Open AccessPlatelet Aggregation Assays Do Not Reliably Diagnose Platelet Delta Granule Storage Pool Deficiency
Author(s) -
William T. Gunning,
Lorene Yoxtheimer,
Matthew R. Smith
Publication year - 2021
Publication title -
journal of hematology
Language(s) - English
Resource type - Journals
eISSN - 1927-1220
pISSN - 1927-1212
DOI - 10.14740/jh832
Subject(s) - platelet , medicine , ristocetin , dense granule , adenosine diphosphate , epinephrine , granule (geology) , platelet disorder , bleeding time , platelet aggregation , whole blood , immunology , gastroenterology , biology , paleontology
Patients with platelet dysfunction disorders present with a variety of mucocutaneous bleeding symptoms including easy bruising, frequent epistaxis, bleeding gums upon tooth brushing and for women, heavy menstrual bleeding. Available laboratory assays to evaluate platelet function include the platelet function analyzer (PFA) and in larger centers with coagulation laboratories, light transmission platelet aggregometry (LTA) analyses. Both assays are known to have a number of limitations, especially in the diagnosis of platelet delta granule storage pool deficiency (δ-SPD). δ-SPD is an underdiagnosed condition caused by decreased numbers of platelet dense granules (DGs) and is best diagnosed by electron microscopy (EM). Patients with platelet δ-SPD have a decreased response to low levels of the agonist adenosine diphosphate (ADP) in the second wave of light transmittance with LTA or decreased ADP secretion by fluorescence lumiaggregometry. There are few reports that have evaluated patients with δ-SPD and their respective LTA results. One report published in 1987 described normal LTA assays in 23% of patients with δ-SPD; a more recent report described LTA as having the sensitivity to detect only about 52% of patients with δ-SPD. The purpose of our study was intended to review the LTA and EM results of patients suspected of having a platelet function disorder at our institution for comparison with previously published studies.