郎格罕细胞组织细胞增生症(Langenhanscellhistiocytosis,LCH)原称组织细胞增生症,是一组原因未明的组织细胞增殖性疾患。传统分为三种临床类型即莱特勒西韦综合征,(Litterer-Siwe病,简称L-S病)汉-薛-柯综合征,(Hand-Schuller-Christian病,简称H-S-C病)及骨嗜酸肉芽肿(eosinphilicgranulomaofbone,EGB)病因未明,近年来研究发现多与体内免疫调节紊乱有关。
临床鉴别诊断包括骨纤、骨瘤、皮样囊肿、脑膜瘤、嗜酸性肉芽肿、朗格·汉斯组织细胞增生症、以及转移瘤。
Clinical differential diagnosis includes fibrous dysplasia, osteoma, dermoid cyst, meningioma, eosinophilic granuloma, Lagerhan cell histiocytosis, and metastatic disease.
经病理及免疫组化检查证实为朗格·汉斯细胞组织细胞增生症。
Skin biopsy and immunohistochemical study confirmed the diagnosis of Langerhans cell histiocytosis.
目的了解朗格·汉斯细胞组织细胞增生症(LCH)肺损害的临床情况。
Objective to understand the clinical manifestations of lung injury in Langerhans cell histocytosis (LCH).
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