Shows the platelet abnormalities in patients with trichohepatoenteric syndrome (THES). Thin section electron microscopy of a representative normal (A) and three different THES patients (B–D). The black bar represents 500 nm. Normal α-granules are observed in control platelets (A, white arrowheads) and occasionally in some THES platelets (B) but are frequently absent in others (C, D). Whereas the membranous surface-connected canalicular system appears normal in control platelets (A, white arrow), it was disrupted with prominent tubules and smaller membranous vesicles in THES patient platelets (B–D, white arrows). Lipid inclusions were frequently observed in THES platelets (B, black arrows). Electron dense lysosomal bodies fused with an α-granule were often seen (C, large white arrowhead).