EPO levels are also increased in patients with anemia of bone marrow failure, iron deficiency, or thalassemia. In polycythemia vera, the EPO levels are low as a response to an increased production of red blood cells. Because in some cases the elevated hematocrit is physiologic, phlebotomy should be limited to the extent necessary to relieve symptoms (in contrast to polycythemia vera, where the goal is to normalize the hematocrit). High blood levels of RBC, hemoglobin, hematocrit, or oxygen suppress the release of EPO. Musculoskeletal and Connective Tissue Disorders. Standard hemoglobin electrophoresis may be normal and cannot reliably exclude this cause of erythrocytosis. Common causes of secondary erythrocytosis include, Less common causes include certain congenital disorders such as, Chuvash polycythemia (in which a mutation in the VHL gene affects the hypoxia-sensing pathway), Right to left arteriovenous shunts in the lungs, Proline hydroxylase 2 and hypoxia-inducible factor 2 alpha (HIF-2α) mutations. In tumors secreting erythropoietin, the EPO levels may be abnormally high. Secondary polycythemia may result from increased production of erythropoietin. It has 3 oligosaccharide chains and a molecular mass of 30.4 kDa. Exp Biol Med. Despite the newly-diagnosed polycythemia, the patient denied any significant symptoms or history of blood clots. Controversies exist regarding the diagnostic value of a low EPO level when considering increasing availability of advanced molecular testing. addition of EPO.12,13 This unique finding, along with serum EPO levels, forms the basis for a new diagnostic approach, 5 but has the disadvantages of … In individuals with polycythemia vera, EPO levels are abnormally low. All the other patients with wild-type JAK2 and a normal or elevated EPO level have secondary polycythemia. A disease or the use of certain drugs can cause this type. Erythropoietin levels may be increased with certain kidney diseases, and erythropoietin-like substances may be secreted by certain tumors. This can be a serious clinical situation that can result in red cell aplasia and should prompt expeditious referral to hematologists or immunologists skilled in diagnosing and treating this disorder. Author information: (1)Texas Tech University Health Sciences Center, School of Medicine, Lubbock, TX, USA. This hormone, which is possibly produced and secreted by the kidneys, stimulates bone marrow production of RBCs. This is an overproduction of red blood cells that occurs in response to an event such as low blood oxygen level. Patients with elevated erythropoietin levels (and no indication of hypoxia) or microscopic hematuria should undergo abdominal imaging, central nervous system imaging, or both to seek a renal lesion or other tumor sources of erythropoietin. Secondary polycythemia may also be caused by increased levels of erythropoietin, a hormone that stimulates RBC produc-tion. Appropriate secondary polycythemias (eg, high-altitude living and pulmonary disease) are characterized by hypoxia and a compensatory increase in red cell mass. The diurnal variation is minimal in normal individuals (<20%), but in hospitalized patients with a variety of illnesses, as well as ambulatory patients with chronic lung disease, serum EPO concentrations can be 20% to 60% higher at night than early in the morning. Low erythropoietin (EPO) level may have only moderate predictive accuracy for polycythemia vera (PV), according to study results published in Blood Cells, Molecules and Diseases. Abnormal EPO levels also may be seen in renal failure. The Manual was first published as the Merck Manual in 1899 as a service to the community. Secondary polycythemia most often develops as a response to chronic hypoxemia, which triggers increased production of erythropoietin by the kidneys. In addition to the kidneys, the liver also produces a small amount of EPO. Patients with chronic hypoxemia (arterial hemoglobin oxygen concentration < 92%), typically due to lung disease, right-to-left intracardiac shunts, renal transplantation, prolonged exposure to high altitudes, or hypoventilation syndromes, often develop erythrocytosis. Kopel J(1), Sharma P(2), Warriach I(3), Swarup S(4). 1. In secondary erythrocytosis, only red blood cells (RBCs) are increased, whereas in polycythemia vera, RBCs, white blood cells (WBCs), and platelets will usually be increased. A total of 80.88% (n = 351) of those diagnosed with PV had a JAK2-V617F mutation compared to only 1.47% (n = 2) whose primary diagnosis was secondary polycythemia. erythropoietin is a hormone that tells your bone marrow to make new blood cells. We reviewed current guidelines on the management of polycythemia vera. secondary polycythemia: Definition Secondary polycythemia is an acquired form of a rare disorder characterized by an abnormal increase in the number of mature red cells in the blood. 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