Perioperative Platelet Transfusion Strategies and Multidisciplinary Collaboration Experience in Glanzmann Thrombasthenia Caused by ITGA2B Gene Variation During Pregnancy: A Case Report.
Xue Cui, Nuowei Ji, Shengnan Wang, Huawei Jamal, Jiaxin Liu, Ying Wang, Haiyan Sun
Abstract
Open AccessObjective: To explore multidisciplinary peripartum management strategies and clinical implications for Glanzmann thrombasthenia (GT) caused by ITGA2B gene variation during pregnancy. Case Presentation: A 33-year-old woman at 38+ 5weeks of gestation, diagnosed with GT, was confirmed with biallelic pathogenic ITGA2B mutations (chr17:42452041 and chr17:42,457,372). There was no history of consanguineous marriage in the past three generations of her family. Coagulation dysfunction was observed during pregnancy (fibrinogen: 2.69 g/L; thromboelastography (TEG) maximum clot strength: 12 mm). A multidisciplinary team (MDT) recommended preoperative transfusion of 3 therapeutic doses of platelets and 10 units of cryoprecipitate, with dynamic TEG monitoring showing improved clot strength (post-transfusion maximum clot strength: 29.2 mm). A cesarean section delivered a healthy male infant (3455 g, Apgar score 10). Intraoperative bleeding (900 mL) and postoperative incisional oozing were controlled with compression bandages and platelet transfusion. Both mother and infant had favorable outcomes, with the incision achieving Grade II/A healing. Conclusion: Individualized coagulation regulation, multidisciplinary collaboration, dynamic TEG-guided platelet transfusion, and comprehensive postoperative hemostatic management are critical for GT with pregnancy. This case provides practical insights into peripartum care for GT patients.