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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]
The FDA approval was based on a clinical trial of 372 participants with relapsed mycosis fungoides or Sézary syndrome who received either mogamulizumab or a type of chemotherapy called vorinostat. The FDA granted the application for mogamulizumab priority review, breakthrough therapy, and orphan drug designations. The FDA granted the approval ...
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]
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.
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.
Lutzner cells were discovered by Marvin A. Lutzner, Lucien-Marie Pautrier, and Albert Sézary.These cells are described as the smaller forms of Sézary cells, or Sézary-Lutzner [1] cells, and the two variants are recognised as being morphologically different.
Boys with X-linked immunodeficiency syndrome are at a higher risk of mortality associated with Epstein–Barr virus infections, and are predisposed to develop a lymphoproliferative disorder or lymphoma. [citation needed] Children with common variable immunodeficiency (CVID) are also at a higher risk of developing a lymphoproliferative disorder.
Wildervanck syndrome; Williams syndrome; Williams–Campbell syndrome; Wilson's temperature syndrome; Wilson–Mikity syndrome; Wilson–Turner syndrome; Winchester syndrome; Winter-over syndrome; Wiskott–Aldrich syndrome; Wissler's syndrome; Withdrawal syndrome; Withering abalone syndrome; Wobbly hedgehog syndrome; Wolcott–Rallison syndrome