成人郎格汉斯细胞组织细胞增生症1例报告及文献复习

摘要

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.%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。