title: Early diagnostic and prognostic significance of a specific Th1/Th2 cytokine pattern in children with haemophagocytic syndrome.
abstract: The haemophagocytic syndrome (HPS) is a rare but frequently fatal disorder of immune regulation caused by hypercytokinemia. Using cytometric bead array technique, the serum T-helper cell type 1 (Th1) and 2 (Th2) cytokines including interferon-gamma (IFN-gamma), tumour necrosis factor (TNF), interleukin (IL)-10, IL-6, IL-4 and IL-2 were determined in 24 children with de novo HPS and 87 children as control. The median levels of serum IFN-gamma, IL-10 and IL-6 in the acute phase of HPS were 901.7, 879.0 and 63.8 pg/ml, respectively, significantly higher than those after remission, and in the healthy volunteers and patients with viral infection. IL-4 was slightly elevated while IL-2 and TNF were within normal range in acute phase. Patients with bacterial sepsis showed an extremely high level of IL-6 and moderate level of IL-10, whereas IFN-gamma was only slightly elevated. Five patients were diagnosed with HPS according to the Th1/Th2 cytokine pattern 3-13 d earlier than they fulfilled the relevant diagnostic criteria. IL-10 level >2000 pg/ml was an unfavorable prognostic factor for HPS treatment response (P = 0.033) and outcome (P = 0.009). We conclude that the significant increase of IFN-gamma and IL-10 and a slightly increased level of IL-6 is an early, specific and prognostic cytokine pattern for childhood HPS.
authors.affiliation: Division of Haematology-Oncology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China. y_m_tang@zju.edu.cn
authors.forename: Yongmin
authors.initials: Y
authors.lastname: Tang
authors.forename: Xiaojun
authors.initials: X
authors.lastname: Xu
authors.forename: Hua
authors.initials: H
authors.lastname: Song
authors.forename: Shilong
authors.initials: S
authors.lastname: Yang
authors.forename: Shuwen
authors.initials: S
authors.lastname: Shi
authors.forename: Jian
authors.initials: J
authors.lastname: Wei
authors.forename: Binhua
authors.initials: B
authors.lastname: Pan
authors.forename: Fenying
authors.initials: F
authors.lastname: Zhao
authors.forename: Chan
authors.initials: C
authors.lastname: Liao
authors.forename: Chunfang
authors.initials: C
authors.lastname: Luo
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D016207:Cytokines / Q000097:blood*; D005260:Female; D006801:Humans; D007223:Infant; D007371:Interferon-gamma / Q000276:immunology; D016753:Interleukin-10 / Q000276:immunology; D007376:Interleukin-2 / Q000276:immunology; D015847:Interleukin-4 / Q000276:immunology; D015850:Interleukin-6 / Q000276:immunology; D053208:Kaplan-Meier Estimate; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality; D008297:Male; D011379:Prognosis; D016019:Survival Analysis; D018417:Th1 Cells / Q000276:immunology*; D018418:Th2 Cells / Q000276:immunology*; D014409:Tumor Necrosis Factor-alpha / Q000276:immunology
chemical_list: D015415:Biomarkers; D016207:Cytokines; D007376:Interleukin-2; D015850:Interleukin-6; D014409:Tumor Necrosis Factor-alpha; D016753:Interleukin-10; D015847:Interleukin-4; D007371:Interferon-gamma