Platelet immunophenotyping in health and inherited bleeding disorders, a review and practical hints.

Cytometry. Part B, Clinical Cytometry
Marc FouassierMarie C Béné

Abstract

Inherited platelet function disorders are rare hemorrhagic diseases. The gold standard for their exploration is optical aggregometry; however, investigations by flow cytometry (FCM) are being increasingly used. In this review, the physiology of platelets is first recalled, setting the stage for the compartments of platelets that can be apprehended by specific and appropriate labeling. As this requires some pre-analytical precautions and specific analytical settings, a second part focuses on these characteristic aspects, based on literature and on the authors' experience in the field, for qualitative or quantitative explorations. Membrane labeling with antibodies to CD42a or CD41, respectively, useful to assess the genetic-related defects of Glanzmann thrombocytopenia and Bernard Soulier syndrome are then described. Platelet degranulation disorders are detailed in the next section, as they can be explored, upon platelet activation, by measuring the expression of surface P-Selectin (CD62P) or CD63. Mepacrin uptake and release after activation is another test allowing to explore the function of dense granules. Finally, the flip-flop anomaly related to Scott syndrome is depicted. Tables summarizing possible FCM assays, and characte...Continue Reading

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Citations

Sep 22, 2020·Platelets·Antoine BabutyMarc Fouassier
Nov 28, 2020·Cytometry. Part B, Clinical Cytometry·Frederic I Preffer
Jun 18, 2021·Hämostaseologie·Matthieu PersynMarc Fouassier
Sep 15, 2021·Annals of Hematology·Guillaume BonnardMarc Fouassier

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