Paediatric pulmonary Langerhans cell histiocytosis

Abstract

<jats:p>Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.</jats:p><jats:p>Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.</jats:p><jats:sec><jats:title>Educational aims</jats:title><jats:list list-type="bullet"><jats:list-item><jats:p>To give an overview of paediatric pulmonary LCH.</jats:p></jats:list-item><jats:list-item><jats:p>To discuss the differential diagnosis of paediatric cystic lung disease.</jats:p></jats:list-item></jats:list></jats:sec>

Breathlessness in a Child Exposed to COVID-19

“…Most commonly upper and middle lobe involvement is seen with costophrenic angle involved as well. 4 Lung biopsy or bronchoalveolar lavage showing CD1a (definitive diagnosis) and S100 positivity confirms presence of LCH. Pathologically inflammatory infiltrates with histiocytes can be seen.…”

“…Clinically recurrent pneumothorax is common presentation in these children. 3,4 In multisystem disease lung symptoms are often last ones to appear while extra pulmonary manifestations dominate presentation.…”

“…Presence and demonstration of Birkbeck granules is gold standard. 4 Chemotherapy is mainstay with vincristine and prednisolone being basic therapy. 4,5 Cladribine and cytarabine have also been used.…”

“…4 Chemotherapy is mainstay with vincristine and prednisolone being basic therapy. 4,5 Cladribine and cytarabine have also been used. 4,5 Hematopoietic stem cell transplant has also been considered but still is under research.…”

“…4,5 Cladribine and cytarabine have also been used. 4,5 Hematopoietic stem cell transplant has also been considered but still is under research. Specific management for pulmonary point of view is for recurrent pneumothorax.…”