Department of Anesthesiology and Pain Clinic, Juntendo University Shizuoka Hospital, 1129 Nagaoka, Izunokuni, Shizuoka, 410-2295, Japan.
Department of Advanced Diagnosis, Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan.
J Anesth. 2018 Aug;32(4):641-644. doi: 10.1007/s00540-018-2522-8. Epub 2018 Jun 22.
May-Hegglin anomaly (MHA) is an inherited autosomal dominant disorder characterized by giant platelets and inclusion bodies in granulocytes, and thrombocytopenia. There is no consensus on the perioperative management of this disorder. We report a case involving a patient with MHA who was perioperatively managed without platelet transfusion for cervical laminectomy and laminoplasty. In our case, the platelet count was measured to be 0.6 × 10/µL using an automatic blood cell counter. Peripheral blood smear and genetic test analyses were performed, leading to a definitive diagnosis of MHA. However, clot retraction, serotonin release, and platelet aggregation were normal. Total intravenous anesthesia with propofol and remifentanil, in combination with intermittent injection of fentanyl, was administered. The total blood loss volume was 300 mL, and perioperative course was uneventful. Visual platelet count and platelet function were preserved in this case, although platelet or red blood cell transfusion was not performed. No bleeding tendency was observed during perioperative management.
先天性巨大血小板综合征(May-Hegglin anomaly,MHA)是一种常染色体显性遗传性疾病,其特征为巨大血小板和中性粒细胞中的包涵体,同时伴有血小板减少症。对于这种疾病的围手术期管理尚未达成共识。我们报告了一例 MHA 患者,该患者在接受颈椎板切除术和椎板成形术时未进行血小板输注。在我们的病例中,使用自动血细胞计数器测量血小板计数为 0.6×10/µL。进行了外周血涂片和基因检测分析,明确诊断为 MHA。然而,凝血回缩、血清素释放和血小板聚集正常。采用丙泊酚和瑞芬太尼的全静脉麻醉,并间歇性注射芬太尼。总失血量为 300 毫升,围手术期无并发症。尽管未进行血小板或红细胞输注,但在这种情况下保留了血小板的可视计数和血小板功能。在围手术期管理期间未观察到出血倾向。