From the Departments of Radiology (J.A., S.N.J.P., J.O.S., J.D.G.) and Pathology (R.A.S.), University of Washington, 1959 NE Pacific St, UW Mailbox 357115, Seattle, WA 98195; and Department of Radiology, School of Medicine and Public Health, University of Wisconsin, Madison, Wis (J.P.K., C.A.M.).
Radiographics. 2015 Mar-Apr;35(2):357-70. doi: 10.1148/rg.352140197.
Histiocytic disorders of the chest comprise a broad spectrum of diseases. The lungs may be involved in isolation or as part of systemic disease. Some of these disorders are primary and have unknown etiology, and others result from a histiocytic response to a known cause. Among primary histiocytic disorders, pulmonary Langerhans cell histiocytosis (PLCH) is the most common; others include Erdheim-Chester disease and Rosai-Dorfman disease. Adult PLCH occurs almost exclusively in adults aged 20-40 years who smoke. Pediatric PLCH is extremely rare and typically occurs as part of multisystemic disease. Erdheim-Chester disease affects middle-aged and older adults; thoracic involvement usually occurs as part of systemic disease. Rosai-Dorfman disease affects children and young adults and manifests as painless cervical lymphadenopathy. Examples of secondary histiocytic disorders are storage diseases such as Gaucher disease, Niemann-Pick disease, and Fabry disease; pneumoconiosis such as silicosis and coal workers' pneumoconiosis; and infections such as Whipple disease and malakoplakia. These disorders are characterized at histopathologic examination on the basis of infiltration of alveoli or the pulmonary interstitium by histiocytes, which are a group of cells that includes macrophages and dendritic cells. Dendritic cells are a heterogeneous group of nonphagocytic antigen-presenting immune cells. Immunohistochemical markers help to distinguish among various primary histiocytic disorders. Characteristic radiologic findings in the appropriate clinical context may obviate biopsy to establish a correct diagnosis. However, in the absence of these findings, integration of clinical, pathologic, and radiologic features is required to establish a diagnosis.
胸部组织细胞疾病包括广泛的疾病谱。肺部可能孤立受累，也可能是系统性疾病的一部分。其中一些疾病是原发性的，病因不明，而另一些则是由于组织细胞对已知原因的反应所致。在原发性组织细胞疾病中，肺朗格汉斯细胞组织细胞增生症（PLCH）最为常见；其他还包括 Erdheim-Chester 病和 Rosai-Dorfman 病。成人 PLCH 几乎仅发生于年龄在 20-40 岁的吸烟人群。儿童 PLCH 极为罕见，通常作为多系统疾病的一部分出现。Erdheim-Chester 病影响中老年人；胸部受累通常是系统性疾病的一部分。Rosai-Dorfman 病影响儿童和年轻成人，表现为无痛性颈淋巴结病。继发性组织细胞疾病的例子包括贮积病，如 Gaucher 病、 Niemann-Pick 病和 Fabry 病；尘肺，如矽肺和煤工尘肺；以及感染，如 Whipple 病和马尔尼菲青霉菌病。这些疾病在组织病理学检查中表现为肺泡或肺间质浸润的组织细胞，组织细胞是一组包括巨噬细胞和树突状细胞的细胞。树突状细胞是一组异质性的非吞噬性抗原呈递免疫细胞。免疫组织化学标志物有助于区分各种原发性组织细胞疾病。在适当的临床背景下，具有特征性的放射学发现可能无需活检即可建立正确的诊断。然而，在缺乏这些发现的情况下，需要整合临床、病理和放射学特征来建立诊断。
