Below: Charts showing the results of Lumi-aggregometry for a patient with HPS-1 (traces in black and a normal control (traces in blue \ purple). The bottom traces in each graph refer to ATP release and the top two refer to aggregation. In each instance there is virtually no ATP release from the platelets of the HPS-1 patient. In chart 1, the top trace (in black) illustrates the primary aggregation of platelets followed by disaggregation. The bottom trace is a flat line showing that ATP has NOT been released, either because there are no dense granules or there is a defect preventing the granules from releasing their contents. A similar flat line trace can also be seen in charts 1-3.
Left: Graph showing platelet aggregation response to ADP and Epinephrine. HPS patient in red showing primary aggregation in response to ADP followed by disaggregation