Despite the newly-diagnosed polycythemia, the patient denied any significant symptoms or history of blood clots. Merck & Co., Inc., Kenilworth, NJ, USA (known as MSD outside of the US and Canada) is a global healthcare leader working to help the world be well. a very low level can be another sign of polycythemia vera. Casadeval N: Pure red cell aplasia and anti-erythropoietin antibodies in patients treated with epoetin. Erythropoietin levels may be increased with certain kidney diseases, and erythropoietin-like substances may be secreted by certain tumors. erythropoietin is a hormone that tells your bone marrow to make new blood cells. All the other patients with wild-type JAK2 and a normal or elevated EPO level have secondary polycythemia. 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. The increased production may be an appropriate (compensatory) physiologic response to hypoxemia, which may result from: chronic obstructive pulmonary disease Also the total percentage of anemia in COPD patients was 46.3% (19/41), in comparison to 51.3% (21/41) non anemic and 2.4% (1/41) polycythemic. Conversely, an EPO level >3.3 IU/L has a sensitivity of 97% for detecting secondary polycythemia. Drug levels can only be roughly estimated from the cross reactivity of the compounds in EPO assays. Secondary polycythemia would more accurately be called secondary erythrocytosis or erythrocythemia, as those terms specifically denote increased red blood cells. The Manual was first published as the Merck Manual in 1899 as a service to the community. In secondary polycythemia, the number of red blood cells (RBCs) is increased as a result of an underlying condition. doi: 10.1186/2110-5820-1-3, 8. 1 People living at high altitudes may have higher EPO levels than people living at lower altitudes. Common causes of secondary erythrocytosis include Drug levels can only be roughly estimated from the cross reactivity of the compounds in EPO assays. The most common causes of secondary polycythemia include obstructive sleep apnea, obesity hypoventilation syndrome, and chronic obstructive pulmonary disease (COPD). It has 3 oligosaccharide chains and a molecular mass of 30.4 kDa. Increased RBCs result in compensatory suppression of EPO levels. A follow-up study was carried out in some of these patients (27 with secondary polycythemia and 17 with polycythemia vera). Ann Intensive Care. This diagnosis is suggested by a family history of erythrocytosis; it is established by measuring the P50 (the partial pressure of oxygen at which hemoglobin becomes 50% saturated) and, if possible, determining the complete oxyhemoglobin dissociation curve. RESULTS: sEpo levels were higher in SP than in PV and relative polycythemia. This phenomenon is most pronounced in patients with EPO levels within approximately 2-times the upper limit of the normal population reference interval. Low EPO level can be used to diagnose PV, but there has been debate over its diagnostic value in light of the increasing availability of advanced molecular testing. Treatments 5. In polycythemia vera, the EPO levels are low as a response to an increased production of red blood cells. A… High blood levels of RBC, hemoglobin, hematocrit, or oxygen suppress the release of EPO. This is an overproduction of red blood cells that occurs in response to an event such as low blood oxygen level. Clinical Signs With relative polycythemia, vomiting or diarrhea may be pres- From developing new therapies that treat and prevent disease to helping people in need, we are committed to improving health and well-being around the world. Describes reference intervals and additional information for interpretation of test results. The trusted provider of medical information since 1899, Reactive Thrombocytosis (Secondary Thrombocythemia). Hoagland HC: Myelodysplastic (preleukemia) syndromes: the bone marrow factory failure problem. A low serum-erythropoietin (S-epo) level is a minor criterion of the World Health Organization (WHO) recommendations for diagnosing polycythemia vera (PV) even though previous studies indicate that a normal level does not always rule out PV. The legacy of this great resource continues as the MSD Manual outside of North America. EPO levels are also increased in patients with anemia of bone marrow failure, iron deficiency, or thalassemia. Primary polycythemia (polycythemia vera) is a neoplastic (clonal) blood disorder characterized by autonomous production of hematopoietic cells. Primary polycythemia (polycythemia vera) is a neoplastic (clonal) blood disorder characterized by autonomous production of hematopoietic cells. Learn more about our commitment to Global Medical Knowledge. There are no specific assays for measuring recombinant EPO compounds. Secondary polycythemia most often develops as a response to chronic hypoxemia, which triggers increased production of erythropoietin by the kidneys. This assay cannot distinguish between endogenous and exogenous EPO. Erythropoietin (EPO) levels alone cannot reliably distinguish between primary and secondary polycythemia; EPO levels are within normal limits in some patients with primary polycythemia. Exp Biol Med. 2006 Oct 18;(4):CD003967, 6. Heterophile antibodies may interfere in this assay. The Manual was first published as the Merck Manual in 1899 as a service to the community. A disease or the use of certain drugs can cause this type. Clinical Signs With relative polycythemia, vomiting or diarrhea may be pres- Kopel J(1), Sharma P(2), Warriach I(3), Swarup S(4). Secondary polycythemia may also be caused by increased levels of erythropoietin, a hormone that stimulates RBC produc-tion. High oxygen–affinity hemoglobinopathies are very rare. 