Pulmonary
Histiocytosis X
Brief Discussion
Langerhans cell histiocytosis
(histiocytosis X, eosinophilic granuloma)
- Early stage : nodular collections of Langerhans histiocytes and eosinophils (juxtabronchiolar )
- Late stage : fibrosis, honeycombing
- young or middle-aged adults, male predominance, ciagrette smoking history (> 90%), pneumothorax (14%)
- variable PFT pattern/ decreased diffusing capacity
- 60% (lung), 40% (lung + bone, other organ)
- Px : 1/2 favorable, 1/3 stable, 20% progress ( respiratory failure, pulmonary a. HT)
CXR
- nodular, reticulonodular, and cystic abnormalities (upper lung >>)