Paraspinal and Presacral Extramedullary Hematopoiesis: A Rare Manifestation of Polycythemia Vera

Abstract

Extramedullary hematopoiesis is characterized by the presence of hematopoietic tissue outside the bone marrow. Extrathoracic extramedullary hematopoiesis is a rare and usually asymptomatic condition. We report a case of a 38-year-old female with paraspinal and presacral extramedullary hematopoiesis with polycythemia vera. Clinical and laboratory evaluation, along with radiological and histopathological findings are described. The diagnosis of the disease was confirmed by CT-guided biopsy. Review of literature is presented.

A systematic review of presacral extramedullary haematopoiesis: a diagnosis to be considered for presacral masses

“…). Of the remaining 31 articles, there were 37 cases reports of patients with a confirmed presacral EMH . To this group, the two cases presented here were added.…”

Splenic myeloid metaplasia in warm autoimmune hemolytic anemia (wAIHA): a retrospective study

“…Myeloid metaplasia (MM), also known as extramedullary hematopoiesis (EMH), refers to the physiopathological process in which hematopoietic progenitor cells differentiate into blood cells outside of the bone marrow [ 1 ]. EMH, resulting from a compensatory mechanism for insufficient hematopoiesis, usually occurs in the spleen and the liver [ 2 ]. Some authors have proposed that the main etiology of EMH are associated with hypoxia and acidic microenvironment [ 3 4 5 ].…”

Sclerosing Extramedullary Hematopoietic Tumor Mimicking Intra-abdominal Sarcoma

“…EMH is seen in many disorders such as sickle cell anemia, hemoglobinopathies, thalassemia, hereditary spherocytosis, and MF [ 5 ]. EMH is most commonly observed in the liver and spleen and is rarely found in the peritoneum, lymph nodes, kidneys, thymus, central nervous system, retroperitoneum, myocardium, uterus, pleura, paraspinal region, or intestines [ 6 - 9 ].…”