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Br J Haematol. 2015 Aug;170(4):559-63. doi: 10.1111/bjh.13458. Epub 2015 Apr 21.

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

Author information

1
Department of Internal Medicine, University of Pavia-IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
2
Service of Biometry & Statistics, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
3
Universitat Autònoma de Barcelona & Institut de Recerca Biomèdica Sant Pau, Barcelona, Spain.
4
Ematologia, Policlinico Umberto 1, Università Sapienza, Rome, Italy.
5
Dipartimento di Scienze della Salute, Medicina III, Ospedale San Paolo, Università degli Studi di Milano, Milano, Italy.
6
AP-HP, Hôpital Bicêtre, Hématologie Biologique/Centre de Référence Pathologies Plaquettaires, Le Kremlin Bicêtre, France.
7
Department of Medicine, University of Perugia, Perugia, Italy.
8
Department of Medicine, University of Verona, Verona, Italy.
9
Plateforme Technologique et d'Innovation Biomédicale, Hôpital Xavier Arnozan, Pessac, France.
10
Unidad de Hemostasia y Trombosis, La Fe University Hospital, Valencia, Spain.
11
Department of Medicine and Department of Pathology & Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
12
Centre de Compétence Nord-Est des Pathologies Plaquettaires from the Frame of the Reference French Centre and Service d'Hématologie Biologique, Centre Hospitalo-Universitaire, Nancy, France.
13
Haematological Laboratory, AP-HP, Armand Trousseau Children Hospital, French Reference Centre for Inherited Platelet Disorders, Paris, France.
14
Inserm U1170, Villejuif, France.
15
Department of Cell Therapy and Haematology, San Bortolo Hospital, Vicenza, Italy.

Abstract

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.

KEYWORDS:

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

PMID:
25899604
DOI:
10.1111/bjh.13458
[Indexed for MEDLINE]

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