Langerhans Cell Histiocytosis - A Case Report.
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Date
2007-07
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Abstract
Histiocytosis is a heterogenous group of disorders that are characterized by proliferation and activation of
mononuclear phagocyte system. Langerhans Cell Histiocytosis (LCH) or Class I histiocytosis is a rare disorder
of unknown etiology with proliferation of Langerhan cells which may infiltrate a single or multiple organs. This
disease is more common in infants and children. It is usually sporadic but a familial pattern is known. The term
embraces the whole clinical spectrum of the disorder from single bone lesions (eosinophilic granuloma) to an
aggressive widespread multisystem disease in very sick child (Letterer-Siwe disease) with a wide variety of
intermediate forms including the Hand-shuller Christian triad. The cause of LCH is not firmly established and
most investigators in the field have long suspected that LCH is immunologic disorder either in its etiology or in
its pathophysiology1,2. Recent evidence suggests that LCH is a clonal disorder rather than reactive disease3. LCH
is classified according to sites of involvement into single system disease and multisystem disease4. Single system
disease can be either unifocal or multifocal. Multisystem disease can be either without organ dysfunction or with
organ dysfunction. Clinical course of LCH with single system disease is usually benign with high chance of
survival5,6. However, its clinical course is often unpredictable and patients can experience spontaneous remission
and exacerbations. Histiocytic diseases are currently classified by the writing group of the Histiocyte Society7 in
the three classes, namely;
Class I: Langerhans Cell Histiocytosis (LCH)
Class II: Histiocytosis of mononuclear phagocytes other than Langerhans Cells
Class III: Malignant Histiocytic disorders
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Keywords
Langerhans Cell Histiocytosis, Eosinophilic granuloma, Skull, lytic lesion
Citation
Gurubacharya S M, Gurubacharya R L. Langerhans Cell Histiocytosis - A Case Report. Journal of Nepal Paediatric Society. 2007 Jul-Dec; 27(2): 87-89.