Platelet diameters in inherited thrombocytopenias: analysis of 376 patients with all known disorders

被引：108

作者：

Noris, Patrizia ^{[1
]}

Biino, Ginevra ^{[2
]}

Pecci, Alessandro ^{[1
]}

Civaschi, Elisa ^{[1
]}

Savoia, Anna ^{[3
,4
]}

Seri, Marco ^{[5
]}

Melazzini, Federica ^{[1
]}

Loffredo, Giuseppe ^{[6
]}

Russo, Giovanna ^{[7
]}

Bozzi, Valeria ^{[1
]}

Notarangelo, Lucia Dora ^{[8
]}

Gresele, Paolo ^{[9
]}

Heller, Paula G. ^{[10
]}

Pujol-Moix, Nuria ^{[11
]}

Kunishima, Shinji ^{[12
]}

Cattaneo, Marco ^{[13
]}

Bussel, James ^{[14
]}

De Candia, Erica ^{[15
]}

Cagioni, Claudia ^{[1
]}

Ramenghi, Ugo ^{[16
]}

Barozzi, Serena ^{[1
]}

Fabris, Fabrizio ^{[17
]}

Balduini, Carlo L. ^{[1
]}

机构：

[1] Univ Pavia, Dept Internal Med, Ist Ricovero & Cura Carattere Sci Policlin San Ma, I-27100 Pavia, Italy

[2] Natl Res Council Italy, Inst Mol Genet, Pavia, Italy

[3] Univ Trieste, Dept Med Sci, Trieste, Italy

[4] Ist Ricovero & Cura Carattere Sci Burlo Garofolo, Inst Maternal & Child Health, Trieste, Italy

[5] Univ Bologna, Policlin St Orsola Malpighi, Dipartimento Sci Med Chirurg, Bologna, Italy

[6] Pausilipon Hosp, Azienda Santobono Pausilipon, Dept Oncol, Naples, Italy

[7] Univ Catania, Div Pediat Hematol Oncol, Catania, Italy

[8] Spedali Civil Brescia, Osped Bambini, Oncol Haematol & Bone Marrow Transplantat Unit, I-25125 Brescia, Italy

[9] Univ Perugia, Dept Med, Sect Internal & Cardiovasc Med, I-06100 Perugia, Italy

[10] Univ Buenos Aires, Natl Sci & Tech Res Council, Inst Invest Med Alfredo Lanari, Dept Hematol Res, Buenos Aires, DF, Argentina

[11] Univ Autonoma Barcelona, Inst Recerca Biomed St Pau, E-08193 Barcelona, Spain

[12] Natl Hosp Org Nagoya Med Ctr, Clin Res Ctr, Dept Adv Diag, Nagoya, Aichi, Japan

[13] Univ Milan, Dipartimento Sci Salute, Med Osped San Paolo 3, Milan, Italy

[14] Cornell Univ, Weill Med Coll, New York, NY 10021 USA

[15] Univ Cattolica Sacro Cuore, Policlin Agostino Gemelli, Ist Med Interna & Geriatria, Serv Malattie Emorrag & Trombot, Rome, Italy

[16] Univ Turin, Dept Pediat, Hematol Unit, I-10124 Turin, Italy

[17] Univ Padua, Sch Med, Dept Med, Padua, Italy

来源：

BLOOD | 2014年 / 124卷 / 06期

关键词：

ELTROMBOPAG; SIZE;

D O I：

10.1182/blood-2014-03-564328

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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 immunethrombocytopenia 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.

引用

页码：E4 / E10

页数：7

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