Barth J, Kreipe H, Radzun H J, Heidorn K, Petermann W, Bewig B, Parwaresch M R
Department of Medicine, University of Kiel, Germany.
Thorax. 1991 Nov;46(11):835-8. doi: 10.1136/thx.46.11.835.
Pulmonary histiocytosis X is the local manifestation of a systemic disorder of unknown cause characterised by infiltration of Langerhans cell like histiocytes and parenchymal fibrosis. In a male smoker with histologically proved histiocytosis X and functional impairment bronchoalveolar lavage showed an increase in CD-1/OKT-6 antigen positive histiocytes to 8%. Northern blot analysis of RNA from bronchoalveolar lavage cells showed an exaggerated expression of the M-CSF gene and of the c-fms gene encoding for the corresponding receptor. An increased level of c-sis RNA, which encodes the B chain of platelet derived growth factor, was also found. Diffuse reticulonodular infiltrates on the chest radiograph resolved with glucocorticoid treatment and CD-1/OKT-6 antigen positive histiocytes fell to 3%. Macrophage colony stimulating factor, c-fms and c-sis gene expression were reduced almost to normal after treatment. The results suggest that macrophage colony stimulating factor and platelet derived growth factor may have a role in the initiation or maintenance of pathological reactions in pulmonary histiocytosis X.
肺组织细胞增多症X是一种病因不明的全身性疾病的局部表现，其特征为朗格汉斯细胞样组织细胞浸润和实质纤维化。在一名经组织学证实为组织细胞增多症X且有功能损害的男性吸烟者中，支气管肺泡灌洗显示CD-1/OKT-6抗原阳性组织细胞增加至8%。对支气管肺泡灌洗细胞的RNA进行Northern印迹分析显示，M-CSF基因以及编码相应受体的c-fms基因表达过度。还发现编码血小板衍生生长因子B链的c-sis RNA水平升高。胸部X线片上的弥漫性网状结节浸润经糖皮质激素治疗后消退，CD-1/OKT-6抗原阳性组织细胞降至3%。治疗后巨噬细胞集落刺激因子、c-fms和c-sis基因表达几乎降至正常。结果表明，巨噬细胞集落刺激因子和血小板衍生生长因子可能在肺组织细胞增多症X的病理反应的起始或维持中起作用。
