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.
Highlights
Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation and migration of dendritic antigenpresenting histiocytes
No other abdominal abnormalities were found (Figures 4 and 5). These findings suggested Langerhans’ cell histiocytosis with pulmonary and hepatic involvement
No liver nodules were found in follow-up computed tomography (CT) (Figure 8)
Summary
Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation and migration of dendritic antigenpresenting histiocytes. The typical form is more common in females (2/1.5) and usually affects children between 1 and 3 years of age [1]. Multisystem, defined as involvement of two or more organs with or without dysfunction, is subdivided into “low-risk” and “highrisk” groups. Absence of “risk” organs (liver, lungs, spleen, and hematopoietic system) involvement characterizes “lowrisk” patients “High-risk” patients (80%) have at least one risk organ involved and a high mortality rate [3]. Frequency of liver involvement is known to be high (19– 60%) in children and bears a poor prognosis [4, 5]. Liver involvement is rare and is poorly understood, though it is an important cause of morbidity and mortality
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