In MDS cells, sivelestat, a neutrophil elastase inhibitor, increases the expression of PUMA and DNA double-strand breaks and activates caspase-3, which indicates that sivelestat can downregulate HMGB1 and suppress the TLR and NF-κB pathways to promote apoptosis in the BM [99]. Here, HMGB1 is linked to myelodysplastic syndrome.