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Histiocytoses 

Histiocytoses

Chapter:
Histiocytoses
Author(s):

D.K.H Webb

DOI:
10.1093/med/9780199204854.003.220407
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date: 19 August 2017

The histiocytoses are characterized by the infiltration of affected tissues with cells of monocyte/macrophage lineage.

Langerhans’ cell histiocytosis—may present with (1) disease affecting a single organ—typically skin (rash) or bone (pain and soft tissue swelling, or asymptomatic radiographic lesions); or (2) multisystem disease—characteristic features include ear discharge, diabetes insipidus, and lung involvement (diffuse micronodular shadowing on chest radiography, with progression to cyst formation and a honeycomb lung appearance). Diagnosis requires identification of Langerhans’ cells within lesional inflammatory cell infiltrate, with demonstration of either the CD1a surface antigen on immunohistochemistry or the presence of Birbeck granules on electron microscopy. Most cases eventually resolve spontaneously. Immunosuppressive and/or cytotoxic drugs are given when there is progressive organ injury, but the most effective and least toxic approach to treatment is not known....

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