Abstract
Interrelation between mononuclear phagocyte system (MPS) activation and hemostatic abnormality was investigated in 3 patients with hemophagocytic histiocytic syndrome (HH), a representative clinical model of MPS proliferative disorders, accompanied by disseminated intravascular coagulation syndrome (DIC) and multiple organ failure (MOF). A markedly significant decrease of plasma free protein S (PS) levels was found with markedly raised plasma C4b-binding protein (C4bp) levels as compared to non-HH DIC patients with MOF. And also an extreme increase of plasma plasminogen activator inhibitor 1 (PAI-1) and von Willebrand factor antigen (vWF: Ag) levels was found. Moreover, a significant elevation of serum cytokines, especially interleukin-1β and tumor necrosis factor α, was observed when compared to non-HH DIC patients with MOF. Plasma free PS was inversely correlated with these cytokines, while each of C4bp, PAI-1 and vWF: Ag was positively correlated with them.These results imply that markedly reduced plasma free PS and extremely increased plasma PAT-1 and vWF: Ag, which were possibly caused by the modulation of liver cells or vascular endothelial cells by these increased cytokines, would contribute to the pathogenesis of hemostatic abnormalities, particularly DIC and/or MOF in HH.
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