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Langerhans cell histiocytosis (eosinophilic granuloma) of bone in children and adolescents

Kenneth L McClain, MD, PhD
Section Editor
Alberto S Pappo, MD
Deputy Editor
Alan G Rosmarin, MD


Langerhans cell histiocytosis (LCH) of the bone, also called eosinophilic granuloma of bone, was first described in 1940 [1-4]. Patients may present with a solitary lesion (monostotic) or multiple sites of involvement (polyostotic) [5].

In 1953, eosinophilic granuloma of bone was classified along with Hand-Schüller-Christian disease (with the classic triad of exophthalmos, diabetes insipidus, and skull lesions) and Letterer-Siwe disease (lymphadenopathy, skin rash, hepatosplenomegaly, fever, anemia, and thrombocytopenia) as Histiocytosis X [6]. Subsequently, the Langerhans cell of the skin was proposed as the underlying shared pathologic feature among these three disorders [7].

However, gene expression array analysis of CD207+ (langerin) histiocytes from bone lesions compared with the Langerhans cells of skin proves that these are two distinctly different cell types [8]. The pathologic cells in LCH are now known to be derived from circulating myeloid dendritic cells [9].

Under the direction of the Writing Group of the Histiocyte Society, LCH has been adopted as the appropriate clinicopathologic designation that encompasses and essentially replaces the previous historical terms used to classify this category of abnormal histiocyte proliferation [10]. However, many authors continue to utilize the term eosinophilic granuloma of bone; thus, this latter terminology is still both historically and clinically relevant.

This topic review will discuss LCH of the bone in children. Discussions of LCH involving other organs in children and adults are presented separately. (See "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis" and "Pulmonary Langerhans cell histiocytosis".)

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Literature review current through: Nov 2017. | This topic last updated: Oct 28, 2016.
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