Tamaki Kumiko, Otaka Michiro, Sakamoto Naoto, Matsumoto Kenshi, Yamashina Shunhei, Watanabe Sumio
Department of Gastroenterology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-8421, Japan.
J Med Case Rep. 2010 Jan 28;4:25. doi: 10.1186/1752-1947-4-25.
Idiopathic myelofibrosis is a chronic myeloproliferative disorder characterized by leukoerythroblastosis, massive splenomegaly, and increases in the reticular and collagen fibers in the bone marrow. Portal hypertension is observed in some patients with idiopathic myelofibrosis. Gastrointestinal hemorrhages, which are due mostly to the rupture of the esophageal varices, have been sporadically reported to be an infrequent complication of idiopathic myelofibrosis.
We report a case of a Japanese 63-year-old woman with myelofibrosis and variceal hemorrhage, with a background of concomitant portal and pulmonary hypertension. She was successfully treated through a combination of endoscopic variceal ligation and chemotherapy.
This is the first known report on the successful application of endoscopic variceal ligation and chemotherapy as the therapeutic procedure for an esophageal variceal hemorrhage in a patient with myelofibrosis.
原发性骨髓纤维化是一种慢性骨髓增殖性疾病,其特征为幼稚粒-红细胞血症、巨脾以及骨髓中网状纤维和胶原纤维增多。部分原发性骨髓纤维化患者会出现门静脉高压。胃肠道出血主要由食管静脉曲张破裂所致,偶有报道称其是原发性骨髓纤维化的罕见并发症。
我们报告一例63岁日本女性患者,患有骨髓纤维化并伴有静脉曲张出血,同时存在门静脉高压和肺动脉高压。通过内镜下静脉曲张结扎术和化疗联合治疗,患者获得成功救治。
这是已知的首例关于成功应用内镜下静脉曲张结扎术和化疗治疗骨髓纤维化患者食管静脉曲张出血的报告。