Abstract
Extramedullary hematopoiesis (EMH) is a rare disease that is characterized by the presence of hemopoietic tissue outside the bone marrow. The masses that form are usually microscopic and asymptomatic, but occasionally lead to tumor-like masses. A 56-year-old male who initially presented to the First Affiliated Hospital, School of Medicine, Zhejiang University (Hangzhou, China) with upper abdominal pain and jaundice was found to have paravertebral masses in the thorax. Histopathological examination of a computed tomography-guided needle aspiration biopsy of the masses revealed EMH. The current study presents this unusual case, in which EMH was diagnosed by chance in a patient with hereditary spherocytosis. As the intrathoracic EMH was asymptomatic, the patient was discharged with the proviso of regular follow-up examinations. The patient exhibited improved blood cell counts following a splenectomy to reduce the hemolysis and stabilize the thoracic masses. The thoracic masses have been closely followed for one and a half years. A correct diagnosis can thus aid in avoiding unnecessary surgical intervention, particularly in an asymptomatic patient.
Highlights
Extramedullary hematopoiesis (EMH), occurring as a compensatory mechanism for bone marrow dysfunction, is almost always associated with hemoglobinopathies, including thalassemias, sickle cell disease and hereditary spherocytosis (HS), and myelofibrosis, as well as other bone marrow disorders [1]
The current study presents the case of a patient with HS whose primary complaints were abdominal pain and jaundice
The liver, spleen and lymph nodes are the usual sites of EMH, but it may occur in other sites, including the thymus, kidneys, retroperitoneum, lungs, breasts, skin, brain, adrenal glands and paravertebral areas of the thorax [1,2,3,4,5]
Summary
Extramedullary hematopoiesis (EMH), occurring as a compensatory mechanism for bone marrow dysfunction, is almost always associated with hemoglobinopathies, including thalassemias, sickle cell disease and hereditary spherocytosis (HS), and myelofibrosis, as well as other bone marrow disorders [1]. The most common sites of involvement are the spleen, liver and lymph nodes, practically. The current study presents the case of a patient with HS whose primary complaints were abdominal pain and jaundice. The chest radiograph and thoracic computed tomography (CT) scan incidentally revealed posterior mediastinum paravertebral masses, which were diagnosed as EMH by the CT‐guided needle aspiration biopsy.
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