title: A review of platelet secretion assays for the diagnosis of inherited platelet secretion disorders.
abstract: Measurement of platelet granule release to detect inherited platelet secretion disorders (IPSDs) is essential for the evaluation of patients with abnormal bleeding and is necessary to distinguish which granule sub-types are affected and whether there is abnormal granule bio-synthesis or secretion. The radioactive serotonin incorporation and release assay, described before 1970, is still considered the "gold standard" test to assess platelet δ-granule release, although is unsuitable for clinical diagnostic laboratories. Luciferin-based assays, such as lumiaggregometry, are the most widely performed alternatives, although these methods do not distinguish defects in δ-granule biosynthesis from defects in secretion. Platelet α-granule release is commonly evaluated using flow cytometry by measuring surface exposure of P-selectin after platelet activation. However, this assay has poor sensitivity for some α-granule disorders. Only few studies have been published with more recently developed assays and no critical reviews on these methods are available. In this review, we describe the rationale for developing robust and accurate laboratory tests of platelet granule release and describe the characteristics of the currently available tests. We identify an unmet need for further systematic evaluation of new assays and for standardisation of methodologies for clinical diagnostic laboratories.
authors: Andrew D
authors: AD
authors: Mumford
authors: Andrew L
authors: AL
authors: Frelinger
authors: Christian
authors: C
authors: Gachet
authors: Paolo
authors: P
authors: Gresele
authors: Patrizia
authors: P
authors: Noris
authors: Paul
authors: P
authors: Harrison
authors: Diego Mezzano, MD, Department of Hematology-Oncology, School of Medicine, P. Universidad Católica de Chile, Portugal 61, 2nd Floor, Santiago, Chile, Tel.: +56 2 2354 3774, Fax: +56 2 354 3776, E-mail: dmezzano@med.puc.cl.
authors: Diego
authors: D
authors: Mezzano
mesh_terms: D015415:Biomarkers / Q000097:blood; D001791:Blood Platelet Disorders / Q000097:blood / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D001792:Blood Platelets / Q000378:metabolism*; D003594:Cytoplasmic Granules / Q000378:metabolism*; D005089:Exocytosis; D020022:Genetic Predisposition to Disease; D040941:Heredity; D006801:Humans; D010641:Phenotype; D010979:Platelet Function Tests / Q000592:standards; D011237:Predictive Value of Tests; D015203:Reproducibility of Results
chemical_list: D015415:Biomarkers
keywords: Platelet pathology; acquired; diagnosis management; exocytosis; inherited; secretion