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血管免疫母细胞性T细胞淋巴瘤继发中毒性表皮坏死松解症

Toxic epidermal necrolysis secondary to angioimmunoblastic T-cell lymphoma.

作者信息

Jones Brad, Vun Yin, Sabah Muna, Egan Conleth A

机构信息

Department of Dermatology, Our Lady of Lourdes Hospital, Drogheda, Ireland.

出版信息

Australas J Dermatol. 2005 Aug;46(3):187-91. doi: 10.1111/j.1440-0960.2005.00177.x.

Abstract

A 67-year-old man presented with a history of lymphadenopathy, fevers and separate skin eruptions of erythrodermic spongiotic dermatitis initially and subsequent toxic epidermal necrolysis. Initial lymph node biopsies showed non-specific granulomatous changes, and skin biopsies and bone marrow aspirate were not diagnostic. His toxic epidermal necrolysis responded well to 3 days of intravenous immunoglobulin. The patient was discharged from hospital and reviewed regularly as an outpatient. Due to persisting lymphadenopathy, further lymph node biopsy led to the diagnosis of angioimmunoblastic T-cell lymphoma, a rare form of peripheral T-cell lymphoma with a poor prognosis. At the time of diagnosis his condition deteriorated rapidly and he died soon after.

摘要

一名67岁男性患者,最初表现为淋巴结病、发热以及先后出现的红皮病性海绵状皮炎皮肤疹和随后的中毒性表皮坏死松解症。最初的淋巴结活检显示非特异性肉芽肿性改变,皮肤活检和骨髓穿刺均未明确诊断。他的中毒性表皮坏死松解症对静脉注射免疫球蛋白治疗3天反应良好。患者出院后作为门诊病人定期复诊。由于淋巴结病持续存在,进一步的淋巴结活检确诊为血管免疫母细胞性T细胞淋巴瘤,这是一种预后较差的罕见外周T细胞淋巴瘤。确诊时他的病情迅速恶化,不久后死亡。

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