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Cutaneous T-cell lymphoma (CTCL) is a class of non-Hodgkin lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin lymphomas (which are generally B-cell-related), CTCL is caused by a mutation of T cells. The cancerous T cells in the body initially migrate to the skin, causing various lesions to appear.
This lymphoma commonly occurs in men (median age ~60 years) who present with advanced stage III or IV disease (~70% of cases) characterized by T cell infiltrations that cause prevalent lymph node swelling often accompanied by evidence of bone marrow, liver, spleen, and/or skin involvement. [62]
Romidepsin, vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma. [17] Mogamulizumab has been approved in Japan [18] and the United States. [19] The FDA has approved denileukin diftitox-cxdl (Lymphir) for the treatment of patients with relapsed/refractory cutaneous T-cell lymphoma (CTCL) after at least 1 prior ...
Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, [1] is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin. While the cause remains unclear, most cases are not hereditary.
Spread from the skin is unusual, and the prognosis is excellent [2]: 218 with a 5-year survival of over 97%. [2]: 314 The International Extranodal Lymphoma Study Group identified elevated LDH, more than two skin lesions, and nodular lesions as three prognostic factors, that are used to assess a cutaneous lymphoma international prognostic index (CLIPI), which is prognostic of disease-free status.
Lymphoma may present with certain nonspecific symptoms; if the symptoms are persistent, an evaluation to determine their cause, including possible lymphoma, should be undertaken. Lymphadenopathy [19] [20] or swelling of lymph nodes, is the primary presentation in lymphoma. It is generally painless.
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