Successful Management of Endometriosis, in Vitro Fertilization, Pregnancy, and Postpartum Recovery in a Woman with Glanzmann's Thrombasthenia

Background: Glanzmann's thrombasthenia (GT) is an autosomal, qualitative platelet disorder that is genetically recessive. This report is about a patient with GT, complicated by infertility and endometriosis, who had a successful ovarian cystectomy, using recombinant-factor VIIa (rFVIIa; NovoSeven,^® Novo Nordisk). She then had a successful in-vitro fertilization (IVF) pregnancy. Case: A 35-year-old Omani woman, who had GT also had a 6 × 4-cm endometrioma. She underwent laparotomy and ovarian cystectomy with rFVIIa, platelet transfusion, and antifibrinolytics. The surgery and postoperative period were uneventful. Prior to oocyte retrieval for IVF, she received random donor platelets, tranexamic acid, and rFVIIa. At 36 + weeks, labor was induced due to intrauterine growth restriction. While in active labor, she received single-donor platelets, desmopressin (DDAVP^®) nasal spray and tranexamic acid. Results: The patient delivered a 2.215-kg baby girl with normal blood loss. She was readmitted with secondary postpartum hemorrhage, which was managed with methyl ergometrine and blood products, and she was discharged in stable condition after 48 hours. Conclusions: Endometriosis and increased levels of vitronectin appear to be correlated. Patients with deficiency in GPIIIa/IIb have aberrant αVβ3 receptor expression leading to increased levels of vitronectin, a substrate that enhances attachment and proliferation of ectopic endometrial cells. Surgery for such patients can be performed safely with rFVIIa. This might be the first case in the English literature of successful treatment with rFVIIa in endometriosis surgery, oocyte retrieval, and the postpartum period.