title: Light Transmission Aggregometry Does Not Correlate With the Severity of δ-Granule Platelet Storage Pool Deficiency.
abstract: Delta-granule platelet storage pool deficiency (δ-PSPD) is a poorly studied bleeding diathesis resulting from either decreased granule content or decreased average number of platelet δ-granules. Light transmission aggregometry (LTA) is commonly used to evaluate for δ-PSPD and platelet electron microscopy (EM) is used to confirm the diagnosis. Currently, little data exist examining the relationship between the likelihood of abnormal platelet aggregation findings, severity of δ-granule deficiency on platelet EM, and severity of bleeding symptoms in patients with δ-PSPD. Patients diagnosed with δ-PSPD by platelet EM who also underwent LTA testing were identified at a single institution for correlation between severity of bleeding, average number of platelet δ-granules, and number of agonist abnormalities on LTA. No statistically significant association was identified between the average number of δ-granules per platelet and likelihood of an abnormal LTA. LTA abnormalities were quite varied and only 50% diagnosed with δ-PSPD on EM had abnormal aggregation testing. Also, no correlation was seen between the number of clinical bleeding symptoms, number of average δ-granules per platelet, and the number of LTA agonist abnormalities. Our findings highlight the difficulties inherent in the laboratory evaluation of platelet function.
authors.affiliation: *Division of Pediatric Hematology/Oncology, Children's Hospital of the King's Daughters, Norfolk, VA Departments of †Pediatrics ‡Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH.
authors.forename: Gary M
authors.initials: GM
authors.lastname: Woods
authors.forename: Elizabeth L
authors.initials: EL
authors.lastname: Kudron
authors.forename: Kyle
authors.initials: K
authors.lastname: Davis
authors.forename: Joseph
authors.initials: J
authors.lastname: Stanek
authors.forename: Bryce A
authors.initials: BA
authors.lastname: Kerlin
authors.forename: Sarah H
authors.initials: SH
authors.lastname: O'Brien
mesh_terms: D000244:Adenosine Diphosphate / Q000097:blood; D000255:Adenosine Triphosphate / Q000097:blood; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D010974:Platelet Aggregation; D010981:Platelet Storage Pool Deficiency / Q000097:blood*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D000244:Adenosine Diphosphate; D000255:Adenosine Triphosphate