Hemophagocytic lymphohistiocytosis and other hemophagocytic disorders

Abstract

Hemophagocytic disorders result when critical regulatory pathways responsible for the natural termination of immune/inflammatory responses are disrupted or overwhelmed. Hemophagocytic disorders reflect pathologic defects that alter the normal crosstalk between innate and adaptive immune responses, and compromise homeostatic removal of cells that are superfluous or dangerous to the organism. Although hemophagocytic disorders are considered rare, increased awareness of these conditions has led to more frequent diagnoses, more rapid initiation of life-saving treatments, and new insights into the molecules and pathways involved in natural immune down-regulation. Furthermore, improved understanding of the immunologic abnormalities revealed by hemophagocytic disorders informs potential new treatments for life-threatening multisystem organ dysfunction related to sepsis in the intensive care unit setting and severe cases.