Current opinion in allergy and clinical immunology
-
Curr Opin Allergy Clin Immunol · Dec 2006
ReviewHemophagocytic lymphohistiocytosis and related disorders.
The rate of diagnosis of hemophagocytic lymphohistiocytosis, a genetically heterogeneous and, frequently, rapidly fatal autosomal recessive disorder of immune regulation, is increasing worldwide. Awareness has grown through the Histiocyte Society and the publication of newly-recognized genetic causes. I summarize current knowledge regarding the pathophysiology, diagnosis and treatment of hemophagocytic lymphohistiocytosis. ⋯ Bench and clinical research have advanced understanding of the pathophysiology of hemophagocytic lymphohistiocytosis and related disorders, and significantly improved clinical outcomes during the past decade.