Publication:

Optimized outcome of pregnancy in a woman with Glanzmann thrombasthenia with multidisciplinary approach

Glanzmann thrombasthenia (GT), an autosomal recessive disorder of defective platelet aggregation due to abnormalities of platelet receptor GPIIb/IIIa, can result in complication of bleeding during pregnancy. We report multidisciplinary management of a case with optimized outcome. 23 years old short statured (142 cm) primigravida was referred for contracted pelvis at term in labour. She was diagnosed with GT at 12 years of age during evaluation of gum bleeding, easy bruising, and prolonged post traumatic bleeding. She was born of a second-degree consanguineous marriage. At admission she was hemodynamically stable but moderately pale. Her hemoglobin was 8.7 g%, with 4.6 lacs platelets and clotting time of 15 minutes. Multidisciplinary team of hematologist, transfusionist and anesthetist was activated. Arranging single donor platelets in a short notice was challenging. Emergency cesarean section under general anesthesia was performed after transfusing two units packed red blood cell and 2 single donor platelets to deliver an alive male baby of 3.3 kg. Hemostasis was secured with cautery and ligatures. Tranexamic acid infusion and sublingual misoprostol was given prophylactically. she was transfused another 4 random donor platelets during operation and postoperatively. The case details will be presented. GT have normal to high platelet counts though they are normal morphologically, they are dysfunctional. This fact needs to be recognized and referred early to equipped centres. We used component transfusion, active management of third stage and tranexamic acid for optimizing outcome due to haemorrhage. At present, there is lack of consensus regarding optimum treatment of post-partum hemorrhage in patients with GT.