Langerhans Cell Histiocytosis

What is Langerhans Cell Histiocytosis?  
Overview: Langerhans Cells are immature cells that express monocyte marker CD14 which is not found on normal skin Langerhans cells
Langerhans Cells Histiocytosis(LCH) is divided into 3 classes:
dendritic cell-related, macrocyte/monocyte cell related, and malignancy (ie. monocyte-related leukemia)
LCH can be localized or diffuse
Treatment and prognosis are based on organ involvement (spleen, lung, bone marrow)

Pulmonary Histiocytosis strongly associated with tobacco smoking
Usually presents in 20-40 y.o.
Most common presentation includes:
Nonproductive cough (56 to 70 percent)
Dyspnea (40 to 87 percent)
Chest pain, which is frequently pleuritic (10 to 21 percent)
Fatigue (approximately 30 percent)
Weight loss (20 to 30 percent)
Fever (15%)
DI, due to hypothalmic involvement, occurs often and it thought to be poor prognostic indicator
Pulm Histiocytosis leads to pulm fibrosis, honeycombing and cysts
Smoking cessation improves outcomes
Median survival is 12.5 yrs
No great therapy; clinical trials are ongoing: www.histio.org

(Ellen Eaton MD, 11/23/10)