TY - CHAP M1 - Book, Section TI - Hemophagocytic Lymphohistiocytosis (HLH) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Genetic disorder associated with failure to establish an effective immune response to infection. Individual affected with the disease shows clinical symptoms in the first months or years of life. Symptoms include severe infection with fever, hepato- and splenomegaly, cytopenia, and neurological abnormalities. Neurological features include irritability, fatigue, myotonia, seizures, neck stiffness, mental status changes, ataxia, blindness, paralysis, and/or coma. It is caused by normal but overactive histiocytes. Clinical features include fever, hepatomegaly, cytopenia, and neurologic abnormalities. It is a life-threatening medical condition when not treated. Two types have been described, either primary or secondary form. The primary, or “familial,” HLH condition is inherited. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164072397 ER -