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Sézary disease, or Sézary syndrome, [1] is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary. [2] The affected T cells, known as Sézary's cells or Lutzner cells, have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy. [3] [4]
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.
The nuclei are usually round to oval in shape, with occasional patients having cells with a more irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome. [3] A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases. [3]
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.
Disease Type Treatment stage [clarification needed] CFR Notes Reference(s) Transmissible spongiform encephalopathy: Prion: No treatment and no cure [1]: 100% [2]: Includes Creutzfeldt–Jakob disease and all its variants, fatal insomnia, kuru, Gerstmann–Sträussler–Scheinker syndrome, Variably protease-sensitive prionopathy and others.
Sézary syndrome Smoldering adult T cell leukemia/lymphoma Hematologic disorders that may resemble CLL in their clinical presentation, behavior, and microscopic appearance include mantle cell lymphoma, marginal zone lymphoma, B cell prolymphocytic leukemia, and lymphoplasmacytic lymphoma.
Celine Dion has given her first TV interview since her diagnosis of Stiff Person Syndrome (SPS) in 2022, detailing the toll it’s taken on her life in NBC News’ hourlong special “Celine’s ...
The hemophagocytic syndrome (HPS) [ edit ] Hemophagocytic syndrome has been associated with most T-cell lymphoma subtypes, and is commonly characterized by fevers , reduction of lymphocytes numbers, enlarged liver or spleen, and liver dysfunction . [ 2 ]