ECMO-Dependent Fulminant Melioidosis from Caspase 4 Mutation Reversed by Interferon Gamma Therapy.

We describe bedside-to-bench immunological and genetic elucidation of defective pyroptosis attributable to novel Caspase 4 defect mediating pathogen-triggered inflammatory programmed cell death, in setting of severe pneumonia and abscess-forming melioidosis in an overtly healthy host failing to clear Burkholderia pseudomallei, and how targeted adjunctive biological therapy led to successful outcome.