Infantile Histiocytosis X: Case Report


1 Department of Pediatrics,

2 Department of Clinical Anatomical Pathology

3 Oncology Unit, NRITLD, Shaheed Beheshti University of Medical Sciences and Health Services, TEHRAN-IRAN


Langerhans cell histiocytosis (LCH) is a disease of unknown etiology that presents in three forms: Letterer- Siwe disease, Hand-Schuller-Christian disease, and Eosinophilic Granuloma. It is a multifocal disease that usually affects several organs and unifocal forms are rare. Pulmonary involvement occurs in approximately 40% of cases and almost always in young adults. Lungs are rarely affected in infant patients. This report presents a 15-month-old male infant that was admitted for cyanosis and respiratory distress. Diagnostic work up revealed a primary pulmonary histiocytosis and the treatment made a significant improvement of signs and symptoms by the time of discharge. (Tanaffos 2002; 1(3): 57-61)