下顎智歯の抜去手術を施行したMay-Hegglin異常症患者の1例
May-Hegglin anomaly is a rare autosomal dominant inherited disorder characterized by giant platelets, thrombocytopenia, and inclusion bodies in granulocytes. Clinically, because of thrombocytopenia, a mild hemorrhagic diathesis may occur. We extracted the mandibular third molar in a patient with May...
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| Published in | 日本口腔外科学会雑誌 Vol. 65; no. 8; pp. 529 - 533 |
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| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
社団法人 日本口腔外科学会
20.08.2019
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0021-5163 2186-1579 |
| DOI | 10.5794/jjoms.65.529 |
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| Abstract | May-Hegglin anomaly is a rare autosomal dominant inherited disorder characterized by giant platelets, thrombocytopenia, and inclusion bodies in granulocytes. Clinically, because of thrombocytopenia, a mild hemorrhagic diathesis may occur. We extracted the mandibular third molar in a patient with May-Hegglin anomaly whose platelet count was visually reduced to 1.6 × 104/μL. The patient was a 27-year-old woman, and the right mandibular third molar to be removed had completely erupted. In this case, good results were obtained with no postoperative bleeding only with local hemostasis (use of an absorptive hemostatic agent, gingival suture). In conclusion, in patients with May-Hegglin anomaly, even if the platelet count on visual observation is as low as 1.6 × 104/μL, tooth extraction is considered possible when an appropriate local hemostatic method is applied. |
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| AbstractList | May-Hegglin anomaly is a rare autosomal dominant inherited disorder characterized by giant platelets, thrombocytopenia, and inclusion bodies in granulocytes. Clinically, because of thrombocytopenia, a mild hemorrhagic diathesis may occur. We extracted the mandibular third molar in a patient with May-Hegglin anomaly whose platelet count was visually reduced to 1.6 × 104/μL. The patient was a 27-year-old woman, and the right mandibular third molar to be removed had completely erupted. In this case, good results were obtained with no postoperative bleeding only with local hemostasis (use of an absorptive hemostatic agent, gingival suture). In conclusion, in patients with May-Hegglin anomaly, even if the platelet count on visual observation is as low as 1.6 × 104/μL, tooth extraction is considered possible when an appropriate local hemostatic method is applied. |
| Author | 石川, 恵生 笹原, 庸由 逸見, 朋陽 飯野, 光喜 北畠, 健一朗 小林, 武仁 |
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| References | 15) 南 克浩， 森 悦秀， 他：May-Hegglin anomaly患者の抜歯経験．日口外誌 53: 568-571, 2007. 20) Everlien, M, Knoch, K, et al : Coronary bypass surgery in May-Hegglin anomaly requiring craniotomy. Dtsch Med Wochenschr 126: 47-49, 2001. 18) 石田 明， 半田 誠：血小板輸血不応状態．検と技 25: 266-269, 1997. 1) 國島伸治：先天性血小板減少症の診断と分子病態．臨血 55: 882-892, 2014. 10) 松本勇人， 小川一英， 他：特発性血小板減少性紫斑病と間違われたMay-Hegglin異常の一例．東北止血血栓研会誌 22: 9-10, 2008. 14) 鎌田 仁, 更家 誠, 他：全顎抜歯を行ったMay-Hegglin anomalyの1例． 日口外誌 36: 148-153, 1990. 12) Pecci A, Panza E, et al : Position of nonmuscle myosin heavy chainⅡA (NMMHC-ⅡA) mutations predicts the natural history of MYH9-related disease. Hum Mutat 29: 409-417, 2008. 6) Balduini CL, Pecci A, et al : Recent advances in the understanding and management of MYH9-related inherited thrombocyteopenias. Br J Haematol 154: 161-174, 2011. 16) Godwin HA, Ginsberg AD, et al : May-Hegglin anomaly : A defect in megakaryocyte fragmentation? Br J Haematol 26: 117-127, 1974. 19) Nelson LH, Dewan DM, et al : Obsteric and anesthetic considerations in the May-Hegglin anomaly. A case report. J Reprod Med 38: 311-314, 1933. 