Pediatric pulmonary Langerhans cell histiocytosis complicated by recurrent hemopneumothoraces

Abstract

Langerhans cell histiocytosis is a rare disease of clonal proliferation and deposition of immature dendritic cells in various tissues, including the lungs, causing inflammation and organ destruction. We report a case of a 4-year-old girl with refractory multisystem Langerhans cell histiocytosis complicated by recurrent bilateral hemopneumothoraces in a pediatric tertiary hospital in Cape Town, South Africa. Video-assisted thoracoscopy revealed chronic inflammation of her pleura, with pleural fibrosis seen on histology. Despite pleurodesis, she continued to have recurrent hemopneumothoraces until she was started on a hydroxyurea-containing regimen with a good response.