Author/Authors :
Earlam, K. Division of Respirology - The Ottawa Hospital - University of Ottawa, Ottawa, Canada , Souza, C. A. Department of Medical Imaging - The Ottawa Hospital - University of Ottawa, Ottawa, Canada , Glikstein, R. Department of Medical Imaging - The Ottawa Hospital - University of Ottawa, Ottawa, Canada , Gomes, M. M. Department of Pathology and Laboratory Medicine - The Ottawa Hospital - University of Ottawa, Ottawa, Canada , Pakhale, S. Division of Respirology - The Ottawa Hospital - University of Ottawa, Ottawa, Canada
Abstract :
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.