Langerhans cell histiocytosis mimicking acute dacryocystitis

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Abstract

Langerhans cell histiocytosis (LCH) is a myeloid neoplasm characterized by clonal neoplastic proliferation of Langerhans-type dendritic cells associated with an inflammatory infiltrate predominantly composed of lymphocytes and eosinophils. In this article, we present an unusual case of LCH with significant swelling in the left lacrimal sac region in a 3-year-old child, clinically mimicking acute dacryocystitis. Microscopically, it showed intense inflammatory infiltrate and histiocytes with irregular nuclei. The tumor cells were positive for S-100 protein, CD1a, and CD207 (langerin). Molecular study was positive for the V600E/E2/D mutation (EXON 15). This case emphasizes the importance of careful clinical, radiographic, and microscopic evaluation, as some neoplasms may mimic common benign lesions.

模仿急性泪囊炎的朗格汉斯细胞组织细胞增生症。
朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一种髓样肿瘤,其特征是朗格汉斯型树突状细胞的克隆性肿瘤性增生,并伴有主要由淋巴细胞和嗜酸性粒细胞组成的炎性浸润。本文介绍了一例不寻常的 LCH 病例,患者为一名 3 岁儿童,左侧泪囊区肿胀明显,临床症状类似急性泪囊炎。显微镜下,该病例表现为强烈的炎症浸润和核不规则的组织细胞。肿瘤细胞的 S-100 蛋白、CD1a 和 CD207(langerin)均呈阳性。分子研究显示,V600E/E2/D 突变(EXON 15)呈阳性。该病例强调了仔细进行临床、放射学和显微镜评估的重要性,因为有些肿瘤可能会模仿常见的良性病变。
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