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.

The Glanzmann’s Thrombasthenia in Tunisia: A Cohort Study

“…Concerning bleeding in women, we can conclude that menorrhagia has a low frequency (35%), since it occurs only in five women in our cohort, compared to the described frequency in GT [18]. One woman achieved her pregnancy with natural delivery under platelets, while it is rare in a thrombasthenic patient since it often results in important hemorrhage requiring sometimes blood transfusions and threatening the vital prognosis of the mother [19]. For the diagnosis of GT, we use in our laboratory more than one specific test such us LTA which is extremely specific for GT, since platelet fails to aggregate with any agonist [20].…”

Management of Inherited Bleeding Disorders in Pregnancy

“…217 Of 40 pregnancies reviewed, three fetuses (7%) died from ICH between 24 and 31 weeks of gestation. 214 The risk is highest in women who have received multiple platelet transfusions, but may also occur in nontransfused women with absent expression of GP IIb/IIIa who develop antibodies when presented with paternally-derived fetal GP IIb/IIIa epitopes.…”

“…A systematic review 214 showed only partial success with prophylactic platelet transfusion prior to delivery, with PPH in 38% of treated women compared with 63% in women who had not received platelets. Platelets should be HLA-selected, starting with one to two adult doses and further doses depending on observed bleeding.…”

“…The UKHCDO guideline from 2006 suggested that recombinant factor VIIa in combination with tranexamic acid may help to reduce the need for platelet transfusion, 204 however, in three case reports, it was not found to be successful. 214 Evidence level 3…”

“…While antenatal bleeding tended to be mild, postpartum bleeding was frequently severe and occurred up to 20 days after delivery, with a median time of 10 days. 214 Evidence level 2À…”