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Published on: June 16, 2020
Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
Solenne Le Louet1, Mohamed-Aziz Barkaoui2, Jean Miron2
1French Reference Center for Langerhans Cell Histiocytosis, Trousseau Hospital, 26 avenue du Dr Netter, 75012, Paris, France. solennelelouet@gmail.com.
Insights
Severe lung involvement in childhood Langerhans cell histiocytosis (LCH) is rare but carries a high mortality risk. Improved treatment guidelines, including targeted therapies and infection prophylaxis, are crucial for these young patients.
Area of Science:
- Pediatric Oncology
- Pulmonology
- Histiocytosis Research
Background:
- Childhood Langerhans cell histiocytosis (LCH) infrequently involves the lungs, with severe cases being rare but potentially life-threatening.
- A small subset of pediatric LCH patients require intensive care unit (ICU) admission due to respiratory failure.
Purpose of the Study:
- To analyze the characteristics, treatments, and outcomes of pediatric LCH patients with severe lung involvement requiring ICU admission.
- To identify factors contributing to mortality and inform improved management strategies for severe pediatric LCH lung disease.
Main Methods:
- Retrospective analysis of 17 pediatric LCH patients (age <15 years) admitted to the ICU for respiratory failure, identified from the French LCH registry (1994-2018).
- Evaluation of clinical presentation, risk-organ involvement, ICU admission causes (pneumothorax, cystic lesions, diffuse infiltration), therapeutic interventions (first- and second-line therapies including targeted therapy), and outcomes (mortality, survival, long-term follow-up).
Main Results:
- The median age at first ICU admission was 1.3 years. Pneumothorax was the most common reason for ICU admission (63%).
- 35% of patients died, with causes including repeated pneumothorax and diffuse micronodular lung infiltration. Survivors had a median follow-up of 11.2 years, with some remaining asymptomatic despite abnormal imaging.
- First-line therapy involved vinblastine-corticosteroids, with 12 patients requiring second-line treatments such as cladribine or targeted therapy.
Conclusions:
- Severe lung involvement in childhood LCH, though uncommon, is associated with significant mortality.
- Enhanced treatment protocols are needed, emphasizing viral infection prophylaxis and the early use of novel therapies like targeted therapy for pediatric LCH patients with severe lung disease.
Background:
Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed.
Methods:
Among 1482 children (< 15 years) registered in the French LCH registry (1994-2018), 111 (7.4%) had lung involvement. This retrospective study included data for 17 (1.1%) patients that required one or more intensive care unit (ICU) admissions for respiratory failure.
Results:
The median age was 1.3 years at the first ICU hospitalization. Of the 17 patients, 14 presented with lung involvement at the LCH diagnosis, and 7 patients (41%) had concomitant involvement of risk-organ (hematologic, spleen, or liver). Thirty-five ICU hospitalizations were analysed. Among these, 22 (63%) were secondary to a pneumothorax, 5 (14%) were associated with important cystic lesions without pneumothorax, and 8 (23%) included a diffuse micronodular lung infiltration in the context of multisystem disease. First-line vinblastine-corticosteroid combination therapy was administered to 16 patients; 12 patients required a second-line therapy (cladribine: n = 7; etoposide-aracytine: n = 3; targeted therapy n = 2). A total of 6 children (35%) died (repeated pneumothorax: n = 3; diffuse micronodular lung infiltration in the context of multisystem disease: n = 2; following lung transplantation: n = 1). For survivors, the median follow-up after ICU was 11.2 years. Among these, 9 patients remain asymptomatic despite abnormal chest imaging.
Conclusions:
Severe lung involvement is unusual in childhood LCH, but it is associated with high mortality. Treatment guidelines should be improved for this group of patients: viral infection prophylaxis and early administration of a new LCH therapy, such as targeted therapy.
