Extract
Langerhans cell histiocytosis (LCH) is a group of disorders with variable presentations and outcomes. Children with LCH primarily have bone and multisystem involvement with little impact of lung involvement and usually no treatment indication for lung disease [1, 2]. Conversely, pulmonary involvement is the main determinant of morbidity and mortality in adults and is usually resistant to first-line chemotherapeutic agents used in children [3]. Pulmonary LCH belongs to the spectrum of LCH and is primarily found in smoker adults. In most cases, quitting smoking results in clinical, functional and radiological improvement at early or nodulo-cystic stages [4]. In those with progressive disease and significant lung function impairment, cladribine (a purine nucleoside analog) has been proposed as rescue therapy for progressive, refractory nodulo-cystic disease [5, 6]. However, there are currently no treatment options for patients with advanced, cystic pulmonary LCH.
Abstract
Cladribine therapy may be beneficial in advanced forms of pulmonary Langerhans cell histiocytosis, even that with multiple cystic changes http://ow.ly/yeLr30i0Tt6
Footnotes
Conflict of interest: M. Nasser reports personal fees for travel to meetings from Boehinger Ingelheim, outside the submitted work.
Conflict of interest: J. Traclet reports personal fees for travel to meetings from Boehinger Ingelheim, Roche and Actelion, outside the submitted work.
Conflict of interest: V. Cottin reports personal fees for consultancy, travel to meetings and lectures from Actelion, Novartis and Roche. He reports personal fees for developing educational programmes, consultancy, travel to meetings and lectures from Boehringer Ingelheim. He reports personal fees for consultancy from Bayer, Biogen Idec and MSD, and personal fees from Gilead for committee membership. V. Cottin reports personal fees for consultancy and travel to meetings from GSK, and personal fees for consultancy and lectures from Sanofi. He has received grants from Boehringer Ingelheim, GSK, Actelion and Roche, and has received personal fees from Promedior, outside the submitted work.
- Received July 24, 2017.
- Accepted December 21, 2017.
- Copyright ©ERS 2018
This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.