Anesthetic implications of the grey platelet syndrome

Purpose: To describe the obstetrical anesthetic care provided to two sister s with a rare qualitative platelet disorder, the grey platelet syndrome (GP S). Clinical features: Both patients manifested thrombocytopenia prior to deliv ery without previous history of a bleeding diathesis or other abnormal labo ratory tests of coagulation function. The first required emergency Cesarean section due to fetal bradycardia. Due to the thrombocytopenia and the emer gency nature of the procedure, general anesthesia was used. During the C-se ction, 1.5-2 litres of old blood was noted in the abdominal cavity which wa s attributed to an old splenic capsular tear of unknown etiology. Work-up f or the thrombocytopenia revealed large platelets on the peripheral smear wi th abnormal aggregation on platelet function studies. Electron microscopy o f the platelets revealed absent alpha granules, diagnostic of GPS, The seco nd patient, the sister of patient #1, presented in a similar fashion. Howev er, at presentation, the platelet count was 112,000.m(-3) and spinal anesth esia was provided without complication for Cesarean delivery. The same pati ent presented for a second delivery during which fetal bradycardia necessit ated emergency C-section under general anesthesia. Despite administration o f six units of platelets, blood loss was 5,200 mL. Her postpartum course wa s uncomplicated and she and the infant were discharged home on postoperativ e day #4. Conclusion: The primary concerns for the anesthesiologist looking after pat ients with qualitative platelet defects are related to defective coagulatio n which influences the need for perioperative replacement of blood products and limits the use of regional anesthesia.