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Langerhans Cell Histiocytosis

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Evidence-Based Imaging in Pediatrics

Part of the book series: Evidence-Based Imaging ((Evidence-Based Imag.))

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Abstract

Histiocytoses are rare disorders characterized by the pathological proliferation of monocyte and macrophage lineages, of which Langerhans’ cell histiocytosis (LCH) is the most common [1, 2]. Historically, the classification and naming of histiocytic disorders, including LCH, has been challenging due to the variety of clinical presentations which are a function of which organ system(s) have been invaded by the pathological cells. Presentation ranges from a self-limiting skin rash to fulminant multisystem disease with high mortality [3]. Previously described eponyms associated with particular presentations of LCH, such as eosinophilic granuloma, Hand-Schüller-Christian, and Letterer-Siwe disease, are of historical interest but should be disregarded in clinical practice [4]. In 1987, the Writing Group of the Histiocytosis Society proposed that Histiocytosis X be reclassified as LCH, the currently accepted term [5].

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El-Ali, A., Contreras Yametti, G., Robbins, G., Lala, S., Davis, J.C., Degnan, A.J. (2022). Langerhans Cell Histiocytosis. In: Kaplan, S.L., Otero, H., Medina, L.S., Blackmore, C.C., Applegate, K.E. (eds) Evidence-Based Imaging in Pediatrics. Evidence-Based Imaging. Springer, Cham. https://doi.org/10.1007/978-3-030-38095-3_67-1

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