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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]
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.
Human infectious diseases may be characterized by their case fatality rate (CFR), the proportion of people diagnosed with a disease who die from it (cf. mortality rate).It should not be confused with the infection fatality rate (IFR), the estimated proportion of people infected by a disease-causing agent, including asymptomatic and undiagnosed infections, who die from the disease.
Sézary disease shown on a man exhibiting a rash (left) and a micrograph of a blood smear showing neoplastic Sézary cells (right). Signs and Symptoms Rough, red rash; Lymphadenopathy; Abnormal thickening of the palms of the hands and soles of the feet; Hair loss of balding; Nail trauma; Ectropion; Liver and Spleen become swollen; 4 Stages ...
T-PLL is an extremely rare aggressive disease, and patients are not expected to live normal lifespans. Before the recent introduction of better treatments, such as alemtuzumab, the median survival time was 7.5 months after diagnosis. [7] More recently, some patients have survived five years and more, although the median survival is still low.
While the incidence of Non-Hodgkin's lymphoma has plateaued, the rate of T-cell lymphomas has gradually increased over the past few years. However, due to the low frequency and lack of research performed on the disease, the number of cases is relatively underrepresented in comparison to other non-Hodgkin lymphomas. [3]
"Angelman syndrome shares symptoms and characteristics with other disorders including autism, cerebral palsy and Prader-Willi syndrome," read the Angelman Syndrome Foundation website. "People with ...
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