndrome is chronic and systemic (affecting the entire body), it is usually not treated with skin-directed therapies alone. Treatments may be prescribed alone or in combination to achieve the best long-term benefit. Many patients live normal lives while they treat their disease, and some are able to remain in remission for long periods of time. 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), topical chemotherapy, local radiation, methotrexate, photopheresis, ultraviolet light (phototherapy).
Many treatments at various stages of drug development are currently being tested in clinical trials and for various stages of CTCL, including everolimus (Afinitor), lenalidomide (Revlimid), brentuximab vedotin (Adcetris), panobinostat, forodesine, APO866, KW0761, and others. It is critical to remember that today's scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with LRF or with their physician for any treatment updates that may have recently emerged.
Primary cutaneous lymphomas have an estimated annual incidence of 1.0–1.5/100,000 and are the second most common group of extranodal lymphomas.