Early diagnostic and prognostic significance of a specific Th1/Th2 cytokine pattern in children with haemophagocytic syndrome
- 5 September 2008
- journal article
- Published by Wiley in British Journal of Haematology
- Vol. 143 (1), 84-91
- https://doi.org/10.1111/j.1365-2141.2008.07298.x
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.Keywords
This publication has 32 references indexed in Scilit:
- Serum Levels of Thl/Th2 Cytokines, Angiogenic Growth Factors, and Other Prognostic Factors in Young Adult Patients with Hemophagocytic SyndromeLaboratory Hematology, 2006
- Rapid simultaneous measurement of multiple cytokines in childhood oncology patients with febrile neutropenia: increased interleukin (IL)‐8 or IL‐5 correlates with culture‐positive infectionBritish Journal of Haematology, 2005
- Interleukin-6, interleukin-8, and a rapid and sensitive assay for calcitonin precursors for the determination of bacterial sepsis in febrile neutropenic childrenPediatric Critical Care Medicine, 2005
- An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorderBlood, 2004
- Histiocyte disordersCritical Reviews in Oncology/Hematology, 2004
- Hemophagocytic Syndrome in Children: An Important Diagnostic Consideration in Fever of Unknown OriginClinical Infectious Diseases, 2003
- Interleukin-18 in Hemophagocytic LymphohistiocytosisLeukemia & Lymphoma, 2001
- Perforin Gene Defects in Familial Hemophagocytic LymphohistiocytosisScience, 1999
- Cytotoxicity mediated by T cells and natural killer cells is greatly impaired in perforin-deficient miceNature, 1994
- Prognosis of Children with Virus-Associated Hemophagocytic Syndrome and Malignant Histiocytosis: Correlation with Levels of Serum Interleukin-1 and Tumor Necrosis FactorActa Haematologica, 1991