Natural Course of Isolated Pulmonary Langerhans’ Cell Histiocytosis in a Toddler

Abstract

Isolated pulmonary Langerhans’ cell histiocytosis (LCH) is distinctly rare under the age of 15 years, since the majority of patients are young adult males with heavy smoking habits. Isolated pulmonary involvement suggests that antigens inhaled from cigarette smoke are involved. Here we present a case of LCH restricted to the lungs in a toddler whose parents were heavy smokers. Since LCH was not medically treated for 3 years due to parental refusal, the disease can be regarded as having followed its natural course. During the 3-year follow-up, the disease progressed to severe pulmonary fibrosis resulting in honeycomb lungs. Based on the comparative immunohistochemical analyses of the cells obtained from bronchoalveolar lavages during the disease course, it appears that the evolution of fibrosis is rather a result from the accumulating alveolar macrophages than from the persistence of the Langerhans’ cells. Passive cigarette smoking may be considered a significant risk factor in both the pathogenesis and development of pulmonary LCH in a small child.

Isolated Pulmonary Langerhans Cell Histiocytosis in a 3-Year-old Girl With Atypical Radiologic Findings

“…Advanced pulmonary LCH patients may exhibit CT evidence of fibrosis and honeycombing. [4][5][6][7][8][9] In our case, the CT findings differed to the adult pattern. Thin-walled cysts were found throughout the lung parenchyma not sparing the lung bases.…”

“…Pleural effusions occur infrequently but may be observed in association with pneumothorax or after diagnostic thoracotomy. [4][5][6][7][8][9] CT, especially high-resolution CT (HRCT), is superior to radiography in demonstrating the morphology and distribution of lung abnormalities in patients with pulmonary LCH.…”

Primary pulmonary Langerhans cell histiocytosis associated with smoking in an adolescent boy

“…12,13 Few cases of confirmed isolated PLCH have been reported in literature in the pediatric age group. 2,5,8,[14][15][16] In only two of them with eventually stabilized disease. Our patient was symptomatic at the beginning.…”

“…Three of them were active smokers, and two of them were reported as a result of second hand smoking. [3][4][5][6] We now present a rare case of severe PLCH in an adolescent boy who smokes.…”

Decreased VEGF Level Is Associated with Elevated Ferritin Concentration in Bronchoalveolar Lavage Fluid of Children with Interstitial Lung Diseases

“…In addition, there were 17 subjects having no ILD who were eventually excluded from this investigation: 11 suffered from virus-induced interstitial pneumonitis, 2 from lymphoproliferative disease, 1 from Niemann-Pick disease [33], 2 from Langerhans cell histiocytosis [34] and 1 from pulmonary sarcoidosis (data not shown).…”