Premium
Long‐Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis
Author(s) -
Sacco Oliviero,
Moscatelli Andrea,
Conte Massimo,
Grasso Chiara,
Magnano Gian Michele,
Sementa Angela Rita,
Martelli Alberto,
Rossi Giovanni A.
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25912
Subject(s) - medicine , extracorporeal membrane oxygenation , respiratory failure , langerhans cell histiocytosis , histiocytosis , vinblastine , lung , prednisone , lung transplantation , transplantation , surgery , pathology , disease , chemotherapy
Isolated pulmonary involvement in pediatric Langerhans cell histiocytosis (LCH) is extremely rare. While the multisystem‐LCH course varies from spontaneous remission to rapid deterioration with lethal outcome, single system involvement is generally associated with favorable prognosis. A child with isolated pulmonary LCH had an extremely rapid progression leading to respiratory failure, despite treatment with prednisone and vinblastine. Since lung hyperinflation and cystic degeneration contraindicated conventional mechanical ventilation, extracorporeal membrane oxygenation (ECMO) was chosen for 50 days as a bridge to lung transplantation. The mechanisms involved in disease progression and the usefulness of long‐term ECMO are discussed.