Abstract

Background: Langerhans cell histiocytosis (LCH) is a clonal neoplastic disorder characterized by the aberrant proliferation of CD1a+/CD207+dendritic cells that infiltrate tissues and organs, resulting in organ dysfunction.
Methods: This case report describes a 2-year-old boy who presented with abdominal pain and fever. The etiology was ultimately confirmed through clinical symptoms, imaging studies, pulmonary histopathological examination, and genetic testing.
Results: The final diagnosis was pediatric pulmonary Langerhans cell histiocytosis (PLCH).
Conclusions: Although pulmonary involvement is not classified as a high-risk in consensus guidelines, PLCH requires diagnostic consideration in children presenting with persistent respiratory symptoms and recurrent fever.

DOI: 10.7754/Clin.Lab.2025.250761