Carstensen H, Ornvold K
Børneafdeling GGK og børnepatologisk laboratorium, Rigshospitalet, København.
Ugeskr Laeger. 1993 Jun 7;155(23):1779-83.
Langerhans cell histiocytosis (LCH), formerly histiocytosis X, is a rare disorder of unknown aetiology and pathogenesis which is characterized by clinical heterogeneity and an unpredictable course. LCH is considered to be a reactive, proliferative disease. The pathognomonic cell in the lesion has been shown to be identical or very similar to the Langerhans cell. Immunophenotyping studies have shown the cell to be CD1a (OKT-6), S-100 protein, HLA-DR, and CD4 positive; ultrastructurally, the presence of intracytoplasmic Birbeck granules is the hallmark of the entity. The lesions may be localized or generalized. Due to the lack of an accepted classification system for the stage of the disease and its rarity, very few comparative therapy studies have been carried out. Recently, the Histiocyte Society has suggested diagnostic criteria for LCH, and established a program of initial evaluation of the patient in order to start controlled treatment trials. Traditionally, chemotherapy has been preferred with a trend over time towards a more conservative approach. The rate of sequelae is high and connected to a chronic course of recurrent multiosseous disease. Case fatality is strongly associated to development of organ dysfunction seen in disseminated disease, which is especially seen at low age (< 2 years) of onset.
朗格汉斯细胞组织细胞增多症（LCH），以前称为组织细胞增多症X，是一种病因和发病机制不明的罕见疾病，其特点是临床异质性和不可预测的病程。LCH被认为是一种反应性、增殖性疾病。病变中的特征性细胞已被证明与朗格汉斯细胞相同或非常相似。免疫表型研究表明该细胞CD1a（OKT-6）、S-100蛋白、HLA-DR和CD4呈阳性；在超微结构上，胞质内Birbeck颗粒的存在是该实体的标志。病变可以是局限性的或全身性的。由于缺乏被认可的疾病分期分类系统及其罕见性，很少进行比较性治疗研究。最近，组织细胞协会提出了LCH的诊断标准，并制定了患者初始评估方案，以便开展对照治疗试验。传统上，化疗一直是首选，随着时间的推移，有采用更保守方法的趋势。后遗症发生率很高，且与复发性多骨疾病的慢性病程有关。病死率与播散性疾病中出现的器官功能障碍密切相关，这在发病年龄较小（<2岁）时尤为常见。
