Hisada T, Kuwabara H, Tsunoda T, Kaneko K, Kubota S, Miwa Y, Mori M
Department of Internal Medicine, Numata National Hospital.
Intern Med. 1995 Jun;34(6):589-92. doi: 10.2169/internalmedicine.34.589.
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is a bleeding disorder attributed to a vascular developmental abnormality. It is transmitted as an autosomal dominant trait. A 63-year-old female was admitted because of repeated episodes of severe anemia which resulted from bleeding of telangiectases in the gastric mucosa. Conventional therapies including endoscopical microwave coagulation and ethanol injection were not effective. The persistent anemia necessitated frequent blood transfusion. Estrogen was orally administrated and blood transfusion became unnecessary. Thus, estrogen therapy should be considered as one of the effective treatments for recurrent severe anemia due to hereditary hemorrhagic telangiectasia.
遗传性出血性毛细血管扩张症(奥斯勒-韦伯-伦杜病)是一种由血管发育异常引起的出血性疾病。它以常染色体显性性状遗传。一名63岁女性因胃黏膜毛细血管扩张反复出血导致严重贫血反复发作而入院。包括内镜下微波凝固和乙醇注射在内的传统治疗方法均无效。持续性贫血需要频繁输血。口服雌激素后不再需要输血。因此,雌激素治疗应被视为治疗遗传性出血性毛细血管扩张症所致复发性严重贫血的有效治疗方法之一。