Table 2. Platelet function disorder subgroups (described in Adler et al 16 ) .
Disorder | Possible PFD ( n = 64) | Confirmed PFD ( n = 54) |
---|---|---|
Numbers (%) | ||
Glanzmann's thrombasthenia a | 0 | 4 (7.4) |
Gi-like defects b | 20 (31.3) | 18 (33.3) |
TxA2 pathway defects c | 13 (20.3) | 1 (1.9) |
Collagen receptor defects d | 6 (9.4) | 2 (3.7) |
Dense granule disorders e | 1 (1.6) | 4 (7.4) |
α-granule disorders f | 1 (1.6) | 5 (9.3) |
Diminished procoagulant COAT platelets g | 3 (4.7) | 7 (13.0) |
Complex disorders h | 20 (31.3) | 13 (24.1) |
Abbreviations: ADP, adenosine diphosphate; PFD, platelet function disorders; TxA2, thromboxane A2. PFD subgroups were defined as follows.
Defect in GPIIb/IIIa associated with a severely diminished aggregation of all agonists except ristocetin, reduced expression of GPIIb/IIIa, and/or markedly reduced activation of PAC1-binding. 1 3 4 26
Accentuated deficiency in aggregation to the Gi-coupled receptor antagonists ADP and adrenaline, associated with corresponding flow cytometry results. 1 3 26
Absent aggregation in response to arachidonic acid, and possibly associated with an impaired response to other agonists. 1 3 4 24 26
Isolated reduction in aggregation and secretion after stimulation with collagen and convulxin. 1 21 26
Reduced expression and/or secretion of P-selectin, associated with varying impaired aggregation after stimulation with collagen and epinephrine. 1 21
Impaired binding of Annexin-V after incubation with convulxin and thrombin. 21
Defects in a number of agonists (LTA) and/or several flow cytometry results that cannot be attributed to any of the disorders mentioned above.