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Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis

Kenneth L McClain, MD, PhD
Section Editor
Peter Newburger, MD
Deputy Editor
Alan G Rosmarin, MD


Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions demonstrating infiltration with histiocytes having bean-shaped nuclei on biopsy. These histiocytes, along with lymphocytes, macrophages, and eosinophils may infiltrate nearly every organ (most notably the skin, lymph nodes, lungs, thymus, liver, spleen, bone marrow, or central nervous system with the exception of the heart and kidneys).

LCH is so named because the morphology and immunophenotype of the pathologic cells resemble Langerhans cells, which are specialized dendritic cells found in the skin and mucosa. However, LCH is derived from myeloid progenitor cells from the bone marrow, and is not derived from the Langerhans cell of the skin.

For now, "Langerhans cell histiocytosis" remains the preferred nomenclature; the historical terms histiocytosis-X, Letterer-Siwe disease, Hand-Schüller-Christian disease, and diffuse reticuloendotheliosis have been abandoned. The term "eosinophilic granuloma" is sometimes used to describe the pathology of an individual lesion, particularly isolated lytic processes in bone.

The epidemiology, clinical manifestations, pathologic features, diagnosis, and differential diagnosis of LCH will be presented here. The management of LCH is presented separately, as are specific discussions of solitary LCH involving lung or bone. (See "Treatment of Langerhans cell histiocytosis" and "Pulmonary Langerhans cell histiocytosis" and "Langerhans cell histiocytosis (eosinophilic granuloma) of bone in children and adolescents".)


The archaic term "histiocyte" refers to large white blood cells resident in tissues, including Langerhans cells, monocytes/macrophages, and dermal/interstitial dendritic cells [1].

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Literature review current through: Nov 2017. | This topic last updated: Aug 28, 2017.
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