title: Usefulness of Flow Cytometric Mepacrine Uptake/Release Combined with CD63 Assay in Diagnosis of Patients with Suspected Platelet Dense Granule Disorder.
abstract: Dense granule disorder is one of the most common platelet abnormalities, resulting from dense granule deficiency or secretion defect. This study was aimed to evaluate the clinical usefulness of the flow cytometric combination of mepacrine uptake/release assay and CD63 expression detection in the management of patients with suspected dense granule disorder. Over a period of 5 years, patients with abnormal platelet aggregation and/or reduced adenosine triphosphate (ATP) secretion suggestive of dense granule disorder were consecutively enrolled. The flow cytometric assays were systematically performed to further investigate dense granule functionality. Among the 26 included patients, 18 cases showed impaired mepacrine uptake/release and reduced CD63 expression on activated platelets, consistent with δ-storage pool deficiency (SPD). Another seven patients showed decrease in mepacrine release and CD63 expression but mepacrine uptake was normal, indicating secretion defect rather than δ-SPD. Unfortunately, ATP secretion could not be measured in 7 out of the 26 patients due to insufficient sample and/or severe thrombocytopenia. This test combination provides a rapid and effective method to detect the heterogeneous abnormalities of platelet dense granule by distinguishing between storage and release defects. This combination is particularly advantageous for severely thrombocytopenic patients and pediatric patients in which only minimal sample is required.
authors: Service d'Hématologie Biologique, CHRU Nancy, Nancy, France.
authors: Huili
authors: H
authors: Cai
authors: Laboratory of Hematology, Namur Thrombosis and Hemostasis Center (NTHC), Namur Research Institute for Life Sciences, CHU Dinant Godinne UcL Namur, Yvoir, Belgium.
authors: François
authors: F
authors: Mullier
authors: Service d'Hématologie Biologique, CHRU Nancy, Nancy, France.
authors: Birgit
authors: B
authors: Frotscher
authors: Service d'Hématologie Biologique, CHRU Nancy, Nancy, France.
authors: Marie-Elisabeth
authors: ME
authors: Briquel
authors: Service d'Hématologie Biologique, CHRU Nancy, Nancy, France.
authors: Marie
authors: M
authors: Toussaint
authors: Plateforme de Cytométrie en flux, CHRU Nancy, Nancy, France.
authors: Frédéric
authors: F
authors: Massin
authors: Département des Spécialités de Médecine, Service d'Hématologie, Hôpitaux Universitaires de Genève, Suisse.
authors: Thomas
authors: T
authors: Lecompte
authors: Service d'Hématologie Biologique, CHRU Nancy, Nancy, France.
authors: Véronique
authors: V
authors: Latger-Cannard
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D000368:Aged; D001792:Blood Platelets / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005434:Flow Cytometry / Q000379:methods*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D015539:Platelet Activation; D010974:Platelet Aggregation; D010976:Platelet Count; D010979:Platelet Function Tests / Q000379:methods; D010981:Platelet Storage Pool Deficiency / Q000175:diagnosis* / Q000378:metabolism; D011796:Quinacrine / Q000378:metabolism* / Q000493:pharmacokinetics; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D060149:Tetraspanin 30 / Q000378:metabolism*; D055815:Young Adult
chemical_list: D060149:Tetraspanin 30; D000255:Adenosine Triphosphate; D011796:Quinacrine