颞骨朗格汉斯细胞组织细胞增生症1例并文献复习

One Case of Temporal Bone Langerhans Cell Histiocytosis and Literature Review

摘要: 朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis, LCH)是一种罕见的髓样前体细胞单克隆扩增引起的疾病，会导致肉芽肿性病变，其特征性表达CD1a/CD207。由于该病无特异性表现，临床医生对其怀疑度较低，因此可能会延误诊断。本篇文章介绍了一例孤立性朗格汉斯细胞组织细胞增多症的病例，该患儿有左耳后肿胀，术前初步诊断为左耳后脓肿，最终组织学和免疫组化诊断为朗格汉斯细胞组织细胞增多症。通过该病例报道并结合相关文献进行复习，以提高医务人员对该疾病的认识，为临床实践提供参考。

Abstract: Langerhans cell histiocytosis (LCH) is a rare disease caused by the monoclonal expansion of myeloid precursor cells, which leads to granulomatous lesions and is characteristically positive for CD1a/CD207 expression. Due to the lack of specific clinical manifestations of this disease, clinicians usually have a low degree of suspicion for it, which may result in delayed diagnosis. This article reports a case of isolated Langerhans cell histiocytosis in a child who presented with swelling behind the left ear. The preliminary preoperative diagnosis was an abscess behind the left ear, and the final diagnosis of LCH was confirmed by histopathological and immunohistochemical examinations. Through this case report and a review of relevant literature, this article aims to improve medical staff’s understanding of this disease and provide references for clinical practice.