Читать книгу The Peripheral T-Cell Lymphomas - Группа авторов - Страница 101

T‐cell prolymphocytic leukemia (T‐PLL) is a relatively aggressive and extremely rare disease., Although the name suggests “prolymphocyte,” the cell of origin is actually a post‐thymic T cell. T‐PLL accounts for approximately 2% of peripheral T‐cell lymphomas in adults [63, 64]. It has not been definitively reported in children or young adults. Sporadic T‐PLL mainly affects older adults with a mean age at presentation of 63–65 years [65, 66], and there is a slight male predominance with a male to female ratio of 1.33 : 1 [63]. Patients with ataxia telangiectasia have a greatly increased incidence of T‐PLL with a different epidemiologic profile [67]. In contrast to patients with sporadic T‐PLL, the median age of onset of T‐PLL in patients with ataxia telangiectasia is about 30 years, with some cases appearing in adolescence [68].