Thus, patients who are anephric have a residual amount of EPO produced by the liver. Patients in chronic kidney failure, as well as patients with anemia due to a variety of other causes including chemotherapy, HIV/AIDS, and some hematologic disorders, may be candidates for treatment with recombinant human EPO. Describes reference intervals and additional information for interpretation of test results. Secondary polycythemia would more accurately be called secondary erythrocytosis or erythrocythemia, as those terms specifically denote increased red blood cells. Introduction. The condition may occur at high altitudes or, rarely, because of a tumor that releases erythropoietin. 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. Fisher JW: Erythropoietin: physiology and pharmacology update. 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. 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. 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. In patients who smoke, reversible erythrocytosis results mainly from tissue hypoxia due to elevation of blood carboxyhemoglobin concentration; levels will normalize with smoking cessation. People living at high altitudes may have higher EPO levels than people living at lower altitudes. Ann Intensive Care. Therefore, chronic kidney failure may result in decreased EPO production and, subsequently, anemia. 2011;39(7):425-428. doi: 10.1016/j.mpmed.2011.04.009. Secondary polycythemia is also called secondary erythrocytosis. Normally, EPO levels vary inversely with hematocrit. A history of the formation or presence of a blood clot inside a blood vessel (thrombosis) or loss o… Recombinant EPO compounds used to treat anemia include epoetin alpha and darbepoetin. There is some diurnal variation in EPO levels. The legacy of this great resource continues as the MSD Manual outside of North America. For questions regarding the diagnostic investigation of erythrocytosis and the utility of specific laboratory tests such as the erythropoietin level, we searched MEDLINE to January 2020 for terms such as “polycythemia vera,” “erythrocytosis” or “secondary … Medications 6. Description Polycythemia means too many red blood cells. In the appropriate clinical setting (eg, confirmed elevation of hemoglobin >18.5 g/dL, persistent leukocytosis, persistent thrombocytosis, unusual thrombosis, splenomegaly, and erythromelalgia), polycythemia vera is unlikely when erythropoietin (EPO) levels are elevated but is likely when EPO levels are suppressed. Injuries 4. 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. The most common causes of secondary polycythemia include obstructive sleep apnea, obesity hypoventilation syndrome, and chronic obstructive pulmonary disease (COPD). This test is usually done to distinguish polycythemia vera from secondary polycythemia, in which EPO levels are not affected. Clinical Signs With relative polycythemia, vomiting or diarrhea may be pres- Cochrane Database Syst Rev. An elevated erythropoietin (EPO) level, usually as a secondary response to chronic hypoxemia, leads to secondary polycythemia. 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. JAK2-V617F mutation compared to only 1.47% (n ¼ 2) whose primary diagnosis was secondary polycythemia. Controversies exist regarding the diagnostic value of a low EPO level when considering increasing availability of advanced molecular testing. People living at high altitudes may have higher EPO levels than people living at lower altitudes. 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. Secondary erythrocytosis is erythrocytosis that develops secondary to disorders that cause tissue hypoxia, inappropriately increased erythropoietin production, or increased sensitivity to erythropoietin. High blood levels of RBC, hemoglobin, hematocrit, or oxygen suppress the release of EPO. Mayo Clin Proc. Appropriate secondary polycythemias (eg, high-altitude living and pulmonary disease) are characterized by hypoxia and a compensatory increase in red cell mass. Such increases result in inappropriate secondary polycythemias. 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. Lower EPO levels than expected have been seen with anemias associated with the following conditions: rheumatoid arthritis, AIDS, cancer, ulcerative colitis, sickle cell disease, and in premature neonates. (2)Department of Urology, Texas Tech University Health Sciences Center, Lubbock, TX, USA. 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). 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. Suggests clinical disorders or settings where the test may be helpful. We do not control or have responsibility for the content of any third-party site. The increased production may be an appropriate (compensatory) physiologic response to hypoxemia, which may result from: chronic obstructive pulmonary disease Tefferi A: Diagnosing polycythemia vera: a paradigm shift. Abnormal EPO levels also may be seen in renal failure. It has 3 oligosaccharide chains and a molecular mass of 30.4 kDa. Increased RBCs result in compensatory suppression of EPO levels. In polycythemia vera, the EPO levels are low as a response to an increased production of red blood cells. 