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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.

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Summary

Storage pool deficiency (SPD), a platelet disorder, affects 8% of patients evaluated for bleeding issues. Specific tests are crucial, as some SPD patients show normal bleeding times and platelet aggregation, highlighting the need for advanced diagnostics.

Keywords:
Bleeding TimeBlood Platelet DisordersPlatelet Function TestsPlatelet Storage Pool Deficiency

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Area of Science:

  • Hematology
  • Platelet Physiology
  • Hemostasis

Background:

  • Platelet function disorders are a significant cause of bleeding diathesis.
  • Storage pool deficiency (SPD) is characterized by impaired platelet granule release.
  • SPD is increasingly recognized in patients with prolonged bleeding times and normal platelet aggregation.

Purpose of the Study:

  • To determine the prevalence of SPD in patients referred for hemostasis evaluation.
  • To identify diagnostic challenges in detecting SPD.
  • To assess the utility of specific platelet function tests.

Main Methods:

  • Screening of 215 patients with a history of clinical bleeding.
  • Diagnosis of SPD based on combined platelet ATP release and dense granule enumeration.
  • Analysis of bleeding times and platelet aggregation studies.

Main Results:

  • SPD was identified in 8% (17/215) of the studied patients.
  • Prevalence was 11% in patients with prolonged bleeding times and 6% in those with normal bleeding times.
  • Five SPD patients lacked typical bleeding abnormalities, underscoring the need for specific diagnostic tests.

Conclusions:

  • SPD is a relevant diagnosis in patients with bleeding disorders.
  • Standard bleeding time and platelet aggregation tests may not identify all SPD cases.
  • Combined assessment of platelet ATP release and dense granule function is essential for accurate SPD diagnosis.