Smita Ramachandran1, Fauzia Zaidi2, Archana Aggarwal3, Rani Gera4. 1. Department of Pediatrics, Safdarjung Hospital, Mahatma Gandhi Marg, Raj Nagar, Safdargunj, Ansari Nagar West, New Delhi, Delhi 110029, India. Electronic address: smita_rama25@yahoo.com. 2. Department of Pediatrics, Safdarjung Hospital, Mahatma Gandhi Marg, Raj Nagar, Safdargunj, Ansari Nagar West, New Delhi, Delhi 110029, India. Electronic address: fauzia.zaidi@gmail.com. 3. Department of Pediatrics, Safdarjung Hospital, Mahatma Gandhi Marg, Raj Nagar, Safdargunj, Ansari Nagar West, New Delhi, Delhi 110029, India. Electronic address: archana1972bansal@gmail.com. 4. Department of Pediatrics, Safdarjung Hospital, Mahatma Gandhi Marg, Raj Nagar, Safdargunj, Ansari Nagar West, New Delhi, Delhi 110029, India. Electronic address: ranigera@rediffmail.com.
Abstract
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by excessive activation of macrophages and T cells resulting from defective cytotoxicity. It is potentially life threatening due to the large amounts of cytokines released by the activated macrophages and lymphocytes secondary to a hyperinflammatory response. It has a high fatality in children with an incidence of approximately 1.2cases/million per year. METHOD: The literature was extensively searched in PubMed, MEDLINE and Google scholar. RESULTS: A variable and nonspecific symptomatology can delay the diagnosis and hence requires a high index of suspicion in both primary and secondary HLH.
BACKGROUND:Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by excessive activation of macrophages and T cells resulting from defective cytotoxicity. It is potentially life threatening due to the large amounts of cytokines released by the activated macrophages and lymphocytes secondary to a hyperinflammatory response. It has a high fatality in children with an incidence of approximately 1.2cases/million per year. METHOD: The literature was extensively searched in PubMed, MEDLINE and Google scholar. RESULTS: A variable and nonspecific symptomatology can delay the diagnosis and hence requires a high index of suspicion in both primary and secondary HLH.
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