Hemophagocytic Lymphohistiocytosis in Adolescents and Young Adults: Genetic Predisposition and Secondary Disease

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a disorder of impaired immune regulation resulting in hyperinflammation that is ultimately fatal if not treated. HLH is categorized into familial disease, caused by genetic mutations affecting the function of cytotoxic T lymphocytes and natural killer cells, and secondary disease, triggered by infections, malignancies, rheumatologic disorders, or immune deficiency. Adolescent and young adults with HLH represent a unique population with specific diagnostic challenges. Here we review the diagnostic criteria, possible etiologies, pathophysiology, and management of HLH with focus on the adolescent population.

Keywords: Adolescents; Familial HLH; Hemophagocytic lymphohistiocytosis (HLH); Primary immunodeficiency; Secondary HLH; Young adults.