Langerhans cell histiocytosis: two case reports in adults and review of the literature

Langerhans Cell histiocytosis is a rare proliferative histiocytic disorder in which pathologic Langerhans cells accumulate in a variety of organs. The clinical presentation, evolution and therapeutic options are highly variable. Because of its relative rarity and the broad clinical spectrum, the dia...

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Bibliographic Details
Published inActa clinica belgica (English ed. Online) Vol. 65; no. 5; p. 345
Main Authors Decoster, L, De Braekeleer, K, Bourgain, C, Schallier, D
Format Journal Article
LanguageEnglish
Published England 01.09.2010
Subjects
Histiocytosis, Langerhans-Cell - diagnosis
Histiocytosis, Langerhans-Cell - diagnostic imaging
Histiocytosis, Langerhans-Cell - metabolism
Histiocytosis, Langerhans-Cell - pathology
Histiocytosis, Langerhans-Cell - therapy
Humans
Immunohistochemistry
Male
Middle Aged
Prognosis
Radiography
Young Adult
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Summary:Langerhans Cell histiocytosis is a rare proliferative histiocytic disorder in which pathologic Langerhans cells accumulate in a variety of organs. The clinical presentation, evolution and therapeutic options are highly variable. Because of its relative rarity and the broad clinical spectrum, the diagnosis of Langerhans cell histiocytosis is often delayed or missed. At present, many questions with respect to aetiology, pathogenesis and treatment remain unanswered. In the present article we want to raise the awareness of this rare disease in adults and its diversity by the means of two case reports. In addition, the clinical manifestations, diagnosis and the current management are reviewed.
ISSN:1784-3286
DOI:10.1179/acb.2010.074