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 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.
Department of Medical Oncology, UZ Brussel - Vrije Universiteit Brussel, Brussels, Belgium. lore.decoster@uzbrussel.be
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L
Decoster
K
K
De Braekeleer
C
C
Bourgain
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D
Schallier
D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011859:Radiography; D055815:Young Adult