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Henter, J.I., Horne, A., Arico, M., Egeler, R.M., Filipovich, A.H., Imashuku, S., Ladisch, S., McClain, K., Webb, D., Winiarski, J., Janka, G., for the Histiocyte Society (2007) HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 48, 124-131. doi:10.1002/pbc.21039

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• TITLE: Fatal hemophagocytic lymphohistiocytosis presenting as Reye’s syndrome: Report of two cases

  AUTHORS: Shinji Kounami, Koichi Minami, Megumi Yoshiyama, Genkichi Izumi, Noriyuki Aoyagi, Norishige Yoshikawa

  KEYWORDS: Hemophagocytic Lymphohistiocytosis; Reye’s Syndrome; Cytokine; Mitochondria; Encephalopathy

  JOURNAL NAME: Open Journal of Pediatrics, Vol.2 No.2, June 19, 2012

  ABSTRACT: We report two cases of rapidly fatal hemophagocytic lymphohistiocytosis (HLH) that presented as Reye’s syndrome (RS). The patients were referred to our hospital because of altered level of consciousness with hyperammonemia and hypoglycemia. The first patient, a 24-month-old girl (Case 1), died soon after arrival and was clinically diagnosed as having RS, but a diagnosis of HLH was established on the basis of autopsy findings. The other patient, a 4-month-old boy (Case 2), was diagnosed as having HLH in view of the bone marrow findings on admission, but immunosuppressive therapy failed to prevent a rapidly fatal course. Marked hypercytokinemia was found in both patients, and liver pathology demonstrated panlobular microvesicular steatosis of hepatocytes, resembling that in RS. Mononuclear cell infiltration with hemophagocytosis in the liver was evident in case 2, but not in case 1. In both cases, hypercytokinemia was thought to have caused mitochondrial dysfunction, resulting in RS-like microvesicular steatosis of hepatocytes. HLH is an important differential diagnosis of children presenting with RS-like picture.