title: [Anesthetic Management of a Patient with May-Hegglin Anomaly].
abstract: 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.
authors.forename: Takashi
authors.initials: T
authors.lastname: Tennichi
authors.forename: Yoshimi
authors.initials: Y
authors.lastname: Suda
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Taki
authors.forename: Kazuki
authors.initials: K
authors.lastname: Toyama
mesh_terms: D000758:Anesthesia / Q000379:methods*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000097:blood*; D006801:Humans; D007044:Hysterectomy; D008875:Middle Aged; D010976:Platelet Count; D013921:Thrombocytopenia / Q000097:blood / Q000151:congenital*