Management of severe pulmonary Langerhans cell histiocytosis in children

Pediatr Pulmonol. 2020 Aug;55(8):2074-2081. doi: 10.1002/ppul.24822. Epub 2020 Jun 8.


Patients with pulmonary Langerhans cell histiocytosis (LCH) typically have a benign course but may have extensive cystic lung disease with rare life-threatening complications including multiple and recurrent pneumothoraces and respiratory failure. We report seven severely affected pediatric patients treated with chemotherapy, aggressive chest tube management, and pleurodesis of whom five survived. Patients with extraordinary amounts of pulmonary cystic disease and multiple pneumothoraces due to LCH can have remarkable, curative outcomes with early recognition, optimal LCH-directed therapy, and supportive care.

Keywords: Langerhans cell histiocytosis; chemotherapy; cystic lung disease; pleurodesis; pneumothorax.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Chest Tubes
  • Child, Preschool
  • Female
  • Histiocytosis / therapy*
  • Humans
  • Infant
  • Infant, Newborn
  • Lung Diseases / therapy*
  • Male
  • Pleurodesis
  • Pneumothorax / therapy*

Supplementary concepts

  • Cystic Disease Of Lung