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      Renal biopsy cases in myeloproliferative neoplasms (MPN)

      case-report

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          Abstract

          We performed renal biopsy in three cases complicated by myeloproliferative neoplasms (MPN). Although several cases of glomerulonephritis associated with MPN have been reported, the etiologies of the renal disorders were not established (Plomley et al., Aust NZ J Med, 13:125–129, 1983; Sharma et al., Nephron, 69:361, 1995; Kanauchi et al., Intern Med, 33:36–40, 1994; Kasuno et al., Nephrol Dial Transplant, 12:212–215, 1997; Au et al., Am J Kid Dis, 34:889–893, 1999; Kosch et al., Nephrol Dial Transplant, 15:1710–1711, 2000; Oymak et al., Nephron, 86:346–347, 2000; Chun et al., Am J Nephrol, 20:344–346, 2000; Chung et al., Am J Nephrol, 22:397–401, 2002; Asaba et al., Clin Exp Nephrol, 7:296–300, 2003; Haraguchi et al., Clin Exp Nephrol, 10:74–77, 2006; Saigusa et al., J Nephrol, 19:656–658, 2006; Okuyama et al., Clin Nephrol, 6:412–415, 2007; Nishi et al., Clin Nephrol, 5:393–398, 2010; Ulusoy et al., Intern Med, 49:2477, 2010). A review of previous reports of renal biopsy cases with MPN in the English literature suggested that circulation control is important for the treatment of renal disorders that mimic glomerulonephritis in MPN.

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          Glomerular cell proliferation and PDGF expression precede glomerulosclerosis in the remnant kidney model.

          Increasing evidence supports a role of glomerular cell proliferation in the development of focal or diffuse glomerulosclerosis. This study investigates the chronology and sequence of cellular events that precede glomerulosclerosis in 5/6 nephrectomized rats. Within three days of renal ablation, a phenotypic switch occurred in which some mesangial cells expressed alpha-smooth muscle actin. This was followed by proliferation of mesangial cells, and to a lesser degree endothelial cells from day 5 to week 4 as detected by immunostaining for the proliferating cell nuclear antigen (PCNA). Glomerular cell proliferation was accompanied by increased immunohistochemical expression of PDGF B-chain. In situ hybridization showed no glomerular PDGF B-chain mRNA expression at the induction of proliferation (day 5), and a marked increase between week 1 and 4 in operated rats. In parallel, increased expression of PDGF receptor beta-subunit protein and mRNA was demonstrated by immunohistochemistry and Northern analysis of total glomerular RNA. The onset of glomerular cell proliferation was also associated with mild glomerular platelet accumulation (as defined by 111In-labelled platelet studies) as well as with fibrinogen deposition. Proteinuria, glomerular sclerotic changes, and leukocyte infiltration all followed cell proliferation. The glomerular leukocyte infiltrate consisted of monocytes/macrophages and increased markedly at week 10 in rats with renal ablation. Thus, our results suggest that in the remnant kidney model: 1) proliferation of intrinsic glomerular cells precedes glomerulosclerosis; 2) proliferation may be initiated by degranulating platelets and sustained by PDGF released from intrinsic glomerular cells; and 3) glomerular monocyte/macrophage infiltration occurs after the proliferation, and may possibly contribute to the development of glomerular sclerotic changes.
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            Focal segmental glomerulosclerosis and mesangial sclerosis associated with myeloproliferative disorders.

            The myeloproliferative disorders (MPDs) are clonal disorders of the hematopoietic stem cell and classified as polycythemia vera (PV), essential thrombocythemia (ET), or agnogenic myeloid metaplasia (AMM), depending on the main hematopoietic lineage involved. Primary renal parenchymal lesions are not commonly reported in these cases. We conducted a retrospective analysis of 138 consecutive patients with MPD to determine the frequency of renal parenchymal complications. Five patients (3.6%) (two PV, two ET, one AMM) were found to have focal segmental glomerulosclerosis (FSGS) and diffuse mesangial sclerosis, presenting as proteinuria in all the cases and progressing to chronic renal failure in two cases. A possible common risk factor was a high platelet count, because abnormal platelet activation in MPD has been shown to contribute to the development of glomerulosclerosis. The pathophysiologic basis of our observations and the implications in management of MPD patients remain to be studied.
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              Absence of hypoalbuminemia despite nephrotic proteinuria in focal segmental glomerulosclerosis secondary to polycythemia vera.

              In addition to displaying geographic variation, focal segmental glomerulosclerosis (FSGS) has become the commonest cause of the nephrotic syndrome seen in adults in recent years. Secondary FSGS in particular, is observed when glomerular workload is increased. Polycythemia vera (PV) is a hematological disease characterized by abnormal proliferation in the erythroid series. The number of case reports belonging to glomerulonephritis secondary to PV is limited. In the literature, there are few reports of FSGS. One study pointed out that the presence of normoalbuminemia was detected in patients with FSGS secondary to hyperfiltration when there was nephrotic proteinuria. Here, we report a case of FSGS following a course with normoalbuminemia despite nephrotic range proteinuria developing secondary to PV. Our case is the first report in the literature with thes characteristics.
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                Author and article information

                Contributors
                0743-63-5611 , 0743-63-1530 , fujiko@tenriyorozu.jp
                Journal
                CEN Case Rep
                CEN Case Rep
                CEN Case Reports
                Springer Japan (Tokyo )
                2192-4449
                12 March 2013
                12 March 2013
                November 2013
                : 2
                : 2
                : 215-221
                Affiliations
                [1 ]Tenri Institute of Medical Research, 200 Mishima-cho, Tenri, Nara Japan
                [2 ]GRID grid.416952.d, ISNI 0000000403784277, , Tenri Hospital, Department of General Medicine, ; 200 Mishima-cho, Tenri, Nara Japan
                Article
                67
                10.1007/s13730-013-0067-0
                5411553
                28509296
                3ca0ccb7-e951-41ee-8474-dc91e153fa56
                © The Author(s) 2013
                History
                : 6 November 2012
                : 4 February 2013
                Categories
                Case Report
                Custom metadata
                © Japanese Society of Nephrology 2013

                polycythemia vera,essential thrombocytosis,renal biopsy,glomerulonephritis,antihypertensive agent

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