Langerhans cell histiocytosis manifesting as recurrent simultaneous bilateral spontaneous pneumothorax in early infancy.
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by infiltration of either single or multiple organs by a distinct cell type that is S-100 and CD1a positive and contains ultrastructural Birbeck granules on electron microscopy. Historically, LCH included four main clinical forms: Letter-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma (together grouped as histiocytosis) and Hashimoto-Pritzker disease. The writing group of the Histiocytotic Society in 1987 proposed the uniform term of 'Langerhans cell histiocytosis' to encompass all the aforementioned eponymous forms. Lung involvement occurs in up to half of all children with multisystem disease and usually parallels overall disease activity. Spontaneous pneumothorax (SP) occurs in approximately 10% of children with pulmonary disease and may be a fatal complication. Patients with pulmonary LCH are likely predisposed to the development of pneumothorax based on destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH in which the patient presented at 2 months of age because of simultaneous bilateral pneumothorax.
Department of Pediatric Hematology/Oncology, Mofid Children's Hospital, Shahid Beheshti Medical University, Tehran, Iran. samin_alavi@yahoo.com
Samin
S
Alavi
Zahra
Z
Ashena
Afshin
A
Paydar
Nadereh
N
Hemmati
D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008168:Lung / Q000000981:diagnostic imaging; D011030:Pneumothorax / Q000000981:diagnostic imaging / Q000209:etiology*; D011859:Radiography