RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164072397 T1 Hemophagocytic Lymphohistiocytosis (HLH) T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164072397 RD 2024/03/28 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.