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pulmonary Langerhans cell granulomatosis
Etiology: cigarette smoking is risk factor
Epidemiology:
1) more common in women [2]; young males [3]
2) more common in smokers (95% of patients)
3) more common in whites, rare in blacks
4) uncommon disease, 1500 cases reported as of 1998
Pathology:
1) proliferation of histiocytes with numerous eosinophils interspersed
2) pulmonary fibrosis replaces granulomatous changes as the disease progresses
3) Langerhans' cells detected by electron microscopy (EM)
Clinical manifestations:
1) dyspnea in 40%
a) osteolytic rib lesions
b) recurrent pneumothorax
2) fever
3) fatigue
4) weight loss
5) spontaneous resolution is not uncommon
Laboratory:
1) lung biopsy or bronchoalveolar lavage (BAL)
2) S100 staining on light microscopy
3) no eosinophils in peripheral blood
Special laboratory:
Pulmonary function testing:
1) restrictive pattern
a) decreased lung volumes
b) normal flow rate
c) decreased diffusion capacity
2) generally, relatively good pulmonary function despite extensive chest x-ray abnormalities
Radiology:
1) chest X-ray
a) involvement is typically diffuse, bilateral & more pronounced in upper 2/3 of lungs
b) honeycomb cyst pattern
c) pneumothorax
2) high resolution computed tomography (CT):
- cystic, nodular changes
Management:
1) smoking cessation
2) supportive
3) corticosteroids
4) Vinca alkaloids
Related
eosinophilia
vinca alkaloid
General
Langerhans cell histiocytosis (LCH); histiocytosis X
References
- Contributions from Peter Baylor, UCSF Fresno
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 755-56
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14,
American College of Physicians, Philadelphia 1998, 2006