Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia 30322, USA.
Surv Ophthalmol. 2013 Jul-Aug;58(4):330-40. doi: 10.1016/j.survophthal.2012.09.004. Epub 2012 Dec 13.
Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. We describe the clinical and histologic spectrum of LCH of the orbit in our five cases. One patient exhibited multifocal unisystem disease; the other four had a localized process. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. Transmission electron microscopy demonstrated characteristic intracytoplasmic Birbeck granules. We review the different ophthalmic manifestations of LCH and treatment strategies. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH should included in the differential diagnosis in tumors of the ocular adnexae, especially in young children.
朗格汉斯细胞组织细胞增生症（LCH）是朗格汉斯细胞与炎症细胞（特别是嗜酸性粒细胞）混合增生的一种疾病，可表现为单系统（单发或多发灶）或多系统疾病。眼眶受累通常表现为单一病变，预后良好。我们描述了 5 例眼眶 LCH 的临床和组织学特征。1 例患者表现为多灶性单系统疾病，其余 4 例患者为局限性病变。典型的组织学特征包括数量不等的组织细胞，伴有不同程度的巨细胞形成和散在的嗜酸性粒细胞。通过 CD1a 和 S100 免疫组化证实了朗格汉斯细胞的存在。透射电镜显示特征性的细胞内 Birbeck 颗粒。我们回顾了 LCH 的不同眼部表现和治疗策略。由于 LCH 可能仅累及眼眶，因此治疗取决于器官受累的程度。在眼眶附属器肿瘤中，尤其是在儿童中，应将 LCH 纳入鉴别诊断。
