CD14dim/CD16bright monocytes in hemophagocytic lymphohistiocytosis

✍ Scribed by Wolfgang Emminger; Gerhard J. Zlabinger; Gerhard Fritsch; Radvan Urbanek

• Book ID: 101385396
• Publisher: John Wiley and Sons
• Year: 2001
• Tongue: English
• Weight: 119 KB
• Volume: 31
• Category: Article
• ISSN: 0014-2980
• DOI: 10.1002/1521-4141(200106)31:6<1716::aid-immu1716>3.0.co;2-x

✦ Synopsis

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-1 g , IL-6 and TNF- § . 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.