Mitochondrial defects lie at the basis of neutropenia in Barth syndrome

It seems unlikely that neutropenia in BTHS is caused by apoptosis of the myeloid precursor cells or end-stage neutrophils. Instead, mitochondria-derived reactive oxygen species may act as signaling intermediates that trigger phosphatidyl serine exposure. This, in turn, appears to lead to increased clearance of neutrophils by tissue macrophages.