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

HLH is a rare disease which is fatal without treatment and maintaining a high index of suspicion in patients with evidence of hyperinflammation is of utmost importance. With increasing knowledge of the genetic bases of the disease and the understanding that familial HLH is not a disease exclusive of infants, adolescents with HLH should undergo genetic evaluation for familial HLH. Primary immune defi-ciencies and dysregulation disorders should also be considered simultaneously with evaluation for other secondary causes. Prompt diagnosis and initiation of treat-ment is needed to prevent morbidity and mortality related to the hyperinflammation of HLH.

Automated summary

HLH is a rare but fatal disease, and maintaining a high level of suspicion is crucial for patients with evidence of hyperinflammation. Adolescents with HLH should undergo genetic evaluation for familial HLH, and primary immune deficiencies and dysregulation disorders should also be considered. Early diagnosis and treatment are necessary to prevent morbidity and mortality related to HLH's hyperinflammation.