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Management of a Patient with Glanzmann's Thrombasthenia Scheduled for Cesarean Section
Abstract Number: SUN-04
Abstract Type: Case Report/Case Series
Glanzmann's Thrombasthenia (GT) is a rare, autosomal recessive platelet disorder. The defect is an absence of the GPIIb–IIIa complex on the surface of the platelet membrane. Platelet aggregation is severely impaired by the absence of this integral membrane complex.
Case Report: A 35 years female with a diagnosis with GT was scheduled for repeat C-Section at 38 weeks of gestation. History was significant for frequent gum and nose bleeds and multiple transfusions as a child. A multi-disciplinary team discussion between hematologist, obstetrician, blood bank and the anesthesia team was held throughout this pregnancy for her care. Prior to C-Section she was optimized with IV iron therapy and supplemented with oral iron, B12 and pre-natal vitamins.
Pre-operative hematocrit was 32 and platelets were 224. Amicar and tranexamic acid, as well as 1 unit of HLA matched platelets for transfusion were available prior to C-Section. To treat uncontrolled bleeding, recombinant Factor VIIa was obtained. A right antecubital 7 french RICC catheter and 18 gauge IV catheter in the left arm were placed in the holding area. HLA matched platelets were transfused en route to the operating room.
In the OR, GA was induced with 200 mg of propofol and 100 mg of succinylcholine and intubated with cricoid pressure. 5 grams loading dose of amicar was administered and maintained at 1 gram per hour. A propofol infusion at 75 mcg/kg/hr and 0.4% Sevoflurane with 50% nitrous oxide was started. Baby was delivered with apgar scores of 8 and 9. Excessive oozing was noted upon closure of fascia and one unit of single donor platelets was transfused. Final EBL was 1500 L, and 2500 ml of crystalloids was administered. Patient was extubated in the OR and transferred to PACU. Patient remained hemodynamically stable without signs of abnormal bleeding. Post-op CBC: HCT 28 PLT 117K. Patient was discharged on POD 3.
The incidence of morbidity and mortality associated with GT is largely unknown although it is estimated that 1:1,000,000 have GT. Clinical presentation of patients with the disorder includes hemorrhagic manifestations like purpurae, epistaxis, gingival hemorrhage, menorrhagia and mucocutaneous bleeding. Laboratory studies show prolonged bleeding time, with normal platelet count and normal coagulation studies. Light transmission aggregometry is the gold standard of diagnosis.
Patients are at increased risk for severe bleeding during pregnancy extending well into the postpartum period. Various peripartum treatments are described to limit associated obstetric hemorrhage; platelet and factor 7 transfusion, gamma globulins and plasmapheresis. Studies have suggested allogeneic matched bone marrow transplantation to be the definitive therapy in patients with intractable bleeding and has resulted in successful resolution of bleeding episodes. However, there remains no consensus regarding best management strategies.