Usefulness of Flow Cytometric Mepacrine Uptake/Release Combined with CD63 Assay in Diagnosis of Patients with Suspected Platelet Dense Granule Disorder.

Autor:	Cai H; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France., Mullier F; Laboratory of Hematology, Namur Thrombosis and Hemostasis Center (NTHC), Namur Research Institute for Life Sciences, CHU Dinant Godinne UcL Namur, Yvoir, Belgium., Frotscher B; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France., Briquel ME; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France., Toussaint M; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France., Massin F; Plateforme de Cytométrie en flux, CHRU Nancy, Nancy, France., Lecompte T; Département des Spécialités de Médecine, Service d'Hématologie, Hôpitaux Universitaires de Genève, Suisse., Latger-Cannard V; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France.
Jazyk:	angličtina
Zdroj:	Seminars in thrombosis and hemostasis [Semin Thromb Hemost] 2016 Apr; Vol. 42 (3), pp. 282-91. Date of Electronic Publication: 2016 Feb 12.
DOI:	10.1055/s-0035-1564836
Abstrakt:	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. (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)
Databáze:	MEDLINE
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