Lacronique J, Basset F, Hance A
Service de Pneumologie, Hopital Cochin, Paris.
Rev Pneumol Clin. 1993;49(6):299-309.
Langerhans' cell granulomatosis, formerly known as histiocytosis X include several granulomatosis of unknown aetiology. The original pathological finding is a Langerhans' cell granuloma. Recent progress has improved our understanding of the pathogenesis of this group of diseases and facilitated the diagnosis. We describe here the cell populations involved, the general pathology of Langerhans' cells, proposed pathogenesis, and the relationship between Langerhans' cell pulmonary granulomatosis and other localizations of Langerhans' cell granulomatosis. Finally, the clinical manifestations, elements of diagnosis and changing attitudes of current therapy are discussed.
朗格汉斯细胞组织细胞增生症,以前称为组织细胞增多症X,包括几种病因不明的肉芽肿病。最初的病理发现是朗格汉斯细胞肉芽肿。最近的进展增进了我们对这组疾病发病机制的理解,并促进了诊断。我们在此描述所涉及的细胞群体、朗格汉斯细胞的一般病理学、提出的发病机制,以及朗格汉斯细胞肺部肉芽肿病与朗格汉斯细胞肉芽肿病其他部位病变之间的关系。最后,讨论了临床表现、诊断要点以及当前治疗观念的转变。