Earlam K, Souza C A, Glikstein R, Gomes M M, Pakhalé S
Division of Respirology, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada K1H 8L6.
Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada K1H 8L6.
Can Respir J. 2016;2016:3740902. doi: 10.1155/2016/3740902. Epub 2016 Apr 11.
Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.
朗格汉斯细胞组织细胞增多症的特征是组织细胞在各个器官系统中出现异常的结节状增殖。在年轻成年人中出现的肺部受累几乎总是在过去或当前吸烟的背景下出现。尽管它往往是一种单系统疾病，但累及皮肤、骨骼和下丘脑 - 垂体轴的肺外表现也是可能的。胸部高分辨率CT（HRCT）表现包括小叶中心结节和囊肿，其形状怪异、大小不一且壁薄。通常根据适当的临床表现和典型的影像学表现即可做出诊断。治疗包括戒烟，以及根据疾病严重程度和多系统受累情况可能使用糖皮质激素或细胞毒性药物。
