Pulmonary Langerhans cell histiocytosis - early nodular stage

Case contributed by Dr Yune Kwong


Non-smoker. Shortness of breath.

Patient Data

Age: 25
Gender: Male

Multiple small nodular opacities in both lungs.

Multiple small nodules which are ill-defined and appear ground glass. Some sub-pleural sparing is seen. All lobes are involved, but there is slightly less involvement of the extreme bases and costophrenic angles.

Bronchoscopic biopsy was non-diagnostic, and the patient underwent VATS biopsy.

Right upper lobe wedge resection: A wedge of lung measuring 30 x 8 x 10mm. On sectioning, the lung parenchyma has a dark grey and cream nodular appearance.
Sections of the lung show variably dilated alveolar spaces, most of which appear rather cellular. There is a polymorphic infiltrate of cells which include eosinophils, lymphocytes, histiocytes and groups of cells with moderate  amounts  of  pale cytoplasm and medium sized, round, oval or indented nuclei and delicate chromatin. Some of the nuclei  have  distinct  grooves. There  are  occasional  multinucleated cells included. These cells show strong staining with CD1a and S-100 and are negative with CD68.  The CD68 stain however marks the alveolar macrophages. The features are consistent with Langerhans cell histiocytosis. There are no granulomas. There is no neoplasia in the sections examined.
DIAGNOSIS: Features consistent with Langerhans cell histiocytosis.

Case Discussion

CD1a antigens marks the Langerhans cells.

In the acute phase of the disease, the nodules are poorly defined and they become better defined as the disease progresses.

This case is atypical given the absence of a smoking history (90-100% of cases associated with smoking). Also the absence of cysts is atypical, but this is explained by the early stage of the disease.

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Case information

rID: 30766
Published: 2nd Sep 2014
Last edited: 14th Aug 2019
System: Chest
Inclusion in quiz mode: Included

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