Line-selective macrophage activation with an anti-CD40 antibody drives a hemophagocytic syndrome in mice.

ABSTRACT: Hemophagocytic syndromes comprise a cluster of hyperinflammatory disorders, including hemophagocytic lymphohistiocytosis and macrophage activation syndrome. Overwhelming macrophage activation has long been considered a final common pathway in the pathophysiology of hemophagocytic syndromes leading to the characteristic cytokine storm, laboratory abnormalities, and organ injuries that define the clinical spectrum of the disease. So far, it is unknown whether primary macrophage activation alone can induce the disease phenotype. In this study, we established a novel mouse model of a hemophagocytic syndrome by treating mice with an agonistic anti-CD40 antibody (Ab). The response in wild-type mice is characterized by a cytokine storm, associated with hyperferritinemia, high soluble CD25, erythr

SUBMITTER: Ingoglia G 

PROVIDER: S-EPMC7322947 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature