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)-gamma, 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-gamma 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.