Correction of the platelet adhesion defect in delta-storage pool deficiency at elevated hematocrit--possible role of adenosine diphosphate.
Previous studies on patients with storage pool deficiency (SPD) who are specifically deficient in platelet dense granules (delta-SPD) have suggested a role for dense granule substances, in all likelihood adenosine diphosphate (ADP), in mediating thrombus formation on subendothelium at high shear rates. The role of dense granule substances in mediating platelet adhesion appears to be more complicated Previous studies in delta-SPD suggested an adhesion defect that was strongly influenced by the patient's hematocrit (Hct) value. To explore further the possibility that red blood cells (RBCs) may influence the role that platelet storage granules play in mediating adhesion at high shear rates, we have measured adhesion (and thrombus formation) throughout a preselected range of Hct values (30% to 60%) in normal subjects and in patients with delta-SPD. The present studies confirm the defect in platelet adhesion in patients with delta-SPD, most significantly at Hct values of 30% to 40%. This defect (but not that of thrombus formation) can be completely corrected by the addition of RBCs. The correction of the platelet adhesion defect by RBCs was specific for delta-SPD; it was not observed in either von Willebrand's disease or thrombasthenia. Studies performed on normal blood under conditions that could be expected to block any effect of ADP on adhesion and an analysis of the type of adhesion defect in delta-SPD suggest that ADP may be involved in the process required for platelet spreading on the subendothelium. The corrective effect of RBCs on platelet adhesion in delta-SPD appears to be chemical rather than physical in nature, possibly due to shear-induced release of RBC ADP or to other recently described properties of RBCs that enhance collagen-induced platelet interactions.
Weiss
H J
HJ
Department of Medicine, St. Luke's-Roosevelt Hospital Center, New York, NY 10019, USA.
Lages
B
B
Hoffmann
T
T
Turitto
V T
VT
D000244:Adenosine Diphosphate / Q000097:blood / Q000502:physiology*; D000255:Adenosine Triphosphate / Q000097:blood; D000328:Adult; D001792:Blood Platelets / Q000737:chemistry; D001809:Blood Viscosity; D003594:Cytoplasmic Granules / Q000737:chemistry; D004730:Endothelium, Vascular / Q000503:physiopathology; D006400:Hematocrit; D018056:Hemorheology; D006801:Humans; D008875:Middle Aged; D010973:Platelet Adhesiveness / Q000502:physiology*; D010976:Platelet Count; D010981:Platelet Storage Pool Deficiency / Q000097:blood*; D012701:Serotonin / Q000097:blood; D013314:Stress, Mechanical; D013915:Thrombasthenia / Q000097:blood; D014842:von Willebrand Diseases / Q000097:blood
D012701:Serotonin; D000244:Adenosine Diphosphate; D000255:Adenosine Triphosphate