8例郎格汉斯细胞组织细胞增生症患儿Birbeck颗粒的超微结构观察

Ultrastructural features of Birbeck granules in 8 cases with Langerhans cell histiocytosis

目的探讨郎格汉斯细胞组织细胞增生症(LCH)病损区郎格汉斯细胞(LC)及其中Birbeck颗粒的超微结构,为研究Birbeck颗粒的来源、形态、空间结构提供依据。方法透射电镜观察8例儿童LCH患者病变区LC及Birbeck颗粒的超微结构。结果 8例LCH患儿病损区均可见典型LC,其中6例LC中可见Birbeck颗粒,大部分位于胞浆中,部分位于细胞膜内侧,与细胞膜相连续,细胞膜外也可见Birbeck颗粒样结构存在;典型Birbeck颗粒呈棒状、网球拍样,也可呈不规则形,周围常伴有圆形空泡样结构。结论 Birbeck颗粒可能起源于细胞膜,空间结构可能为不规则形,其形态具有多样性,不规则性,非典型Birbeck颗粒也具有诊断意义。

Objective To observe the ultrastructural features of Langerhans cells （LCs）, especially Birbeck granules in Langerhans cell histiocytosis （LCH） and provide the evidence to clarify its origin, function and stereoscopic structure of Birbeck granules. Methods Transmission electron microscope （TEM） was used to detect the ultrastructure of Birbeck granules in 8 patients with LCH. Results All 8 cases showed typical LCs, among which, 6 revealed typical rod- or tennis-racquet-like Birbeck granules. Some Birbeck granules were presented as irregular shapes accompanied with vacuole-like structure. Most Birbeck granules were located in cytoplasma, and some of them were close and merged to the cell membrane. Birbeck granule-like structure could also be observed outside of the cell membrane in one case with muhisystem LCH. Conclusions The ultrastructure of Birbeck granules was presented as diverse and irregular. Those atypical granules were useful for the diagnosis of LCH. Birbeck granules may arise from the cell membrane, and form irregular stereoscopic structure.