How I manage pregnancy in women with Glanzmann thrombasthenia

Abstract

Glanzmann thrombasthenia (GT) is a rare inherited platelet function disorder caused by a quantitative and/or qualitative defect of the αIIbβ3 integrin. Pregnancy and delivery is a recognized risk period for bleeding in women with GT. The newborn may also be affected by fetal and neonatal immune thrombocytopenia induced by the transplacental passage of maternal anti-αIIbβ3 antibodies, which can lead to severe hemorrhage and fetal loss. Pregnancy in GT women thus requires a multidisciplinary approach, including prepregnancy counseling, and a treatment plan for delivery for both the mother and child. In this article, we summarize the current knowledge on pregnancy in women with GT and describe how we manage this severe platelet disorder in our clinical practice.

JTH in Clinic ‐ Obstetric bleeding: VWD and other inherited bleeding disorders

“…10,55,59,60 Rare platelet disorders require similar care in the approach. [61][62][63] Until there is more evidence, it is reasonable to treat childbirth as a major hemostatic challenge in all IBDs, and all are likely to benefit from tranexamic acid.…”

Treatment of Inherited Platelet Disorders: Current Status and Future Options

“…85,86 Platelet antibodies can cross the placenta, causing fetal or neonatal harm. [85][86][87] Neonatal alloimmune thrombocytopenia may occur in up to 30% of newborns of mothers with GT. 88 Thus, before and during pregnancy, antibodies should be monitored.…”

Vaginal Delivery in a Primipara with Glanzmann Thrombasthenia

“…For this reason, forceps are the better choice for instrumental delivery. 10 Epidural analgesia is contraindicated because of the potential risk of epidural or spinal hematoma during insertion and removal of the catheter. Therefore, current best practice is to decide the most appropriate mode of delivery based on the specific situation of the mother and the fetus.…”

“…This disease presents significant challenges to women during pregnancy, delivery, and postpartum. A systematic review of the literature (in Chinese and English) was carried out using PubMed and Web of Science without time restrictions; this identified 12 cases of women with GT who delivered a viable baby vaginally [1][2][3][4][5][6][7][8][9][10] ; the characteristics of these women are presented in SDC Table 1, http:// links.lww.com/MFM/A33. All of the reported cases delivered vaginally.…”

“…Compared with forceps, vacuum extraction increases the risk of cephalhematomas, diffuse subcutaneous hematomas of the scalp, and intracranial hemorrhage. For this reason, forceps are the better choice for instrumental delivery 10 . Epidural analgesia is contraindicated because of the potential risk of epidural or spinal hematoma during insertion and removal of the catheter.…”