Ultrastructural observation of platelets from patients with progressive systemic sclerosis (PSS)

We observed the ultrastructure of platelets from patients with PSS (7 cases; 48.2 +/- 12.3 y-old; M:F = 1:6_ and healthy controls (HC) (5 cases; 44.8 +/- 8.0 y-old; M:F = 1:4) by using transmission (TEM) and freeze-fracture electron microscopy (FEM). The open canalicular system (OCS) connected with the plasma membrane (PM) formed pinhole-like invaginations (50 nm in diameter) in the cleaved face (P-face) of the plasma membrane seen from the outside of the platelets and sharply elevated structures in the cleaved face (E-face) of PM seen from the inside of the platelets by FEM. The density of OCS on the surface of the platelets from PSS patients was 3 +/- 1/micron 2, which was higher than that from HC (1 +/- 0.5/micron 2) (p < 0.02). Dome-shaped structures, which clearly differ from OCS and were 80-150 nm in diameter without intramembranous particles, were seen in the P-face, and the complementary depressed structures were seen in the E-face. These structures were thought to be vesicles fused onto the PM of the platelets. The total volume of platelets (7.62 +/- 0.11 micron 3), total volume of granules (0.79 +/- 0.01 micron 3) and vacuoles including OCS (0.78 +/- 0.05 micron 3), and the total surface area of platelets (17.25 +/- 1.30 micron 2) from four PSS patients calculated by the morphometrical method were similar to those from four HC (7.32 +/- 0.25 micron 3, 0.76 +/- 0.03 micron 3, 0.80 +/- 0.05 micron 3, 18.75 +/- 0.35 micron 2, respectively); there were no statistical significances between the data from PSS patients and HC. The total volumes of vacuoles in platelets from both PSS patients and HC significantly decreased after a 2 min-vibration stress of the hands (p < 0.02) and the total volume of granules in platelets from PSS patients decreased significantly after the same stress (p < 0.002), although that from HC showed no similar significant change. However, there were no statistically significant differences in total volume or total surface of platelets from PSS patients and HC after the stress. These data may suggest that depletion of granules occurred due to activation of platelets from PSS patients following a secretion of their proteins, because their plasma protein levels were elevated after the stress (Jpn J Dermatol, 98; 1205, 1988). Higher density of OCS on the surface of the platelets from PSS patients may play an important role in secretion of their proteins, although the detailed mechanism of secretion of specific proteins derived from platelet granules is still unknown. These ultrastructural abnormalities of platelets may correlate with some involvement of a platelet disorder and with a possible role for the activation of platelets from PSS patients.