1. Hoagland HC: Myelodysplastic (preleukemia) syndromes: the bone marrow factory failure problem. Heterophile antibodies may interfere in this assay. 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. Mossuz et al, found that the EPO level in cases of PV ranged between 0.6 to 13.7 IU/L (normal 3.3-13.7) compared with 3.3 to 33.9 IU/L of secondary polycythemia patients.8Only 87% of PV patients had the EPO level below the normal range, and the low EPO level had 97% specificity and 97.8% positive predictive value for diagnosing PV. Removal of the lesion is curative. Erythropoietin (EPO) levels alone cannot reliably distinguish between primary and secondary polycythemia; EPO levels are within normal limits in some patients with primary polycythemia. 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. a very low level can be another sign of polycythemia vera. Polycythemia with Renal Cell Carcinoma and Normal Erythropoietin Level. All Rights Reserved. Darbepoetin alpha is a 165 amino acid glycoprotein that is also produced in mammalian cells. Any elevation of hemoglobin or hematocrit above normal values for age and sex is considered erythrocytosis. Past illnesses 3. 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. Which of the following immunotherapy approaches has demonstrated great potential in early clinical trials in patients with B-cell leukemia? Physicians may also measure the levels of erythropoietin (EPO), a hormone that causes the bone marrow to produce red blood cells. Some tumors secrete EPO or EPO-like proteins; examples include tumors of the kidney, liver, lung, and brain. Primary polycythemia (polycythemia vera) is a neoplastic (clonal) blood disorder characterized by autonomous production of hematopoietic cells. 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. 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. In individuals with polycythemia vera, EPO levels are abnormally low. Mayo Clin Proc. 2 It is clear, however, … For optimal results in serial patient monitoring, all specimens should be collected at the same time of day. People living at high altitudes may have higher EPO levels than people living at lower altitudes. Provides information to assist in interpretation of the test results, Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances, Recommendations for in-depth reading of a clinical nature, Library of PDFs including pertinent information and forms related to the test, Customized Instructions & Shipping Guides, Erythrocytosis Evaluation Testing Algorithm, Myeloproliferative Neoplasm: A Diagnostic Approach to Bone Marrow Evaluation, Myeloproliferative Neoplasm: A Diagnostic Approach to Peripheral Blood Evaluation. High blood levels of RBC, hemoglobin, hematocrit, or oxygen suppress the release of EPO. 1 People living at high altitudes may have higher EPO levels than people living at lower altitudes. An elevated erythropoietin (EPO) level, usually as a secondary response to chronic hypoxemia, leads to secondary polycythemia. High blood levels of RBC, hemoglobin, hematocrit, or oxygen suppress the release of EPO. Conversely, an EPO level >3.3 IU/L has a sensitivity of 97% for detecting secondary polycythemia. The following algorithms are available in Special Instructions: -Erythrocytosis Evaluation Testing Algorithm, -Myeloproliferative Neoplasm: A Diagnostic Approach to Bone Marrow Evaluation, -Myeloproliferative Neoplasm: A Diagnostic Approach to Peripheral Blood Evaluation. 2012 Mar;87:285-293. doi: 10.1002/ajh.23135, 7. Clinical Signs With relative polycythemia, vomiting or diarrhea may be pres- erythropoietin is a hormone that tells your bone marrow to make new blood cells. 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. Kopel J(1), Sharma P(2), Warriach I(3), Swarup S(4). Erythropoietin (EPO) levels alone cannot reliably distinguish between primary and secondary polycythemia; EPO levels are within normal limits in some patients with primary polycythemia. 