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. According to in-house studies, epoetin and darbepoetin show approximately 58% and 36% cross-reactivity, respectively, in the EPO assay. 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. Secondary polycythemias may either be due to an appropriate or an inappropriate increase in red cell mass. In addition to the kidneys, the liver also produces a small amount of EPO. In secondary polycythemia, 6 to 8 million and occasionally 9 million erythrocytes may occur per cubic millimeter of blood. The link you have selected will take you to a third-party website. 2011 March;1(3). In individuals with polycythemia vera, EPO levels are abnormally low. Evidence used in this review. Spurious erythrocytosis may occur with hemoconcentration (eg, due to burns, diarrhea, or diuretic use). 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. Results: A large majority of PV patients (87% or 101/116) had a serum Epo level below the normal range in healthy patients (3.3 IU/L), giving this value a specificity of 97% with a 97.8% positive predictive value for the diagnosis of PV. People living at high altitudes may have higher EPO levels than people living at lower altitudes. High blood levels of RBC, hemoglobin, hematocrit, or oxygen suppress the release of EPO. An EPO level <1.4 IU/L is 100% specific for a diagnosis of PV, while an EPO level of >13.7 IU/L is 100% specific for the diagnosis of secondary polycythemia. , MD, James P. Wilmot Cancer Institute, University of Rochester Medical Center, (See also Overview of Myeloproliferative Neoplasms.). (2)Department of Urology, Texas Tech University Health Sciences Center, Lubbock, TX, USA. © 1995–2021 Mayo Foundation for Medical Education and Research. Erythropoietin (EPO) levels alone cannot reliably distinguish between primary and secondary polycythemia; EPO levels are within normal limits in some patients with primary polycythemia. Epoetin alpha is a 165 amino acid glycoprotein produced in mammalian cells and has an identical amino acid sequence to natural human EPO. Introduction. Mayo Clin Proc. 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. This hormone, which is possibly produced and secreted by the kidneys, stimulates bone marrow production of RBCs. Tumor-associated erythrocytosis can occur when renal tumors, cysts, hepatomas, cerebellar hemangioblastomas, or uterine leiomyomas secrete erythropoietin. This site complies with the HONcode standard for trustworthy health information: verify here. 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. METHODS: sEpo was assessed in 190 patients with polycythemia of different etiologies. Controversies exist regarding the diagnostic value of a low EPO level when considering increasing availability of advanced molecular testing. Hypoxia stimulates EPO release, which, in turn, stimulates bone marrow erythrocyte production. The majority of EPO production is in the kidneys. Patients, who have either a poor or no erythropoietic response to EPO therapy, but high-normal or high EPO levels, may have additional, unrecognized causes for their anemia. A serum erythropoietin assay is only helpful if elevated, since this excludes polycythemia vera as a cause for erythrocytosis; a normal erythropoietin level … Erythropoietin (EPO) levels alone cannot reliably distinguish between primary and secondary polycythemia; EPO levels are within normal limits in some patients with primary polycythemia. 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. If no contributing factors can be identified after adequate further study, the possibility that the patient may have developed EPO-antibodies should be considered. When comparing JAK2-V617 mutation to the EPO level, the area under the curve of JAK2-V617 (0.8970) was statistically larger than that of EPO test (0.6765). Tefferi A: Polycythemia vera and essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management. 2003;228:1-14, 5. 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. Removal of the lesion is curative. 1999;74:159-162, 2. Secondary erythrocytosis is erythrocytosis that develops secondary to disorders that cause tissue hypoxia, inappropriately increased erythropoietin production, or increased sensitivity to erythropoietin. 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.. Low EPO level can be used to diagnose PV, but there has been debate over its diagnostic value in light of the increasing availability of advanced molecular testing. Am J Hematol. A history of the formation or presence of a blood clot inside a blood vessel (thrombosis) or loss o… According to Mayo Clinic in-house studies, epoetin and darbepoetin show approximately 58% and 36% cross reactivity, respectively, in the EPO assay. Erythropoietin (EPO) blood levels may also be helpful, although the results need to be interpreted carefully, as the level may be high in response to chronic hypoxia. 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. 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 … A 61-year-old obese Caucasian male with past medical history of smoking, hypertension, chronic obstructive pulmonary disease (COPD), and sleep apnea presented to the hematology clinic with polycythemia. May include intervals based on age and sex when appropriate. We do not control or have responsibility for the content of any third-party site. Showed that the erythropoietin level was 15.24 ± 2.6 in stage 1, 22.61 ± 5.68 in stage 2, 33.59 ± 4, in stage 3, then 17.9 ± 3.3 in stage 4. 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