Hemophagocytic Lymphohistiocytosis in Adolescents and Young Adults

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.