Management of May–Hegglin anomaly referred for coronary artery bypass

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.

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Anesthetic management without perioperative platelet transfusion for cervical laminectomy and laminoplasty in a case of May–Hegglin anomaly

Kumemura

Omae

Kou

et al. 2018

J Anesth

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Perioperative Management of a Patient With Profound Thrombocytopenia Secondary to MYH9-RD Presenting for Thoracic Aortic Aneurysm Repair and Aortic Valve Replacement

Wakefield

Diehl

Neff

et al. 2021

Journal of Cardiothoracic and Vascular Anesthesia

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Thrombocytopenia: Perioperative Considerations for Patients Undergoing Cardiac Surgery

Klompas

Boswell

Plack

et al. 2022

Journal of Cardiothoracic and Vascular Anesthesia

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Management of May–Hegglin anomaly referred for coronary artery bypass

Abstract: We report the case of a patient with May-Hegglin anomaly who underwent a coronary bypass graft using an extra-corporeal circulation and discuss the perioperative and anesthetic management.

Cited by 3 publications

References 7 publications

Anesthetic management without perioperative platelet transfusion for cervical laminectomy and laminoplasty in a case of May–Hegglin anomaly

Anesthetic management without perioperative platelet transfusion for cervical laminectomy and laminoplasty in a case of May–Hegglin anomaly

Perioperative Management of a Patient With Profound Thrombocytopenia Secondary to MYH9-RD Presenting for Thoracic Aortic Aneurysm Repair and Aortic Valve Replacement

Thrombocytopenia: Perioperative Considerations for Patients Undergoing Cardiac Surgery

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