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Uterine Rupture in a Parturient with Gray Platelet Syndrome
Abstract Number: F 47
Abstract Type: Case Report/Case Series
Introduction: Anesthetic management of women with thrombocytopenia in labor and delivery has been a source of controversy. There are no formal recommendations on an acceptable lower limit of platelet counts required to safely administer neuraxial anesthesia. We present the case of a parturient with Gray Platelet Syndrome (GPS) resulting from a novel mutation requiring an urgent cesarean delivery.
Case: A 19 year-old Gravida2 Para1 Hispanic female presented to our institution at 39 weeks gestation in active labor. Her pertinent history included a diagnosis of thrombocytopenia at thirteen years of age with reported menorrhagia, petechiae, gingival bleeding, and easy bruising. Peripheral blood smear demonstrated giant platelets with the absence of alpha-granules. A genetic mutation analysis was sent but pending at the time of delivery. A complete blood count demonstrated a platelet count of 23,000/µL. Obstetrical anesthesiology was consulted secondary to concern for the patient’s increase risk of uterine rupture. She was transfused a unit of single-donor platelets with an increase in her platelet count to 46,000/µL. After this transfusion, she was taken for an urgent cesarean section. Two large bore peripheral intravenous catheters were secured, a rapid fluid infuser was prepared, and blood products were ordered and made immediately available in the operating room. A rapid sequence induction was performed and the infant was delivered uneventfully with adequate hemostasis, despite a partial uterine dehiscence. The patient received 60 units of oxytocin and was hemodynamically stable. She was extubated in the operating room and transported to the recovery room. She developed post-partum hemorrhage requiring three units of packed red blood cells and was discharged home on postoperative day 3 with a platelet count of 54,000/µL. Genetic analysis later revealed a homozygous mutation of the NBEAL2 gene.
Discussion: GPS is a rare congenital thrombocytopenia characterized by the absence of alpha-granules in platelets1. The absence of alpha-granules results in decreased levels of fibrinogen, von Willebrand factor, and factor V; and abnormal secondary platelet aggregation2 resulting in a qualitative and often quantitative defect. Successful management of these patients must include communication among perinatology, anesthesiology, obstetrics, and hematology. Preoperative evaluation should include a thorough history and physical exam and the availability of blood and component-specific products. Perioperative management necessitates large-bore intravenous access and the availability of rapid resuscitation equipment. Given that GPS is both a quantitative and qualitative platelet disorder, we recommend avoiding neuraxial anesthesia.
1.Bain BJ. Bhavnani M. Gray Platelet Syndrome. Amer J Hematology. 86(12):1027, 2011 Dec.
2.Clements et al. Expanding perfusion across disciplines: the use of thromboelastography in GPS. Perfusion. 26(3):181-4, 2011 May.