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Hyper-Ige Syndrome with Epstein-Barr Virus Associated Extranodal NK/T Cell Lymphoma of Skin

IgE過高症候群合併EB病毒有關皮膚NK/T淋巴瘤

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摘要


於1966年,Davis第一個描述有關IgE過高症候群,稱為Job氏症候群。有報告指出這會增加惡性腫瘤的機率。我們提出一位18歲年輕女性,在臉及右前臂上出現數顆紅色丘疹和有潰爛的結節。病患從童年起就因反覆性肺炎和泌尿道感染而需入院治療。皮膚切片顯示非典型的淋巴球浸潤於血管周圍並且造成破壞。利用原位雜交方式發現EB病毒感染的淋巴球細胞。這表示IgE過高症候群合併EB病毒感染引發皮膚NK/T淋巴瘤。

並列摘要


Hyper-IgE syndrome, also known as Job's syndrome, is a rare primary immunodeficiency that was first described in 1966. This syndrome is associated with an increased risk for malignancies. Here, we report an 18-year-old woman who was frequently hospitalized since childhood because of recurrent pneumonia and urinary tract infection. Multiple erythematous papules and nodules with ulceration were found on her face and right forearm. A skin biopsy showed angiocentric and angiodestructive atypical lymphoid infiltration. In situ hybridization revealed latent Epstein-Barr virus-infected lymphoid cells. Accordingly, this indicates that Epstein-Barr virus infection may have induced the extranodal natural killer/T cell lymphoma in this patient with hyper-IgE syndrome.

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