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International Journal of Advances In Case Reports

Volume 11, Issue 1, 2024
Mcmed International
International Journal of Advances In Case Reports
Issn
XXX-XXXX (Print), 2349 - 8005 (Online)
Frequency
bi-annual
Email
editorijacr@mcmed.us
Journal Home page
http://mcmed.us/about/ijacr
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Abstract
Title
PERIOPERATIVE MANAGEMENT OF PATIENT WITH MAY-HEGGLIN ANOMALY
Author
Jin Sun Yoon, Hee Jung Jeon*, Sam Soon Cho, Jae Do Lee, Rak Min Choi, Jun-ho Jo, Young Soo Kim, Ju Young Park, Junhyug Jeong
Email
hjjunn@hanmail.net
keyword
May-Hegglin anomaly, Thrombocytopenia, Desmopressin.
Abstract
The May-Hegglin anomaly (MHA) is a rare autosomal dominant hematological disorder characterized by varying degrees of thrombocytopenia, Dohle like inclusion bodies in the granulocytes and mutations of the MYH9 gene. The association between MHA and the risk of excessive bleeding during surgical procedures is unclear. We report here an experience during the administration of anesthesia to a patient with MHA who had thrombocytopenia and required Miles operation. Twenty units of platelet were transfused to correct thrombocytopenia and reduce oozing at the operation site during the intraoperative period, and intravenous desmopressin was administered in an attempt to control postoperative bleeding. After the administration of desmopressin, no further transfusions were required.
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