Is cesarean section better or vaginal delivery in pregnant woman with Glanzmann's thrombasthenia?

مقدمه : Glanzmann thrombocytopenia (GT) is a genetic disease of the AR (autosomal recessive disorder) associated with platelet dysfunction. Platelets are special non-nuclear cells that participate in complex reactions to prevent bleeding. Point mutations, deletions, and genetic additions lead to defects in complex proteins encoded by the ITGA۲B and ITGB۳ genes.Platelets naturally bind to von Willebrand and collagen beneath the endothelium of the vessel wall via surface glycoprotein ۱b-IX-V and pass through the site of injury to the vessel. Activates platelets and also exposes the fibrinogen receptor GP IIb / IIIa. Platelets contain defective levels or small amounts of the GP IIB / IIIa glycoprotein in this rare inherited bleeding disorder. As a result, there is no fibrinogen bridge between one platelet and other platelets, and the bleeding time will be significantly longer.Although due to various complications such as preeclampsia, uterine atony, etc., Bleeding volume during delivery is different, bleeding during delivery is irreversible.For this reason, controlling bleeding in people with underlying coagulation disorders raises the concerns of obstetricians.هدف : The aim of this study is to express the favorable outcome of planned cesarean delivery in pregnant woman with Glanzmann's thrombasthenia.معرفی بیمار : The patient of a ۲۸-year-old G۱ woman with a gestational age of ۳۸ weeks +۵ days known case of GT due to Gross hematuria was referred to our hospital the day before. She suffered from vaginal spotting and recurrent epistaxis several times during her pregnancy but was not hospitalized. A gynecologist and hematologist monitored the patient during pregnancy. The initial manifestations of the disease occurred at the age of one year after severe bleeding after piercing the ear to install earrings. Due to frequent epistaxis, bleeding gums, and the development of petechiae and ecchymosis following minor trauma, a thorough examination was performed, and she was diagnosed with mild GT. The patient was the only child in the family and the result of a consanguineous marriage. She did not report a family history of GT in first- and second-degree relatives and said only one case of a similar disease in distant relatives.Her period at ۱۳ was accompanied by heavy and prolonged bleeding that led to a blood transfusion. She was also treated with iron, combined contraceptives, and tranexamic acid to prevent severe bleeding and treat iron deficiency throughout her life.