Abstract

In 3 patients with hemophagocytic histiocytosis (HH) 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. Moreover, a significant elevation of serum cytokines, interleukin-lβ and tumor necrosis factorα, was observed when compared to non-HH DIC patients with MOF. Plasma free PS was inversely correlated with these two cytokines, while plasma C4bp was positively correlated with them.These results imply that abnormal plasma PS distribution, that is, markedly reduced free PS with raised C4bp-PS complex which was closely associated with hypercytokinemia would contribute to the pathogenesis of DIC and/or MOF in HH.

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