Increased serum monocyte chemoattractant protein-1, macrophage inflammatory protein-1β, and interleukin-8 concentrations in hemophagocytic lymphohistiocytosis

Background Hemophagocytic lymphohistiocytosis (HLH) is characterized by hypercytokinemia caused by macrophage and T cell activation. We analyzed the serum concentrations of monocyte chemoattractant protein (MCP)-1, macrophage inflammatory protein (MIP)-1β, and interleukin (IL)-8 to investigate the roles of these chemokines in the pathophysiology of HLH. Methods Seven patients clinically diagnosed with HLH were examined. Serum cytokines and chemokines were measured. The differences in the serum concentrations between the patients with HLH and the controls were investigated. Results In patients with an active phase of HLH, the serum MCP-1, MIP-1β, and IL-8 levels all were significantly higher than in healthy controls. The chemokine elevations decreased rapidly after initiation of chemotherapy. During increases in disease activity, elevation of MCP-1 and MIP-1β preceded elevation of the serum ferritin level, which is a clinical indicator of HLH disease activity. Conclusions These results suggest that MCP-1, MIP-1β, and IL-8 play important roles in the pathophysiology of HLH. In addition, the serum concentrations of these chemokines may be sensitive markers for assessing disease activity in patients with HLH. Pediatr Blood Cancer 2008;51:662–668. © 2008 Wiley-Liss, Inc.