Extramedullary hematopoiesis (EMH or sometimes EH) refers to hematopoiesis occurring outside of the medulla of the bone (bone marrow). It can be physiologic or pathologic.
Physiologic EMH occurs during embryonic and fetal development; during this time the main site of fetal hematopoiesis are liver and the spleen.
Pathologic EMH can occur during adulthood when physiologic hematopoiesis can't work properly in the bone marrow and the hematopoietic stem cells (HSC) have to migrate to other tissues in order to continue with the formation of blood cellular components. Pathologic EMH can be caused by myelofibrosis, thalassemias or disorders caused in the hematopoietic system.
During fetal development, hematopoiesis occurs mainly in the fetal liver and in the spleen followed by localization to the bone marrow. Hematopoiesis also takes place in many other tissues or organs such as the yolk sac, the aorta-gonad mesonephros (AGM) region, and lymph nodes. During development, vertebrates go through a primitive and a definitive phase of hematopoiesis. The lungs also play a role in platelet production in adults.
Primitive hematopoiesis occurs in the yolk sac during early embryonic development. It is characterized by the production of primitive nucleated erythroid cells, which is thought to originate from endothelial cells or hemangioblasts, which are capable of forming both endothelium and primitive blood cells. The main objective of the production of these cells will be the facilitation of tissue oxygenation to support rapid embryonic growth. This primitive phase is transitory and the cells that are produced express embryonic hemoglobins (HBZ and HBE1 genes produce the alpha and beta chains, respectively) aren't pluripotent, and aren't capable of self-renewal.
Definitive hematopoiesis differs from the primitive phase through the production of hematopoietic stem cells. The formation of these cells occurs in the AGM (aorta-gonad-mesonephros) later in development.
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