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      Polycythemia vera Concurrent with Chronic Renal Failure

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      S. Karger AG

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          Polycystic kidney disease and polycythemia vera. Occurrence in a patient receiving hemodialysis.

          Symptomatic erythrocytosis developed in a 59-year-old man with polycystic kidney disease (PKD) while he was receiving maintenance hemodialysis. Major clinical and laboratory data suggested a diagnosis of polycythemia vera (PV), despite a normal serum alkaline phosphatase level and leukocyte count. Secondary erythrocytosis, related to chronic hypoxemia and increased erythropoietin production, was excluded by appropriate laboratory studies. Despite previous documentation of secondary erythrocytosis in patients receiving hemodialysis, to my knowledge, PV has not been described in this population.
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            End-Stage Renal Disease following Polycythemia vera: In vitro and in vivo Response of Erythroid Progenitors to Erythropoietin and Effects of Sera on Normal Erythropoiesis

            We investigated the in vitro erythroid progenitor growth and the effects of sera on normal-marrow CFU-E (colony-forming units – erythroid) growth in 2 patients with renal failure on regular hemodialysis following a prior history of polycythemia vera (PV). PV was diagnosed 3 and 11 years, respectively, before the development of terminal renal failure. One of the patients had entered a spent phase of PV as characterized by diffuse extensive myelofibrosis and anemia; the other also had mild myelofibrosis. The serum erythropoietin (EPO) levels were low or normal on serial measurements by radioimmunoassay. There was no correlation between the hematocrit values and serum EPO levels. EPO-independent erythroid colonies were present in the cultures of bone marrow and peripheral blood cells from both patients after renal failure in the anemic state. With the addition of various concentrations of EPO, the number of erythroid colonies increased as the concentrations of EPO increased which was in accordance with the clinical observation that 1 patient with postpolycythemic myeloid metaplasia partially responded to recombinant human EPO therapy. In the EPO-dependent CFU-E assay, normal-marrow CFU-E numbers supported by 10% of the patient sera were less than those by normal sera. In the absence of EPO in cultures, no erythropoietic activity was found in the patients’ sera. Our study on uremic patients with underlying PV showed that the biologic characteristics of autonomous erythroid progenitor growth for PV persisted during the spent phase and after the development of terminal renal failure with anemia. The erythroid progenitors responded to EPO both in vitro and in vivo. Their sera exhibited an inhibiting effect on the growth of normal-marrow erythroid progenitors.

              Author and article information

              S. Karger AG
              December 1998
              07 December 1998
              : 80
              : 4
              : 486-487
              Departments of Internal Medicine and Nephrology, Gülhane Military Medical Academy, Ankara, Turkey
              45231 Nephron 1998;80:486–487
              © 1998 S. Karger AG, Basel

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              References: 5, Pages: 2
              Self URI (application/pdf): https://www.karger.com/Article/Pdf/45231
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              Cardiovascular Medicine, Nephrology


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