Hypercytokinemia in Hemophagocytic Syndrome

Purpose The study was performed to clarify in the hemophagocytic syndrome (HPS) how cytokinemia plays a role in its pathogenesis and if cytokinemia is of prognostic value. Patients and Methods Serum concentrations of ferritin, interferon (IFN)-y, soluble interleukin-2 (IL-2) receptor, IL-6, and other cytokines were determined during the acute phase of the HPS in 29 children and three adults. Data comparing malignancy-associated (MAHS; n = 17) and infection-associated hemophagocytic syndrome (IAHS; n = 15) and those comparing surviving and fatal cases were assessed. Results Hyperferritinemia and hypercytokinemia were present in all patients with HPS. Eleven of the 17 MAHS and three of the 15 IAHS cases were fatal (p < 0.05). No significant difference in cytokine concentrations was observed between MAHS and IAHS. Conclusions In terms of cytokine effect on patient outcome, serum concentrations of IL-6 >300 ng/L and IFN-γ concentrations >30 U/ml or soluble IL-2 receptor (sIL-2R) concentrations >10,000 U/ml were considered to reflect a poor prognosis in HPS patients.