吉林大学学报(医学版) ›› 2016, Vol. 42 ›› Issue (05): 985-987.doi: 10.13481/j.1671-587x.20160528

• 临床医学 • 上一篇    下一篇

成人郎格汉斯细胞组织细胞增生症1例报告及文献复习

温泉, 王园园, 司鹤南, 田亚萍   

  1. 吉林大学第一医院皮肤性病科, 吉林 长春 130021
  • 收稿日期:2016-04-21 出版日期:2016-09-28 发布日期:2016-09-29
  • 通讯作者: 田亚萍,副教授,硕士研究生导师(Tel:0431-88782663,E-mail:yapingtian@163.com) E-mail:yapingtian@163.com
  • 作者简介:温泉(1972-),女,吉林省长春市人,主管护师,主要从事皮肤性病学方面的研究。
  • 基金资助:

    国家自然科学基金资助课题(30900591)

Adult Langerhans cell histiocytosis: A case report and literature review

WEN Quan, WANG Yuanyuan, SI Henan, TIAN Yaping   

  1. Department of Dermatology and Venerology, First Hospital, Jilin University, Changchun 130021, China
  • Received:2016-04-21 Online:2016-09-28 Published:2016-09-29

摘要:

目的:探讨郎格汉斯细胞组织细胞增生症(LCH)的临床和病理特征,为其诊治提供参考。方法:回顾性分析1例LCH患者的临床表现,结合皮肤组织病理检查、影像学检查及随访资料,总结本病例特点,并复习相关文献。结果:该例患者相继出现多囊肺、皮肤溃疡、尿崩和泌乳等典型症状。皮肤病理学检查发现真皮内密集分布的单个核细胞浸润,免疫组织化学染色CD1a(+)。随访7年,最终因心肺功能衰竭死亡。结论:LCH临床表现多种多样,确诊需要结合临床症状、病理和影像学等方法。成人LCH患者多系统重要器官受累提示预后不良。

关键词: 郎格汉斯细胞组织细胞增生症, CD1a, 尿崩症, 免疫组织化学

Abstract:

Objective: To study the clinical and pathological features of Langerhans cell histiocytosis(LCH), and to provide the reference for its diagnosis and treatment. Methods: The manifestation of one LCH patient was retrospectively analyzed. The features of the LCH patients were explored by analyzing the results of skin biopsy, radiological test and follow-up. The associated literatures were reviewed. Results: The patient presented the typical symptoms gradually, including polycystic lung, skin ulcer, diabetes insipidus,and lactation. The skin pathological findings showed the densely distributed mononuclear cell infiltration in dermis and the immunohistological staining result showed positive CD1a. The patient was follwed up for 7 years and died of heart and lung failure. Conclusion: LHC has various manifestations and should be confirmed by clinical features,pathological features and imaging examination. The adult patients with multisystem and vital organ involvement suggest the poor prognosis.

Key words: langerhans cell histiocytosis, CD1a, diabetes insipidus, immunohistochemistry

中图分类号: 

  • R733