Analysis of 65 pregnancies in 34 women with five different forms of inherited platelet function disorders

被引：32

作者：

Civaschi, Elisa ^{[1
]}

Klersy, Catherine ^{[2
]}

Melazzini, Federica ^{[1
]}

Pujol-Moix, Nuria ^{[3
,4
]}

Santoro, Cristina ^{[5
]}

Cattaneo, Marco ^{[6
]}

Lavenu-Bombled, Cecile ^{[7
]}

Bury, Loredana ^{[8
]}

Minuz, Pietro ^{[9
]}

Nurden, Paquita ^{[10
]}

Cid, Ana R. ^{[11
]}

Cuker, Adam ^{[12
,13
]}

Latger-Cannard, Veronique ^{[14
]}

Favier, Remi ^{[15
,16
]}

Nichele, Ilaria ^{[17
]}

Noris, Patrizia ^{[1
]}

机构：

[1] Univ Pavia, IRCCS Policlin San Matteo Fdn, Dept Internal Med, I-27100 Pavia, Italy

[2] IRCCS Policlin San Matteo Fdn, Serv Biometry & Stat, Pavia, Italy

[3] Univ Autonoma Barcelona, E-08193 Barcelona, Spain

[4] Inst Recerca Biomed St Pau, Barcelona, Spain

[5] Univ Roma La Sapienza, Policlin Umberto 1, Ematol, Rome, Italy

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

[7] Hop Bicetre, AP HP, Hematol Biol Ctr Reference Pathol Plaquettaires, Le Kremlin Bicetre, France

[8] Univ Perugia, Dept Med, I-06100 Perugia, Italy

[9] Univ Verona, Dept Med, I-37100 Verona, Italy

[10] Hop Xavier Arnozan, Plateforme Technol & Innovat Biomed, Pessac, France

[11] La Fe Univ Hosp, Unidad Hemostasia & Trombosis, Valencia, Spain

[12] Univ Penn, Perelman Sch Med, Dept Med, Philadelphia, PA 19104 USA

[13] Univ Penn, Perelman Sch Med, Dept Pathol & Lab Med, Philadelphia, PA 19104 USA

[14] Ctr Hosp Univ, Ctr Competence Nord Est Pathol Plaquettaires Fram, Reference French Ctr & Serv Hematol Biol, Nancy, France

[15] Armand Trousseau Children Hosp, AP HP, Haematol Lab, French Reference Ctr Inherited Platelet Disorders, Paris, France

[16] Inserm U1170, Villejuif, France

[17] San Bortolo Hosp, Dept Cell Therapy & Haematol, Vicenza, Italy

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2015年 / 170卷 / 04期

关键词：

inherited platelet disorders; bleeding diathesis; pregnancy; perinatal haemostasis; bleeding risk;

D O I：

10.1111/bjh.13458

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

This study evaluated 65 pregnancies in 34 women with five different inherited platelet function disorders. Gestation was similar to that of the general population. Severe bleeds requiring blood transfusions were observed in 50% of deliveries in Glanzmann thrombasthenia (GT), but not in the patients with delta storage pool disease, Hermansky-Pudlak syndrome, P2Y12 defect or defect of thromboxane A2 receptor. Of note, severe haemorrhage also occurred in women with GT who had received prophylactic platelet transfusions, suggesting that better preventive treatments are required. Diagnosis and degree of spontaneous bleeding tendency before pregnancy were reliable parameters to predict the delivery-related bleeding risk.

引用

页码：559 / 563

页数：5

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