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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]
It is rare for mycosis fungoides to appear before age 20; the average age of onset is between 45 and 55 years of age for people with patch and plaque disease only, but is over 60 for people who present with tumours, erythroderma (red skin) or a leukemic form (Sézary syndrome). Mycosis fungoides is more common in males than in females with ...
The presentation depends if it is mycosis fungoides or Sézary syndrome, the most common, though not the only types. Among the symptoms for the aforementioned types are: enlarged lymph nodes, an enlarged liver and spleen, and non-specific dermatitis. [1]
Mycosis fungoides/Sézary syndrome; Most common cutaneous lymphoid malignancy Usually small lymphoid cells with convoluted nuclei that often infiltrate the epidermis, creating Pautrier microabscesseses CD4 5-year survival 75% [38]
An accumulation of Lutzner cells in the layers of the skin can cause cutaneous T-cell lymphoma. Cutaneous cell lymphoma is the second most common form of non-Hodgkin's lymphoma. [11] Two forms of cutaneous T-cell lymphoma associated with abnormal T-lymphocytes or Lutzner cells are Mycosis Fungoides and Sézary Syndrome. [12]
It is a form of cutaneous T-cell lymphoma [2] and a variant of mycosis fungoides. [3] See also. List of cutaneous conditions; References
Fungi that cause infections in people include yeasts, molds and fungi that are able to exist as both a mold and yeast. [3] The yeast Candida albicans can live in people without producing symptoms, and is able to cause both superficial mild candidiasis in healthy people, such as oral thrush or vaginal yeast infection , and severe systemic ...
Mycosis fungoides; Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma; Nonspecific cutaneous conditions associated with leukemia (leukemid) Pagetoid reticulosis (acral mycoses fungoides, localized epidermotropic reticulosis, mycosis fungoides palmaris et plantaris, unilesional mycosis fungoides, Woringer–Kolopp disease)