Abstract
Adult pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder of unknown etiology that occurs predominantly in young smokers, with an incidence peak at 20-40 years of age. In adults, pulmonary involvement with Langerhans cell histiocytosis usually occurs as a single-organ disease and is characterized by focal Langerhans cell granulomas infiltrating and destroying distal bronchioles. We report a 23-year-old asymptomatic male smoker with a non specific interstitial infiltrate found in preventive chest X ray examination. A high resolution chest CT scan showed multiple cystic structures predominating in the upper lobes, with small centrilobular nodules. A transbronchial biopsy showed a lymphocytic lung infiltrate with Langerhans cells. A surgical biopsy confirmed the diagnosis of pulmonary Langerhans cell histiocytosis. After six months of follow up, the patient is in good conditions.
Highlights
Adult pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder of unknown etiology that occurs predominantly in young smokers, with an incidence peak at 20-40 years of age
Pulmonary involvement with Langerhans cell histiocytosis usually occurs as a single-organ disease and is characterized by focal Langerhans cell granulomas infiltrating and destroying distal bronchioles
A surgical biopsy confirmed the diagnosis of pulmonary Langerhans cell histiocytosis
Summary
Recibido el 4 de mayo de 2010, aceptado el 16 de diciembre de 2010. Departamento de Enfermedades Respiratorias Pontificia Universidad Católica de Chile Teléfonos: 6331541 - 3543242. Adult pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder of unknown etiology that occurs predominantly in young smokers, with an incidence peak at 20-40 years of age. Pulmonary involvement with Langerhans cell histiocytosis usually occurs as a single-organ disease and is characterized by focal Langerhans cell granulomas infiltrating and destroying distal bronchioles. A transbronchial biopsy showed a lymphocytic lung infiltrate with Langerhans cells. A surgical biopsy confirmed the diagnosis of pulmonary Langerhans cell histiocytosis. La histiocitosis de células de Langerhans corresponde a un grupo heterogéneo de enfermedades de origen desconocido, con manifestaciones clínicas y pronóstico variable, caracterizadas por la infiltración de histiocitos anormales en los tejidos, a menudo organizados en granulomas[1,2,3]. En relación a un caso clínico revisaremos la epidemiología, etiopatogenia, evaluación funcional, diagnóstico y tratamiento de esta entidad
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