Clonal rearrangement of the T cell receptor beta gene in the circulating lymphocytes of erythrodermic follicular mucinosis.
نویسندگان
چکیده
Follicular mucinosis is a condition characterized by the abnormal accumulation of acidic mucopolysaccharides in hair follicles. It is classically described as occurring idiopathically in young persons and within the infiltrates of mycosis fungoides in older individuals. We report a 12-year-old girl who had erythrodermic follicular mucinosis, hypereosinophilia, circulating Sezary cells, and both immunophenotypic and genotypic evidence of T cell neoplasia. Erythrodermic follicular mucinosis may represent an unusual variant of the Sezary syndrome, which to date has not been described in children or adolescents.
منابع مشابه
Clonal Rearrangement of the T Cell Receptor $ Gene in the Circulating Lymphocytes of Erythrodermic Follicular
F OLLICULAR MUCINOSIS is a histologic process whose hallmark is the accumulation of acid mucopolysaccharides within hair follicle epithelium.’ Follicular mucinosis has been reported in four clinical settings.”7 In the first group of cases, follicular mucinosis occurs as slightly raised and boggy plaques on the face and scalp, usually of children, adolescents, or young adults. A second group has...
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متن کاملThe clonal nature of circulating Sezary cells.
To determine if circulating Sezary cells can be classified as reactive or neoplastic based on the ability to detect the presence or absence of clonal T-cell receptor beta chain (TCR-beta) gene rearrangements by Southern blot analysis, we evaluated the peripheral blood of 25 patients: 11 patients with Sezary syndrome (SS), 11 with benign inflammatory dermatoses (BID), and three normal controls. ...
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ورودعنوان ژورنال:
- Blood
دوره 71 5 شماره
صفحات -
تاریخ انتشار 1988