D.K.H Webb

in Oxford Textbook of Medicine

Published on behalf of Oxford University Press

Published in print May 2010 | ISBN: 9780199204854
Published online May 2010 | e-ISBN: 9780199570973 | DOI:

Series: Oxford Textbooks


Show Summary Details


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....

Chapter.  4106 words.  Illustrated.

Subjects: Haematology

Full text: subscription required

How to subscribe Recommend to my Librarian

Buy this work at Oxford University Press »

Users without a subscription are not able to see the full content. Please, subscribe or login to access all content.