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Neuraxial Anesthesia for a Parturient with Giant Platelet Syndrome
Abstract Number: F-51
Abstract Type: Case Report/Case Series
Introduction: Giant platelet syndrome is a rare spectrum of inherited diseases characterized by abnormally large platelets and varying degrees of platelet dysfunction. It is described in hematology, but not in obstetric anesthesiology literature. We describe anesthetic care for a parturient with giant platelet syndrome.
Case Description: A 29-year old G1P0 Southeast Asian woman at 36 weeks gestation with thrombocytopenia presented for a non-routine anesthesia consult to determine the suitability of epidural placement during labor. Symptoms included epistaxis and mild gingival bleeding with flossing. Her history was significant for root canals without excess bleeding.
A hematology referral was made, and workup found low MCV anemia consistent with hemoglobin E, giant sized platelets on peripheral smear and 24% immature platelets. A manual platelet count was 150,000. Coagulation, platelet aggregation and secretion studies were normal. The patient was diagnosed with congenital giant platelet syndrome. Since she had normal platelet function and no evidence of bleeding diathesis, it was estimated that her risk for neuraxial anesthesia was similar to that of the general population. The patient presented at 40 weeks gestation with spontaneous rupture of membranes and a platelet count of 109,000. An L4-L5 epidural was placed with a 17 g Tuohy needle and dosed with bupivacaine 0.125% and fentanyl 2 mcg/mL. An uncomplicated cesarean section was performed for non-reassuring fetal heart tracing and chorioamnionitis. The epidural was dosed with a total of 15 mL lidocaine 2% with epinephrine for a bilateral T4 block. It was used for postoperative analgesia and removed 24 hrs after surgery without complication.
Discussion: The differential diagnosis of thrombocytopenia includes acquired and inherited platelet disorders, which can be differentiated on peripheral blood smear.1 Inherited giant platelet diseases are rare disorders characterized by thrombocytopenia, large platelets and variable bleeding symptoms ranging from asymptomatic to severe and in some cases, neonatal alloimmune thrombocytopenia.1 Platelet counts range from normal to severe thrombocytopenia and normal to severe platlet dysfunction. A systematic review of 18 patients with Bernard-Soulier Syndrome, one type of giant platelet disorder, showed high incidence of primary and secondary post-partum hemorrhage, blood transfusion, and in two patients, emergent hysterectomy.2 Since our patient presented with stable thrombocytopenia and no signs of bleeding, the anesthesia team proceeded with administering successful epidural anesthesia without complication.
1. Mhawech P, Saleem A. Inherited giant platelet disorders. Classification and literature review. Am J Clin Pathol 2000; 113:176.
2. Peitsidis P, Datta T, Pafilis I, Otomewo O, Tuddenham EG, Kadir RA. Bernard Soulier syndrome in pregnancy: a systematic review. Haemophilia. 2010 Jul 1;16(4):584-91.