Severe isolated pulmonary Langerhans cell histiocytosis in a 6-year-old girl

European Journal of Pediatrics
Johannes SchulzeStefan Zielen

Abstract

Langerhans cell histiocytosis (LCH) usually affects different organs or bones. Isolated pulmonary disease is rare in childhood. We report about a 6-year-old girl with progressive pulmonary insufficiency, onset of clubbing at 4 years of age and honeycombing lung infiltrations on X-ray films. The radiological suspicion of primary pulmonary LCH was confirmed by the presence of CD1a positive cells in the bronchoalveolar lavage fluid. Other organs were not involved. The girl was treated according to the LCH-III International Study Protocol with a good response. Follow-up showed no reactivation of LCH but a reduced vital capacity and signs of interstitial pulmonary involvement on a CT scan. Langerhans cell histiocytosis should be considered in the aetiology of cystic lung diseases. Early responders to treatment have a high likelihood of becoming free of disease. However, pulmonary fibrosis is an important mechanism of lung remodelling in pulmonary Langerhans cell histiocytosis and the long-term prognosis is unclear.

Citations

Apr 7, 2009·Indian Journal of Pediatrics·Nishith Kumar JetleyAbdul Lateef Softha
Feb 8, 2006·Journal of Pediatric Hematology/oncology·Hassan A Al-TrabolsiAhmed A Al-Barki
Feb 7, 2007·Journal of Pediatric Hematology/oncology·Edna P AdamsAnthony Cecalupo
Dec 22, 2005·Respiration; International Review of Thoracic Diseases·Béla NagyBalázs Dezso
Sep 17, 2013·Indian Journal of Pediatrics·Sanjay VermaSunit C Singhi
Sep 25, 2012·Indian Journal of Pediatrics·Sneha VarkkiSophy Korula
Jul 3, 2015·Experimental and Therapeutic Medicine·Lintao BiZhenxing Zhu
Oct 20, 2004·Journal of Cellular and Molecular Medicine·Natalia BuzaSalima Haque
Jun 19, 2013·European Journal of Radiology·Shahina BanoKaushik Majumdar
May 29, 2007·Journal of Thoracic Imaging·Anastasia OikonomouJohn N Tsanakas
Jan 1, 2013·Journal of Clinical Imaging Science·Kemal OdevHüseyin Ozbiner
May 24, 2017·Orphanet Journal of Rare Diseases·Abdellatif TaziSylvie Chevret
Sep 11, 2020·Orphanet Journal of Rare Diseases·Solenne Le LouetSébastien Héritier

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