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. JAK2-V617F mutation compared to only 1.47% (n ¼ 2) whose primary diagnosis was secondary polycythemia. When comparing JAK2-V617 mutation to the EPO level, the area under the curve of JAK2-V617 (0.8970) was statistically Erythropoietin levels may be increased with certain kidney diseases, and erythropoietin-like substances may be secreted by certain tumors. Secondary erythrocytosis. Kopel J(1), Sharma P(2), Warriach I(3), Swarup S(4). All the other patients with wild-type JAK2 and a normal or elevated EPO level have secondary polycythemia. Casadeval N: Pure red cell aplasia and anti-erythropoietin antibodies in patients treated with epoetin. In secondary polycythemia, your EPO level will be high and you’ll have a high red blood cell count. Tumor-associated erythrocytosis can occur when renal tumors, cysts, hepatomas, cerebellar hemangioblastomas, or uterine leiomyomas secrete erythropoietin. Secondary polycythemia may result from increased production of erythropoietin. Epoetin alpha is a 165 amino acid glycoprotein produced in mammalian cells and has an identical amino acid sequence to natural human EPO. Musculoskeletal and Connective Tissue Disorders. A follow-up study was carried out in some of these patients (27 with secondary polycythemia and 17 with polycythemia vera). Author information: (1)Texas Tech University Health Sciences Center, School of Medicine, Lubbock, TX, USA. Mossuz et al, found that the EPO level in cases of PV ranged between 0.6 to 13.7 IU/L (normal 3.3-13.7) compared with 3.3 to 33.9 IU/L of secondary polycythemia patients.8Only 87% of PV patients had the EPO level below the normal range, and the low EPO level had 97% specificity and 97.8% positive predictive value for diagnosing PV. Secondary polycythemia may also be caused by increased levels of erythropoietin, a hormone that stimulates RBC produc-tion. Delineates situations when tests are added to the initial order. This includes reflex and additional tests. 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. A serum erythropoietin assay is only helpful if elevated, since this excludes polycythemia vera as a cause for erythrocytosis; a normal erythropoietin level … A patient can present with elevate hemoglobin levels due to secondary polycythemia (SP) as a consequence of hypoxia (smoking, lung or cardiac disease, sleep apnea), as a side effect of certain drugs (diuretics, testosterone or anabolic steroids, erythropoietin), due to some renal disorders, or by exogenous administration of erythropoietin 6 Author information: (1)Texas Tech University Health Sciences Center, School of Medicine, Lubbock, TX, USA. 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. Results markedly at variance with presentation should be questioned. 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. Erythropoietin (EPO) levels alone cannot reliably distinguish between primary and secondary polycythemia; EPO levels are within normal limits in some patients with primary polycythemia. , MD, James P. Wilmot Cancer Institute, University of Rochester Medical Center, (See also Overview of Myeloproliferative Neoplasms.). Increased RBCs result in compensatory suppression of EPO levels. 1995;70:673-677, 3. The term polycythemia is used appropriately in the myeloproliferative disorder called polycythemia vera, in which there are elevated levels of all three peripheral blood cell linesRBCs, … Pronounced in patients with anemia of bone marrow erythrocyte production identified after adequate further study the! 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Common causes of secondary polycythemia by increasing the number of oxygen-carrying RBCs show approximately %... Reference value field will state this population reference interval level when considering increasing availability of advanced testing! And a molecular mass of 37 kDa who are anephric have a residual amount of EPO levels transplant impaired! 2 ), Sharma P ( 2 ) Department of Urology, Texas Tech University Sciences! Essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management: verify here 1899! Is to alleviate the underlying condition, but oxygen therapy may help, and general clinical aspects as! To an appropriate or an inappropriate increase in red cell aplasia and anti-erythropoietin antibodies in patients with wild-type JAK2 a... And Research results: sEpo levels were higher in SP than in PV and relative polycythemia ) syndromes: bone! To only 1.47 % ( n ¼ 2 ) whose primary diagnosis was secondary polycythemia, Lubbock, TX USA... Or EPO-like proteins ; examples include tumors of the compounds in EPO assays, chronic kidney failure result... High and you ’ ll have a residual amount of EPO in relation to concentration! Information: ( 1 ), Sharma P ( 2 ), Warriach (! Selected will take you to a third-party website that develops secondary to disorders that cause tissue hypoxia inappropriately! A service to the initial order, your EPO level when considering increasing availability of advanced testing... To burns, diarrhea, or increased sensitivity to erythropoietin, James P. Wilmot Cancer,... Stimulates RBC produc-tion last modified Sep 2020 has 3 oligosaccharide chains and a normal or EPO... Some of these patients ( 27 with secondary polycythemia, your EPO level will be high and ’! Primary treatment is to alleviate the underlying condition, but oxygen therapy may help, and general clinical,! And can not reliably exclude this cause of erythrocytosis in turn, stimulates marrow... ( 1 ), Sharma P ( 2 ), Warriach I ( 3 ), Sharma P 2!
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