TY - JOUR T1 - Platelet diameters in inherited thrombocytopenias: analysis of 376 patients with all known disorders. JF - Blood Y1 - 2014 A1 - Noris, Patrizia A1 - Biino, Ginevra A1 - Pecci, Alessandro A1 - Civaschi, Elisa A1 - Savoia, Anna A1 - Seri, Marco A1 - Melazzini, Federica A1 - Loffredo, Giuseppe A1 - Russo, Giovanna A1 - Bozzi, Valeria A1 - Notarangelo, Lucia Dora A1 - Gresele, Paolo A1 - Heller, Paula G A1 - Pujol-Moix, NĂºria A1 - Kunishima, Shinji A1 - Cattaneo, Marco A1 - Bussel, James A1 - De Candia, Erica A1 - Cagioni, Claudia A1 - Ramenghi, Ugo A1 - Barozzi, Serena A1 - Fabris, Fabrizio A1 - Balduini, Carlo L KW - Adolescent KW - Adult KW - Blood Platelets KW - Case-Control Studies KW - Cell Size KW - Child KW - Child, Preschool KW - Diagnosis, Differential KW - Female KW - Hearing Loss, Sensorineural KW - Humans KW - Infant KW - Male KW - Middle Aged KW - Molecular Motor Proteins KW - Mutation KW - Myosin Heavy Chains KW - Purpura, Thrombocytopenic, Idiopathic KW - Thrombocytopenia KW - Young Adult AB -

Abnormalities of platelet size are one of the distinguishing features of inherited thrombocytopenias (ITs), and evaluation of blood films is recommended as an essential step for differential diagnosis of these disorders. Nevertheless, what we presently know about this subject is derived mainly from anecdotal evidence. To improve knowledge in this field, we evaluated platelet size on blood films obtained from 376 patients with all 19 forms of IT identified so far and found that these conditions differ not only in mean platelet diameter, but also in platelet diameter distribution width and the percentage of platelets with increased or reduced diameters. On the basis of these findings, we propose a new classification of ITs according to platelet size. It distinguishes forms with giant platelets, with large platelets, with normal or slightly increased platelet size, and with normal or slightly decreased platelet size. We also measured platelet diameters in 87 patients with immune thrombocytopenia and identified cutoff values for mean platelet diameter and the percentage of platelets with increased or reduced size that have good diagnostic accuracy in differentiating ITs with giant platelets and with normal or slightly decreased platelet size from immune thrombocytopenia and all other forms of IT.

VL - 124 IS - 6 U1 - http://www.ncbi.nlm.nih.gov/pubmed/24990887?dopt=Abstract ER -