Table 4.
Maximal aggregation (%) of patients and controls
| Platelet count (×10 9 L −1 ) | TRAP (25 μM) | ADP (10 μM) | Ristocetin (1.25 mg mL −1 ) | Arachidonic acid (1.6 mM) | Collagen (2 μg mL −1 ) | PMA (100 nM) | |
|---|---|---|---|---|---|---|---|
| GT (n = 13) | 171.7 ± 42.5 | 18.3 ± 4.5 | 3.0 ± 5.2 | 74.1 ± 24.7 | 9.8 ± 5.0 | 8.2 ± 9.6 | 8.8 ± 4.4 |
| BSS (n = 8) | * | 49.4 ± 21.1 | - | 4.4 ± 2.5 | - | - | - |
| SSD (n = 19) | 197.6 ± 77.8 | 80.7 ± 11.2 | 53.9 ± 27.0 | 84.6 ± 17.8 | 72.1 ± 25.8 | 45.7 ± 36.4 | - |
| Parallel controls (n = 31)‡ | 216.3 ± 48.9 | 83.7 ± 12.7 | 78.1 ± 16.8 | 80.3 ± 19.4 | 77.4 ± 23.3 | 78.0 ± 18.6 | 45.2 ± 22.3# |
| Fresh controls (n = 40) | 210.6 ± 32.4 | 90.7 ± 7.8 | 86.3 ± 7.3 | 90.9 ± 7.1 | 88.8 ± 6.6 | 88.1 ± 6.8 | 58.5 ± 20.4# |
The Table reflects aggregation responses of patients diagnosed with GT, BSS, SSD, parallel controls (drawn simultaneously to patients), and controls drawn at the time of analysis. All values are mean ± standard deviation. *Always <20 × 109 platelet L−1; #Aggregation responses to PMA were assessed in controls only when the paired patient’s aggregation profile was consistent with GT. ‡Blood samples from nine patients diagnosed with secretion and signal transduction platelet defects were drawn at our institution, and in those cases blood specimens were only collected from fresh (not parallel) controls.
Abbreviations: ADP Adenosine diphosphate, BSS Bernard Soulier Syndrome, GT Glanzmann Thrombasthenia, TRAP thrombin receptor activating peptide, PMA phorbol myristate acetate, SSD platelet secretion and signal transduction defects.