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Published on: November 11, 2011
Identification of Patients with Storage Pool Deficiency Using ATP Release and Dense Granule Counts
S J Israels1,2, C Robertson2, A Mcnicol3
1a Depts. of Pediatrics University of Manitoba Winnipeg , Manitoba.
Recent reports have suggested that a significant number of patients with long bleeding times and normal platelet aggregation have storage pool deficiency (SPD). The present study screened 215 patients referred for evaluation of haemostasis because of a clinical bleeding history. SPD was identified by the presence of combined abnormalities in platelet ATP release and electron microscopic dense granule enumeration. Eight percent (17/215) of the patients studied were found to have SPD. Eleven percent (9/80) of patients with prolonged bleeding times had SPD, compared to 6% (8/135) of those with normal bleeding times. Two patients were identified as having both SPD and von Willebrand Disease. Although 12 of 17 SPD patients had prolonged bleeding times and/or abnormal platelet aggregation studies, five patients had neither abnormality and would not have been identified without the more specific tests for dense granule number and function.
Recent reports have suggested that a significant number of patients with long bleeding times and normal platelet aggregation have storage pool deficiency (SPD). The present study screened 215 patients referred for evaluation of haemostasis because of a clinical bleeding history. SPD was identified by the presence of combined abnormalities in platelet ATP release and electron microscopic dense granule enumeration. Eight percent (17/215) of the patients studied were found to have SPD. Eleven percent (9/80) of patients with prolonged bleeding times had SPD, compared to 6% (8/135) of those with normal bleeding times. Two patients were identified as having both SPD and von Willebrand Disease. Although 12 of 17 SPD patients had prolonged bleeding times and/or abnormal platelet aggregation studies, five patients had neither abnormality and would not have been identified without the more specific tests for dense granule number and function.
