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9
Correspondence to: Dr A J Hance
| The first 150 words of the full text of this article appear below. |
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Introduction |
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In the early 1950s Lichtenstein introduced the term histiocytosis X to describe a group of syndromes with seemingly unrelated clinical features, but whose pathological findings were characterised by the infiltration of involved tissues with large numbers of unusual histiocytes, often organised as granulomas.1 Subsequently it was shown that these histiocytes were similar to Langerhans' cells normally present in the skin and other epithelia, a finding that led to the current designation of Langerhans' cell histiocytosis (LCH) for this group of disorders.2 3
Although the different forms of LCH are united by a common
histopathological finding
the Langerhans' cell granulomathe
clinical spectrum of these disorders is extremely large, extending from an acute disseminated form which usually occurs in infants and carries
a poor prognosis, to the presence of lesions localised to a single
tissue which follows a more benign clinical course (table
1).4-8 Pulmonary involvement is not unusual in systemic
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