Intestinal obstruction caused by extramedullary hematopoiesis and ascites in primary myelofibrosis

World J Gastroenterol. 2014 Sep 7;20(33):11921-6. doi: 10.3748/wjg.v20.i33.11921.

Abstract

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.

Keywords: Ascites; Extramedullary hematopoiesis; Intestinal obstruction; Primary myelofibrosis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Angiogenesis Inhibitors / therapeutic use
  • Ascites / diagnosis
  • Ascites / drug therapy
  • Ascites / etiology*
  • Diagnosis, Differential
  • Diuretics / therapeutic use
  • Hematopoiesis, Extramedullary*
  • Humans
  • Hypertension, Portal / diagnosis
  • Hypertension, Portal / etiology
  • Ileal Diseases / diagnosis
  • Ileal Diseases / etiology*
  • Ileal Diseases / surgery
  • Intestinal Obstruction / diagnosis
  • Intestinal Obstruction / etiology*
  • Intestinal Obstruction / surgery
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Primary Myelofibrosis / complications*
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / drug therapy
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Angiogenesis Inhibitors
  • Diuretics