演題詳細

一般口演 / Oral Session

一般口演 88 (Oral Session 88) :ITP・トロンボポエチン (ITP and Thrombopoietin)

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日程
2013年10月13日(日)
時間
09:15 - 10:45
会場
第13会場 / Room No.13 (札幌市教育文化会館 3F 研修室301)
座長・司会
宮崎 浩二 (Koji Miyazaki):1
1:北里大学医学部 血液内科学
 
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Successful treatment of MYH9 disorders with romiplostim for neurosurgery

演題番号 : OS-3-82

山之内 純 (Jun Yamanouchi):1、羽藤 高明 (Takaaki Hato):2、新家 敏之 (Toshiyuki Niiya):3、國島 伸治 (Shinji Kunishima):4、安川 正貴 (Masaki Yasukawa):1

1:Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University、2:Division of Blood Transfusion and Cell Therapy, Ehime University Hospital、3:Division of Laboratory Medicine, Ehime University Hospital、4:Department of Advanced Diagnosis, Nagoya Medical Center

 

MYH9 disorders is characterized by macrothrombocytopenia and Alport manifestation, both of which derive from mutations of the MYH9 gene encoding nonmuscle myosin heavy chain IIA (NMHC IIA). Although most patients have mild bleeding, it might be desirable to increase the platelet count during major surgery. We report a patient with MYH9 disorders who was successfully treated with romiplostim for neurosurgery. A 42-year-old female presented with long-standing purpura and thrombocytopenia. There were many giant platelets in her peripheral blood and the platelet count, as determined by phase-contrast microscopy, was 25 x 109/L. This finding of macrothrombocytopenia led us to sequence analysis of the MYH9 gene, which revealed a novel missense mutation of c.5507A>G (p.Q1836R substitution). Immunofluorescence of neutrophil NMHC IIA showed fine aggregate formation. Audiograms showed high-frequency hearing loss. She had cerebral aneurysm and craniotomy was planned in order to prevent the rupture of aneurysm. After approval of the IRB, 1μg/kg romiplostim was sarted 6 weeks before the scheduled day of surgery and gradually increased to 5μg/kg. She had severe headache 2 days before the surgery, and CT scan showed mild subarachnoid hemorrhage when her platelet count was 84 x 109/L. The surgical procedure was performed without any bleeding complications and no neurological sequelae were left. Romiplostim was tapered and discontinued after 1 month. We demonstrate a novel mutation of the MYH9 gene and suggest the efficacy of romiplostim for treatment of MYH9 disorders during surgery.

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