難治性扁桃出血を契機に診断された家族歴を有するvon Willebrand病例

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  • A Case of von Willebrand’s Disease Diagnosed by Refractory Tonsillar Hemorrhage with a Family History
  • ナンチセイ ヘントウ シュッケツ オ ケイキ ニ シンダン サレタ カゾクレキ オ ユウスル von Willebrandビョウレイ

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<p>The disease termed von Willebrand’s disease (vWD) is an autosomal-dominant hereditary bleeding disorder caused by dysfunction or deficiency of the clotting factor called von Willebrand factor (vWF); vWF is produced by the vascular endothelial cells and bone marrow megakaryocytes, and plays a major role in platelet adhesion and aggregation in the processes of primary hemostasis.</p><p>We reported the case of 3-year-old girl with vWD, who presented us with refractory tonsillar hemorrhage. Because of the family history of refractory bleeding, the concentration and activity of vWF in the blood were measured. Accordingly, the child was diagnosed as having vWD as she met the following diagnostic criteria: 1) abnormal bleeding episode; 2) vWF antigen titer or vWF activity less than 30% or equivalent to the mean -2SD. After additional laboratory examination, we diagnosed her as having type2A vWD. Moreover, her mother, grandmother and 2 second cousins were also diagnosed having type2A vWD. Following the diagnosis, the refractory pharyngeal bleeding was controlled by intravenous factor VIII/vWF supplementation.</p><p>Because refractory epistaxis and oral mucosal bleeding are common symptoms in patients with vWD, it is important for otolaryngologists to promptly diagnose this disease by type and advise patients on the appropriate course of treatment.</p>

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