Trocme S D, Baker R H, Bartley G B, Henderson J W, Leiferman K M
Department of Ophthalmology, Mayo Clinic, Rochester, MN 55905.
Ophthalmology. 1991 Mar;98(3):353-6. doi: 10.1016/s0161-6420(91)32289-9.
Although eosinophils are prominent in orbital lesions of patients with histiocytosis X (Langerhans' cell histiocytosis), little is known of their pathogenic significance in the disease. To determine whether eosinophils degranulate and deposit toxic proteins in orbital histiocytosis X, the authors examined lesions by indirect immunofluorescence for localization of the core granule protein (major basic protein) outside of eosinophils. Four patients with histiocytosis X were studied: three with eosinophilic granuloma and one with Hand-Schüller-Christian disease. Tissue eosinophilia was prominent in all specimens; striking extracellular deposition of eosinophil major basic protein was noted in three patients, and focal deposition was present in the fourth patient. Orbital specimens obtained at autopsy from patients without orbital disease were studied as control specimens; no tissue eosinophilia or deposition of eosinophil major basic protein was observed. These findings indicate that eosinophils likely degranulate in lesions of orbital histiocytosis X and may participate in the pathogenesis of the disease.
尽管嗜酸性粒细胞在组织细胞增生症X（朗格汉斯细胞组织细胞增生症）患者的眼眶病变中很突出，但对其在该疾病中的致病意义知之甚少。为了确定嗜酸性粒细胞是否在眼眶组织细胞增生症X中脱颗粒并沉积毒性蛋白，作者通过间接免疫荧光检查病变，以确定核心颗粒蛋白（主要碱性蛋白）在嗜酸性粒细胞外的定位。研究了4例组织细胞增生症X患者：3例为嗜酸性肉芽肿，1例为汉-许-克病。所有标本中组织嗜酸性粒细胞增多均很明显；3例患者可见明显的嗜酸性粒细胞主要碱性蛋白细胞外沉积，第4例患者存在局灶性沉积。将无眼眶疾病患者尸检时获得的眼眶标本作为对照标本进行研究；未观察到组织嗜酸性粒细胞增多或嗜酸性粒细胞主要碱性蛋白沉积。这些发现表明，嗜酸性粒细胞可能在眼眶组织细胞增生症X的病变中脱颗粒，并可能参与该疾病的发病机制。
