Korean J Gastrointest Endosc > Volume 21(1); 2000 > Article
Korean Journal of Gastrointestinal Endoscopy 2000;21(1): 549-553.
식도 정맥류 출혈을 동반한 원발성 골수섬유증 1예 ( A Case of Idiopathic Myelofibrosis Causing an Esophageal Variceal Hemorrhage )
장희철(Hee Cheol Jang),이미화(Me Hwa Lee),유진우(Jin Woo Yoo),한현영(Hyun Young Han),강동욱(Dong Wook Kang),윤현수(Hyeon Soo Yoon),이수진(Su Jin Lee),박성규(Seong Kyu Park),최덕례(Duk Yeii Choi)
Idiopathic Myelofibrosis (IMF), a clonal disorder of a multipotent hemtopoietic progenitor cell of unknown etiology, is characterized by massive splenomegaly, leukoerythroblastic blood changes, and bone marrow fibrosis. Portal hypertension occurs in approximately 10% of patients with IMF. Alsom there mey be bleeding from esophageal varices. A 59-year-old woman was admitted with intermittent abdominal distension and melena. An esophagogastroduodenoscopy revealed extensive esophageal varices with red colored signs, and subsequently esophageal variceal ligation was performed. Marked splenomegaly and ascites was observed through abdominal US and MRI scan. Bone marrow biopsy revealed an increase in reticulin and fibrosis. Extramedullary hematopoiesis and sinusoidal change were observed in the liver biopsy specimen. This case of IMF causing an esophageal variceal hemorrhage is herein reported. (Korean J Gastrointest Endosc 2000;21:549-553)
Key Words: Myelofibrosis, Portal hypertension, Esophageal varices
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