Adult langerhans cell histiocytosis with hepatic and pulmonary involvement

Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.

Figure 1

Thoracic X-ray: multiple pulmonary nodules are identified, with upper lobe predominance. Note that some nodules show cavitation.

Figure 2

Axial thoracic CT (lung window): multiple bilateral pulmonary nodules are identified in different stages of cavitation, a typical finding of pulmonary LCH.

Figure 3

Axial thoracic CT image (lung window) in a lower thoracic CT slice: less nodules are identified. Note the normal appearance of the lung parenchyma other than the nodules.

Figure 4

Axial contrast-enhanced abdominal CT: the patient presented increased liver dimension (hepatomegaly) in a biopsy proven liver acute involvement of LCH. Multiple hypodense nodules are identified, some of which confluent. No bile duct dilatation is present.

Figure 5

Magnification of axial contrast-enhanced abdominal CT: liver nodules can be best appreciated, some confluent. Note the hypodense area (fat attenuation values) of one of the nodules, indicating xanthomatous type lesion.

Figure 6

Photomicrographs (hematoxylin-eosin stain 100x magnification) of the liver biopsy specimens: normal hepatic parenchyma can be identified (circle) with cords of hepatocytes and normal portal spaces. Lymphocytic infiltration of the liver parenchyma can be identified (arrow) with some multinucleated cells (Langerhans cell).

Figure 7

Photomicrographs (hematoxylin-eosin (H-E) stain 200x magnification and CD1a immunolabeling) of the liver biopsy specimens: lymphocytic infiltrate is identified with multiple Langerhans cell intermixed that stain positive to CD1a, compatible with liver involvement of LCH.

Figure 8

Axial contrast-enhanced abdominal CT images: comparison of CT abdominal images from 2008 and 2010 after chemotherapy, demonstrating resolution of the liver nodules, common in acute liver involvement of LCH.