Premium
Management of severe pulmonary Langerhans cell histiocytosis in children
Author(s) -
Eckstein Olive S.,
Nuchtern Jed G.,
Mallory George B.,
Guillerman R. Paul,
Musick Matthew A.,
Barclay Mhairi,
Bhatt Jayesh M.,
Davies Patrick,
Grundy Richard G.,
Martin Alice,
Hilliard Tom,
Lowis Stephen P.,
Picton Susan,
Nanduri Vasanta,
Visser Johannes,
Allen Carl E.,
McClain Kenneth L.
Publication year - 2020
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.24822
Subject(s) - medicine , langerhans cell histiocytosis , histiocytosis , respiratory failure , pleurodesis , chemotherapy , respiratory disease , lung , disease , surgery , pneumothorax
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.