[Anesthetic Management of a Patient with May-Hegglin Anomaly]

We report the case of a 52-year-old woman with May-Hegglin Anomaly (MHA) and a platelet count of 1.9 x 10(4) x mm(-3). She was scheduled to undergo abdominal total hysterectomy and adnexectomy with uterine fibroids. Anesthesia was maintained by inhalation of sevoflurane (1.5%), continuous intravenous infusion of remifentanil (0.2-0.3 μg x kg(-1) x min(-1)), intermittent intravenous infusion of fentanyl (35 μg), and the transversus abdominis plane block with 20 ml of 0.375% ropivacaine. The amount of bleeding during surgery was 150 ml and her postoperative course was uneventful. MHA is a rare hematological disorder inherited as an autosomal dominant trait that is characterized by thrombocytopenia, giant platelets, and inclusion bodies in the granulocytes. A decrease in platelet count is detectable with a blood test But function of platelets is generally normal. Anesthesiologist should understand the characteristics of MHA, and administer anesthetics without giving platelets.