title: A systematic review of the management and outcomes of pregnancy in Glanzmann thrombasthenia.
abstract: Glanzmann Thrombasthenia (GT) is a rare autosomal recessive disorder which usually manifests as severe mucocutaneous bleeding and is caused by deficiency of the platelet glycoprotein IIb-IIIa. Pregnancy in women with GT presents particular challenges as there is increased risk of both maternal and foetal bleeding. To improve understanding and clarify the optimum management of pregnancy in this disorder, we performed a systematic review of the world literature of pregnancy and GT. This identified three single-centre case series of patients with GT that included brief descriptions of women in pregnancy and 31 detailed case reports of 40 pregnancies in 35 women that resulted in 38 live births. Among the detailed case reports, ante-natal bleeding was described in 50% of pregnancies but was usually mild and occurred at mucocutaneous sites. Primary postpartum haemorrhage (PPH) was reported in 34% of pregnancies and secondary PPH in 24%. PPH was frequently severe and occurred up to 20 days after delivery. There was a wide variation in approach to prevention and treatment of PPH but most women received platelet transfusion, sometimes with additional recombinant FVIIa and anti-fibrinolytics. Maternal alloimmunization against platelet antigens was reported in 73% of pregnancies and was associated with four neonatal deaths. These data emphasize the need for multidisciplinary management of pregnancy in women with GT. Delivery plans should recognize the need for prevention and aggressive treatment of PPH and should minimize foetal bleeding risk in pregnancies complicated by alloimmunization.
authors: Department of Haematology, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.
authors: S
authors: S
authors: Siddiq
authors: A
authors: A
authors: Clark
authors: A
authors: A
authors: Mumford
mesh_terms: D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D019468:Disease Management; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000517:prevention & control; D007744:Obstetric Labor Complications / Q000517:prevention & control; D017713:Platelet Transfusion; D006473:Postpartum Hemorrhage / Q000209:etiology / Q000517:prevention & control*; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000209:etiology / Q000517:prevention & control*; D011256:Pregnancy Outcome; D011994:Recombinant Proteins / Q000627:therapeutic use; D013915:Thrombasthenia / Q000150:complications*
chemical_list: D000933:Antifibrinolytic Agents; D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa