Langerhans cell granulomatosis

Czech version

Authors: J. Homolka ^{1, 2}; I. Haškovcová ³
Authors‘ workplace: I. klinika tuberkulózy a respiračních nemocí 1. lékařské fakulty UK a VFN Praha, přednosta prof. MU Dr. Jiří Homolka, DrSc. ¹; Pneumologická klinika 1. lékařské fakulty UK a FTN Praha, přednosta prof. MU Dr. Jiří Homolka, DrSc. ²; Ústav patologie 1. lékařské fakulty UK a VFN Praha, přednosta prof. MU Dr. Ctibor Povýšil, DrSc. ³
Published in: Vnitř Lék 2010; 56(Supplementum 2): 74-75
Category: Langerhans cell histiocytosis and some other Hematology rare diseases

Overview

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.

Key words:
Langerhans cell granulomatosis – diagnostics – BAL – treatment

Sources

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