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伴有巨大淋巴结病的窦组织细胞增生症(罗萨伊-多夫曼病):三种不寻常表现

Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman: three unusual manifestations.

作者信息

Ratzinger Gudrun, Zelger Bettina, Höbling Walter, Mikuz Gregor, Zelger Bernhard W

机构信息

Department of Dermatology and Venerology, University of Innsbruck, Austria.

出版信息

Virchows Arch. 2003 Dec;443(6):797-800. doi: 10.1007/s00428-003-0912-1.

Abstract

We report on three exceptional courses of sinus histiocytosis Rosai-Dorfman. Patient one developed regional lymph-node disease subsequent to two independent malignancies in the right head and neck region. Patient two suffered from extensive extranodal disease with more than 100 mucocutaneous lesions over 17 years, which spontaneously resolved. Patient three showed exclusively extranodal disease, including bilateral conjunctival/scleral lesions, before he developed lung cancer. Our cases are unique for three reasons: the association of the disease with solid malignancies in two cases, the extent and persistence of exclusively extranodal disease in one patient and the appearance of thus far undescribed conjunctival/scleral lesions.

摘要

我们报告了三例罕见的罗萨伊-多夫曼鼻窦组织细胞增生症病程。病例一在右头颈部先后发生两处独立恶性肿瘤后出现区域淋巴结疾病。病例二患有广泛的结外疾病,17年间出现100多处黏膜皮肤病变,这些病变自行消退。病例三在患肺癌之前仅表现为结外疾病,包括双侧结膜/巩膜病变。我们的病例在三个方面具有独特性:两例中该疾病与实体恶性肿瘤相关;一例患者结外疾病的范围和持续性;以及出现了迄今未描述的结膜/巩膜病变。

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