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Pulmonary histiocytosis X with mediastinal lymph node involvement
E Brambilla1, E Fontaine, C M Pison
1Division of Lung Disease, Hôpital A. Michallon, Joseph Fourier University, Grenoble, France.
The American Review of Respiratory Disease
|November 1, 1990
Summary
This report details a pulmonary histiocytosis X case with mediastinal lymph node involvement, confirmed by advanced microscopy and markers. Histological documentation of lymph node infiltration in this condition is presented.
Area of Science:
- Pulmonary Medicine
- Pathology
- Oncology
Background:
- Pulmonary histiocytosis X (PHX), also known as Langerhans cell histiocytosis, is a rare lung disease.
- Mediastinal lymph node enlargement has been anecdotally associated with PHX.
- Histological confirmation of lymph node involvement in PHX is lacking.
Observation:
- A patient presented with bilateral reticulonodular densities, indicative of lung disease.
- The patient also exhibited mediastinal lymph node enlargement.
- Classic histology of pulmonary histiocytosis X was observed.
Findings:
- Diagnosis was confirmed using electron microscopy and immunohistochemical markers (PS100, HLA-DR, CD1).
- These markers identified infiltration of the lymph nodes by X histiocytes.
- This study provides the first histological documentation of mediastinal lymph node involvement in PHX.
Implications:
- This finding expands the understanding of the pathological spectrum of pulmonary histiocytosis X.
- It highlights the importance of investigating mediastinal lymphadenopathy in patients with PHX.
- Further research may elucidate the clinical significance and treatment implications of lymph node involvement in PHX.