Intestinal obstruction caused by extramedullary hematopoiesis and ascites in primary myelofibrosis

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.

Cytodiagnosis of extramedullary hematopoiesis in serous effusion: A rare presentation unfolding the underlying etiology

“…EMH is usually noted in reticuloendothelial organs such as spleen, liver, and lymph nodes and may also involve other sites such as kidneys, adrenals, gastrointestinal tract, lung, breast, skin, joints, posterior mediastinum, pleura, pericardium, and brain. [ 1 2 3 ] Very rarely, EMH can present as gastrointestinal masses which may adhere to adjacent structure resulting in obstructive and neurological symptoms. [ 3 ]…”

“…[ 1 2 3 ] Very rarely, EMH can present as gastrointestinal masses which may adhere to adjacent structure resulting in obstructive and neurological symptoms. [ 3 ]…”

“…EMH in serous fluids has been reported rarely with few case reports in literature. [ 3 4 5 6 7 ] EMH in pleural fluid is more rare than in peritoneal fluid. In our case, both peritoneal and pleural fluid involvement was seen with EMH.…”

Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis

“…In literature, 2 forms of implants are described: a microscopic one, evident only at pathological examination, and a macro-infiltrative one. The latter gives rise to mostly singular implants that growing on visceral peritoneum can cause bowel stenosis or occlusion [4] .…”

“…Extramedullary hematopoiesis mimicking acute appendicitis and intestinal obstruction, rectal stenosis, gastric outlet obstruction and bladder outlet obstruction due to extramedullary hematopoiesis have been reported [4] , [5] , [6] .…”