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.
Ascites ; Biopsy ; Bone Marrow ; Endoscopy, Digestive System ; Esophageal and Gastric Varices ; Female ; Fibrosis ; Hematopoiesis, Extramedullary ; Hemorrhage* ; Humans ; Hypertension, Portal ; Ligation ; Liver ; Magnetic Resonance Imaging ; Melena ; Middle Aged ; Primary Myelofibrosis* ; Reticulin ; Splenomegaly ; Stem Cells