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      Idiopathic erythrocytosis in IgA nephropathy

      case-report

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          Abstract

          We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN.

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          Circulating erythroid progenitors in polycythemia vera are hypersensitive to insulin-like growth factor-1 in vitro: studies in an improved serum-free medium.

          We have investigated the question of erythropoietin (Epo) hypersensitivity versus Epo independence as the basis for the endogenous erythroid bursts (EEBs) that develop in cultures without added Epo from hematopoietic cells of polycythemia vera (PV) patients. Using an improved serum-free (SF) medium containing interleukin (IL)-3, but no insulin-like growth factor-1 (IGF-1), and devoid of contaminants that influence erythropoiesis, we compared circulating normal and PV early erythroid progenitors (BFU-E) with respect to their responses in vitro to recombinant human (rHu) Epo. Cultures were seeded with Ficoll-Hypaque density-separated peripheral blood (PB) mononuclear cells (MNCs), and erythroid bursts, together with their component colonies of > or = 50 cells, were scored in situ at 13 to 16 days of culture. The Epo dose-response curve of BFU-E from PV patients was found to be statistically indistinguishable from that of normal subjects. This observation provides compelling evidence against the Epo-hypersensitivity hypothesis. In the complete SF medium minus Epo, the sensitivity of BFU-E to IGF-1 was much greater in PV than in normals, the dose-response curve being shifted to the left by at least 2 orders of magnitude. These data show that the erythroid progenitor cell response in PV is hypersensitive to IGF-1, and independent of Epo. The data also emphasize the importance of truly SF medium conditions for assessment of progenitor cell sensitivities to recombinant growth factors. Depletion of adherent cells totally prevented erythroid burst formation by normal circulating progenitors, but did not prevent the hypersensitive response to IGF-1 of such cells from PV patients. Hence, again unlike its normal counterpart, the progenitor cell response in PV appears to be independent of adherent cell control.
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            Polycythemia vera associated with IgA nephropathy: A case report and literature review.

            A case of polycythemia vera (PV) associated with immunoglobulin A nephropathy (IgAN) in a 57-year-old man is described. The patient had a mild enlargement of the kidneys and elevated serum creatinine level, whereas the glomerular filtration rate was normal. Pathological observation under a light microscope showed mild mesangial hyperplasia. The urinary protein level was found to be positively correlated with changes in blood cell counts. After controlling blood pressure, anti-platelet, hydroxyurea treatment, the patient's proteinuria decreased and renal function remained in the normal range. PV associated with renal disease is rare and generally considered to be associated with hypervolemia or high-viscosity-induced renal hyperperfusion and hyperfiltration. This is a rare case of PV associated with IgAN without a high filtration rate. A review of the clinical features of PV associated with renal disease from previous literature was also conducted. The histological results of the cases varied and included IgAN, focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis.
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              Thrombotic microangiopathy associated with interferon therapy for patients with chronic myelogenous leukemia: coincidence or true side effect?

              Interferon-alpha (rIFN-alpha) is an established therapy for patients with myeloproliferative disorders. Unusual immune-mediated side effects have been associated with rIFN-alpha therapy. The association of rIFN-alpha therapy with hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) has been reported infrequently. Two patients with chronic myelogenous leukemia (CML) treated with rIFN-alpha-based regimens at the University of Texas M. D. Anderson Cancer Center developed thrombotic microangiopathy (HUS/TTP). The course of their disease is described. A third patient who developed renal failure while receiving rIFN-alpha therapy and had no other causative factor for his renal failure is also described. The patients were ages 24, 49, and 36 years, and they had received rIFN-alpha therapy for 37, 67, and 92 months, respectively, prior to the development of the disorder. One patient had discontinued rIFN-alpha 1 month before the event because of presumed rIFN-alpha-related cardiomyopathy. Two patients received hydroxyurea and cytarabine as part of their therapy. No patient was receiving any medication known to be associated with HUS/TTP. None had a history of diarrheal illness, but Escherichia coli OH157.H7 was grown from the stool of one patient. Two patients responded to plasmapheresis with normalization of counts and other indices, but both developed renal failure and became dependent on dialysis. One patient had evidence of disease progression and died of multiorgan failure. The third patient required dialysis for 18 months but is currently off dialysis; this patient has some residual renal impairment. Although no definitive association between rIFN-alpha therapy and thrombotic microangiopathies can be concluded from these data, these and other previously reported cases suggest that HUS/TTP is a rare side effect of rIFN-alpha therapy that should be managed in the standard fashion. Hypotheses regarding the mechanism underlying this association are discussed in this article.
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                Author and article information

                Journal
                Indian J Nephrol
                Indian J Nephrol
                IJN
                Indian Journal of Nephrology
                Medknow Publications & Media Pvt Ltd (India )
                0971-4065
                1998-3662
                Jan-Feb 2017
                : 27
                : 1
                : 72-73
                Affiliations
                [1]Department of Nephrology, M S Ramaiah Medical College and Hospitals, Bengaluru, Karnataka, India
                Author notes
                Address for correspondence: Dr. P. R. Madhyastha, Department of Nephrology, M S Medical College and Hospitals, Bengaluru - 560 054, Karnataka, India. E-mail: rakeshmadhyastha@ 123456gmail.com
                Article
                IJN-27-72
                10.4103/0971-4065.194390
                5255996
                b46fa3fc-3f3c-4038-9b53-394998f64d25
                Copyright: © 2017 Indian Journal of Nephrology

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                Categories
                Case Report

                Nephrology
                erythrocytosis,iga nephropathy,nephrotic syndrome,polycythemia
                Nephrology
                erythrocytosis, iga nephropathy, nephrotic syndrome, polycythemia

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