Vnitr Lek 2010, 56(Suppl 2):74-75

Langerhans cell granulomatosis

J. Homolka^1,2,*, I. Haškovcová³

¹ I. klinika tuberkulózy a respiračních nemocí 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Jiří Homolka, DrSc.

² Pneumologická klinika 1. lékařské fakulty UK a FTN Praha, přednosta prof. MUDr. Jiří Homolka, DrSc.

³ Ústav patologie 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Ctibor Povýšil, DrSc.

Langerhans cell granulomatosis is a rare disease of unknown aetiology and characterized by an infiltration of alveolar septa with activated Langerhans cells and eosinophylic granulocytes. Accumulation of these cells leads to a development of multiple cystic formations. The involvement of the lungs is usually present on its own, more rarely it is seen as part of a systemic disease involving skeleton or posterior pituitary. The disease prognosis in adult patients is usually good, 80% of patients achieve remission or stabilization of the disease either spontaneously or following a corticosteroid therapy, 10-20% of cases progress to respiratory failure.

Keywords: Langerhans cell granulomatosis; diagnostics; BAL; treatment

Received: July 21, 2010; Published: April 1, 2010  Show citation