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Hiroshi Tsujimoto, Shinji Kounami, Takayuki Ichikawa, Taketsugu Hama, Hiroyuki SuzukiDepartment of Pediatrics, Wakayama Medical University, Wakayama, JapanCorrespondence: Hiroshi TsujimotoDepartment of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City 641-8509, JapanTel +81 73 441 0633Fax +81 73 444 9055Email tujimoto@wakayama-med.ac.jpPurpose: Hemophagocytic lymphohistiocytosis (HLH) is a potentially lethal hyperinflammatory disorder. For further understanding of the pathogenesis of HLH, we examined serum levels of high mobility group box protein 1 (HMGB1) in children with HLH.Patients and methods: Serum HMGB1 levels were measured in 28 patients with HLH and 6 normal controls using a quantitative enzyme-linked immunosorbent assay. The patients were 21 boys and 7 girls, aged from 10 days to 21 years, with a median age of 8.5 years. The underlying conditions of HLH were infection-associated HLH in 18 patients, malignancy-associated HLH in 7 patients, and genetic HLH in 3 patients. The relations between serum HMGB1 levels and clinical symptoms and laboratory parameters were analyzed.Results: Serum HMGB1 levels were significantly higher in patients with HLH than in normal controls (median, 6.5 ng/mL, interquartile range, 4.25–13.1). The serial serum HMGB1 levels in one patient fell to reflect the disease activity. Serum HMGB1 levels were significantly higher in patients with disseminated intravascular coagulation (DIC) than in patients without DIC (p