Adult langerhans cell histiocytosis

Abstract

Most of the knowledge regarding the diagnosis and treatment of adult Langerhans cell histiocytosis (LCH) patients is derived from pediatric studies. Adult LCH patients are often evaluated by physicians who focus only on the most obviously affected organ without sufficient evaluation of other systems, which may lead to an erroneous diagnosis in some patients. In addition, treatment with pediatric-based regimens, often referred to as standard therapy, seems to be less effective and sometimes more toxic in adults. The published literature on adult LCH lacks a comprehensive discussion on the differences between pediatric and adult patients, and it is still debatable what and how much can be extrapolated from the younger age cohort. Isolated primary pulmonary LCH, in almost all cases associated with smoking, is a common presenting pattern in adults and has particular clinico-radiographic and treatment features that warrant its characterization as a separate entity.