Pulmonary Langerhans Cell Histiocytosis and Other Histiocytic Lung Diseases

Histiocytic neoplasms represent a rare and heterogeneous group of disorders that include primarily Langerhans cell histiocytosis, Erdheim–Chester disease and Rosai–Dorfman disease, out of which Langerhans Cell Histiocytosis is the most common disorder to affect the lung. However, each of these entities have different respiratory manifestations thus posing to be a diagnostic challenge to the clinician with consequent delays in treatment. Recent advances in understanding the pathology of these histiocytic neoplasms have resulted in the development of targeted therapies with significant clinical implications. This book chapter will review the different histiocytic neoplasms, their clinical manifestations and management which will help improve the awareness and help early recognition of these rare lung disorders.