CD14(dim)/CD16(bright) monocytes in hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is an extremely rare and highly lethal chronic inflammatory disease, which is mediated by proinflammatory cytokines. In the peripheral blood of a boy suffering from HLH, a chronic expansion of CD14(dim)/CD16(bright) inflammatory monocytes was detected. Compared with CD14(bright) monocytes, their immunophenotype correlated with more mature monocytic cells differentiating to macrophages: they showed lower expression of CD11b, CD64 and CD35. Such CD14(dim)/CD16(bright) monocytes produce the inflammatory cytokines IL-1beta, IL-6 and TNF-alpha. They fit in well with the pathophysiological concept of HLH as an inflammatory state of lymphocytes and of the monocyte/macrophage system. In the presented patient the percentage of these circulating inflammatory monocytes decreased over time during clinical response to immunosuppressive therapy. This finding may indicate that CD14(dim)/CD16(bright) monocytes represented the degree of inflammation in this extremely rare and highly lethal disease.