2022•Pediatric Hematology Oncology JournalOpen access

Clinicopathological characteristics and management of extramedullary hematopoiesis: A review

Sweety Gupta, Ajay S Krishnan, Jyoti Prakash Singh, Amit Gupta, Manoj Gupta

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Abstract

Extramedullary hematopoiesis (EMH) is a rare entity most often associated with primary myelofibrosis, chronic myeloproliferative neoplasms, chronic hemolytic states, such as thalassemia, sickle cell anemia and certain solid tumors. Various theories have been postulated as the mechanism for occurrence of EMH. Iron deposition and/or fatty infiltration along with increased density of liver and spleen may be observed on imaging. Also, organs may show diffuse involvement or mass like foci of hematopoiesis on imaging. Treatment includes transfusion, surgery, hydroxyurea and radiation. In this review we narrate the clinicopathological features, radiological imaging and treatment strategies of non hepatosplenic Extramedullary hematopoiesis.

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What this paper is about

Extramedullary hematopoiesis (EMH) is a rare entity most often associated with primary myelofibrosis, chronic myeloproliferative neoplasms, chronic hemolytic states, such as thalassemia, sickle cell anemia and certain solid tumors. Various theories have been postulated as the mechanism for occurrence of EMH. Iron deposition and/or fatty infiltration along with increased density of liver and spleen may be observed on imaging. Also, organs may show diffuse involvement or mass like foci of hematopoiesis on imaging. Treatment includes transfusion, surgery, hydroxyurea and radiation. In this review we narrate the clinicopathological features, radiological imaging and treatment strategies of non hepatosplenic Extramedullary hematopoiesis.

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Available abstract

Extramedullary hematopoiesis (EMH) is a rare entity most often associated with primary myelofibrosis, chronic myeloproliferative neoplasms, chronic hemolytic states, such as thalassemia, sickle cell anemia and certain solid tumors. Various theories have been postulated as the mechanism for occurrence of EMH. Iron deposition and/or fatty infiltration along with increased density of liver and spleen may be observed on imaging. Also, organs may show diffuse involvement or mass like foci of hematopoiesis on imaging. Treatment includes transfusion, surgery, hydroxyurea and radiation. In this review we narrate the clinicopathological features, radiological imaging and treatment strategies of non hepatosplenic Extramedullary hematopoiesis.

Key concepts: Extramedullary hematopoiesis, Myelofibrosis, Medicine, Thalassemia, Pathology, Polycythemia vera, Haematopoiesis, Anemia

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