Intestinal obstruction caused by extramedullary hematopoiesis and ascites in primary myelofibrosis

• Published in: World journal of gastroenterology : WJG Vol. 20; no. 33; pp. 11921 - 11926
• Main Author: Wei, Xiu-Qing
• Format: Journal Article
• Language: English
• Published: United States Baishideng Publishing Group Inc 07.09.2014
• Subjects: Angiogenesis Inhibitors - therapeutic use; Asci; Ascites - diagnosis; Ascites - drug therapy; Ascites - etiology; Case Report; Diagnosis, Differential; Diuretics - therapeutic use; Hematopoiesis, Extramedullary; Humans; Hypertension, Portal - diagnosis; Hypertension, Portal - etiology; Ileal Diseases - diagnosis; Ileal Diseases - etiology; Ileal Diseases - surgery; Intestinal; Intestinal Obstruction - diagnosis; Intestinal Obstruction - etiology; Intestinal Obstruction - surgery; Male; Middle Aged; myelofibrosis; obstruc-tion; Predictive Value of Tests; Primary; Primary Myelofibrosis - complications; Primary Myelofibrosis - diagnosis; Primary Myelofibrosis - drug therapy; Tomography, X-Ray Computed; Treatment Outcome; Intestinal obstruction; Primary myelofibrosis; Ascites; Extramedullary hematopoiesis
• ISSN: 1007-9327; 2219-2840; 2219-2840
• DOI: 10.3748/wjg.v20.i33.11921

Primary myelofibrosis(PMF) is a clonal hematopoietic stem cell disorder. It is characterized by bone marrow fibrosis, extramedullary hematopoiesis with hepatosplenomegaly and leukoerythroblastosis in the peripheral blood. The main clinical manifestations of PMF are anemia, bleeding, hepatosplenomegaly, fatigue, and fever. Here we report a rare case of PMF with anemia, small bowel obstruction and ascites due to extramedullary hematopoiesis and portal hypertension. The diagnosis was difficult to establish before surgery and the differential diagnosis is discussed.