Langerhans Cell Histiocytosis


Langerhans cell histiocytosis (LCH) refers to a collection of syndromes, characterized by infiltration of various tissues by Langerhans cells. In 1941, Farber suggested that eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schüller-Christian disease all represent different manifestations of a single pathologic process, and in 1953, Lichtenstein used the term “histiocytosis X” to encompass these entities (1,2). Subsequent to the description of Birbeck granules as a specific ultrastructural marker for Langerhans cells (3), the infiltrating cells of histiocytosis X were identified as Langerhans cells. In 1987, the Writing Group of the Histiocyte Society proposed that Langerhans cell histiocytosis replace the term histiocytosis X as more appropriate (4).


Hematopoietic Stem Cell Transplantation Eosinophilic Granuloma Seborrheic Dermatitis Birbeck Granule Organ System Involvement 
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