Neutrophil Activation and PSTPIP2 Loss Found in Pediatric CRMO
A study of blood samples from 34 patients with chronic recurrent multifocal osteomyelitis (CRMO) and 20 controls reported chronic neutrophil activation, evidenced by reduced CD62L expression that correlated with MRI lesion burden, and decreased PSTPIP2 protein despite increased mRNA, with no coding mutations identified. The study found that both neutrophil activation and PSTPIP2 downregulation normalized in patients receiving anti-TNF therapy, and transcriptomic profiling identified enrichment of NF-kB signaling and chemotaxis pathways along with several chemokine transcripts correlating with disease burden. The authors state these findings identify neutrophil activation and PSTPIP2 deficiency as central features of human CRMO and mechanistically explain the clinical benefit of TNF blockade.
Most relevant for: Pediatric patients with chronic recurrent multifocal osteomyelitis, particularly those being considered for or treated with anti-TNF therapy.
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