22) Kumemura M, Omae T, et al : Anesthetic management without perioperative platelet transfusion for cervical laminectomy and laminoplasty in a case of May-Hegglin anomaly. J Anesth 32: 641-644, 2018. 4) Kunishima S and Saito H : Advances in the understanding of MYH9 disorders. Curr Opin Hematol 17: 405-410, 2010. 7) 金子 誠：May-Hegglin異常．検と技 43: 1076-1077, 2015. 8) 新美直哉， 谷口由美， 他：埋伏智歯抜歯を行ったMay-Hegglin anomaly患者の1例． 日口外誌 45: 727-729, 1999. 21) 天日 聖， 須田佳美， 他：メイ・ヘグリン異常患者の麻酔経験．麻酔 65: 370-372, 2016. 11) Kunishima S, Matsushita T, et al : Immunofluorescence analysis of neutrophil nonmuscle myosin heavy chain-A in MYH9 disorders : association of subcellular localization with MYH9 mutations. Lab Invest 83: 115-122, 2003. 17) 竹中 徹， 中峯寛和， 他：May-Hegglin異常症の1家系， 2症例における血小板の形態学的， 生化学的および機能的検索．臨血 28: 1442-1448, 1987. 3) Hegglin R : Gleichzeitige konstitutionelle Veranderungen an Neutrophilen und Thrombocyten. Helv Med Acta 12: 439-440, 1945. 2) May R : Leukocyteneinschlusse. Dtsch Arch Klin Med 96 : 1-6, 1909. 9) 國島伸治：MYH9異常症と， その他の先天性血小板減少症．臨検 61: 152-156, 2017. 5) 國島伸治：先天性巨大血小板症の診断．日内会誌 98: 43-48, 2009. 13) 西垣篤正, 中島信也, 他：親子に見られたMay-Hegglin anomaly患者の抜歯経験． 日口外誌 32: 1084-1090, 1986. |
| References_xml | – reference: 18) 石田 明， 半田 誠：血小板輸血不応状態．検と技 25: 266-269, 1997. – reference: 21) 天日 聖， 須田佳美， 他：メイ・ヘグリン異常患者の麻酔経験．麻酔 65: 370-372, 2016. – reference: 17) 竹中 徹， 中峯寛和， 他：May-Hegglin異常症の1家系， 2症例における血小板の形態学的， 生化学的および機能的検索．臨血 28: 1442-1448, 1987. – reference: 11) Kunishima S, Matsushita T, et al : Immunofluorescence analysis of neutrophil nonmuscle myosin heavy chain-A in MYH9 disorders : association of subcellular localization with MYH9 mutations. Lab Invest 83: 115-122, 2003. – reference: 2) May R : Leukocyteneinschlusse. Dtsch Arch Klin Med 96 : 1-6, 1909. – reference: 12) Pecci A, Panza E, et al : Position of nonmuscle myosin heavy chainⅡA (NMMHC-ⅡA) mutations predicts the natural history of MYH9-related disease. Hum Mutat 29: 409-417, 2008. – reference: 14) 鎌田 仁, 更家 誠, 他：全顎抜歯を行ったMay-Hegglin anomalyの1例． 日口外誌 36: 148-153, 1990. – reference: 16) Godwin HA, Ginsberg AD, et al : May-Hegglin anomaly : A defect in megakaryocyte fragmentation? Br J Haematol 26: 117-127, 1974. – reference: 1) 國島伸治：先天性血小板減少症の診断と分子病態．臨血 55: 882-892, 2014. – reference: 19) Nelson LH, Dewan DM, et al : Obsteric and anesthetic considerations in the May-Hegglin anomaly. A case report. J Reprod Med 38: 311-314, 1933. – reference: 9) 國島伸治：MYH9異常症と， その他の先天性血小板減少症．臨検 61: 152-156, 2017. – reference: 20) Everlien, M, Knoch, K, et al : Coronary bypass surgery in May-Hegglin anomaly requiring craniotomy. Dtsch Med Wochenschr 126: 47-49, 2001. – reference: 5) 國島伸治：先天性巨大血小板症の診断．日内会誌 98: 43-48, 2009. – reference: 3) Hegglin R : Gleichzeitige konstitutionelle Veranderungen an Neutrophilen und Thrombocyten. Helv Med Acta 12: 439-440, 1945. – reference: 8) 新美直哉， 谷口由美， 他：埋伏智歯抜歯を行ったMay-Hegglin anomaly患者の1例． 日口外誌 45: 727-729, 1999. – reference: 22) Kumemura M, Omae T, et al : Anesthetic management without perioperative platelet transfusion for cervical laminectomy and laminoplasty in a case of May-Hegglin anomaly. J Anesth 32: 641-644, 2018. – reference: 6) Balduini CL, Pecci A, et al : Recent advances in the understanding and management of MYH9-related inherited thrombocyteopenias. Br J Haematol 154: 161-174, 2011. – reference: 4) Kunishima S and Saito H : Advances in the understanding of MYH9 disorders. Curr Opin Hematol 17: 405-410, 2010. – reference: 7) 金子 誠：May-Hegglin異常．検と技 43: 1076-1077, 2015. – reference: 15) 南 克浩， 森 悦秀， 他：May-Hegglin anomaly患者の抜歯経験．日口外誌 53: 568-571, 2007. – reference: 13) 西垣篤正, 中島信也, 他：親子に見られたMay-Hegglin anomaly患者の抜歯経験． 日口外誌 32: 1084-1090, 1986. – reference: 10) 松本勇人， 小川一英， 他：特発性血小板減少性紫斑病と間違われたMay-Hegglin異常の一例．東北止血血栓研会誌 22: 9-10, 2008. |
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| Title | 下顎智歯の抜去手術を施行したMay-Hegglin異常症患者の1例 |
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