Veyssier-Belot C, Callot V
Service de médecine interne, Hôtel-Dieu, Paris, France.
Rev Med Interne. 1996;17(11):911-23. doi: 10.1016/0248-8663(96)88121-5.
Histiocytic disorders are a group of heterogeneous diseases. A logical classification can be based on the type of proliferating cell, either monocyte-macrophage or Langerhans/dendritic cell, and depends whether the proliferating cells are "reactive" or malignant. The classification now mainly depends on the histological examination. Regarding Langerhans cell histiocytosis (Hand-Schüller-Christian disease, Letterer-Siwe disease and eosinophilic granuloma), the diagnosis suspected on various clinical signs, is confirmed with histological examination showing infiltration with CD1 positive histiocytes disclosing intracytoplasmic Birbeck granules at electron microscopic examination. The prognosis depends on the patient's age at onset and the extension of the disease. Treatment is based on chemotherapy and corticotherapy.
组织细胞疾病是一组异质性疾病。合理的分类可基于增殖细胞的类型,即单核细胞 - 巨噬细胞或朗格汉斯/树突状细胞,并且取决于增殖细胞是“反应性的”还是恶性的。目前的分类主要依赖于组织学检查。关于朗格汉斯细胞组织细胞增多症(汉 - 许 - 克病、勒 - 雪病和嗜酸性肉芽肿),根据各种临床体征怀疑诊断,通过组织学检查证实,显示CD1阳性组织细胞浸润,在电子显微镜检查时可见胞浆内的伯贝克颗粒。预后取决于患者发病时的年龄和疾病的范围。治疗基于化疗和皮质激素治疗。