Rare macrophage diseases in adults

Abstract

Diseases of macrophages are rare entities characterized by the accumulation of macrophages, dendritic cells or monocyte-derived cells in various tissues and organs. This article focuses on macrophage disorders which can be present in adult patients. The review highlights pathogenesis, signs and symptoms, diagnostic criteria and principles of therapy in the most frequent macrophage diseases in adults: hemophagocytic lymphohistiocytosis, Rosai-Dorfman disease, Gaucher disease, Niemann-Pick disease, and Langerhans cell histiocytosis. Since macrophage disorders can be encountered by various medical specialists, basic knowledge of these entities and their diagnostic criteria should be familiar to all physicians, including hematologists.