Development of Focal Segmental Glomerulosclerosis in a Patient with Polycythemia Vera: can Polycythemia Vera be a cause of Focal Segmental Glomerulosclerosis?
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"tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "852" "paginaFinal" => "854" ] ] "autores" => array:1 [ 0 => array:3 [ "autoresLista" => "Erim Gulcan, Rahsan Yildirim, Koray Uludag, Mustafa Keles, Abdullah Uyanik" "autores" => array:5 [ 0 => array:4 [ "nombre" => "Erim" "apellidos" => "Gulcan" "email" => array:1 [ 0 => "drerimgulcan@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 1 => array:3 [ "nombre" => "Rahsan" "apellidos" => "Yildirim" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] ] ] 2 => array:3 [ "nombre" => "Koray" "apellidos" => "Uludag" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "affc" ] ] ] 3 => array:3 [ "nombre" => "Mustafa" "apellidos" => "Keles" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 4 => array:3 [ "nombre" => "Abdullah" "apellidos" => "Uyanik" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Department of Nephrology, Ataturk University Medical Faculty, Erzurum, Turkey, " "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] 1 => array:3 [ "entidad" => "Department of Hematology, Ataturk University Medical Faculty, Erzurum, Turkey, " "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] 2 => array:3 [ "entidad" => "Department of Nephrology, Erzurum Research and Education Hospital, Erzurum, Turkey, " "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "affc" ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig1" "etiqueta" => "Tab. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "11562_108_35467_en_t1.jpg" "Alto" => 821 "Ancho" => 1453 "Tamanyo" => 273814 ] ] "descripcion" => array:1 [ "en" => "Haematological, chemistrical, serological and urinalysis findings of patient" ] ] ] "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">Dear Editor,</span></p><p class="elsevierStylePara">Polycythemia vera (PV) is a myeloproliferative disorder of unknown etiology. This condition is characterized by the abnormal proliferation of erythroid and myeloid series cells in the bone marrow.<span class="elsevierStyleSup">1</span> Focal segmental glomerulosclerosis (FSGS) is a glomerular disease characterized by the presence of nephrotic syndrome, hypertension, and the progressive deterioration of the renal function. Etiology is usually unknown, but it may be seen in secondary conditions.<span class="elsevierStyleSup">2-3 </span>PV in association with FSGS is rare.<span class="elsevierStyleSup">4 </span>As far as we know, only eight cases have been reported in the literature.<span class="elsevierStyleSup">4-10</span> In the report, we have presented a patient who development of FSGS associated with PV.</p><p class="elsevierStylePara">A 46-year-old male patient diagnosed with PV six years earlier was referred to the nephrology clinic due to the detection of proteinuria on routine controls. No important features were found on his history except for his use of the azathioprine for a month. Through a 24-hour urine analysis, 4g/day proteinuria was detected in the patient. The patient was admitted to the clinic. In physical examination is normal without arterial blood pressure of 140/90 and the spleen was 5cm palpable. Renal size and parenchyma were normal in abdominal ultrasonography. Laboratory tests results and examinations of glomerular disease have been showed at Table 1. Hence, causes of nephrotic syndrome were excluded. Renal biopsy was performed. In light microscope were shown 29 glomeruli. Global sclerosis and hyalinization were shown in five glomerule. There was intensive segmental sclerosis in more small segments of the other two to three glomeruli. The also remaining some glomeruli have mesengial cell proliferation and expansion. In Bowmans capsule of one to two glomeruli presence of synechiae noted. Tubulointerstitial area has been examined, focal interstitial mononuclear cell infiltration has been observed. In particular areas of inflammation have attenuation of some tubules epithelium and in the presence of eosinophilic material in the lumen characterized by atrophic changes were observed. In vascular stuctures were normal except for a slight thickening of the wall. Glomerulosclerosis, segmental sclerotic areas and slight thickening of the glomerular basement membrane have been detected through the use of Trichome stain. Furthermore, amiloid staining and immunofluorescence study showed a negative. All above these findings were indicative of FSGS. In arterial blood pressure monitoring, stage 1 hypertension was determined. Perindopril, azathioprine, and ASA were prescribed and the patient was discharged.</p><p class="elsevierStylePara">FSGS is a clinical and pathological disorder involving primarily the glomerulus.<span class="elsevierStyleSup">2-3</span> Progressive glomerular scarring is the most important feature in this disease. Early in the disease process, glomerulosclerosis is both focal, and segmental in nature. Furhermore, in later stages of the disease diffuse and global glomerulosclerosis develops. The loss of filtration barrier, depletion of podocytes and genetic susceptibility are the culprit factors in pathogenesis of FSGS.<span class="elsevierStyleBold"> </span>The condition can be idiopathic or occur secondary to obesity, intrarenal hemodynamic alterations, conditions with glomerulomegaly, the reduced number of nephron, and renal atheroembolic disease.<span class="elsevierStyleSup">2,3 </span>The tendency to thromboses may occur in PV which one of the chronic myeloproliferative disorders.<span class="elsevierStyleSup">1</span> It has been suggested that the increase level of red blood cells, elevation of the platelet count, increase in tissue factor, polymorphonuclear leukocytes, coagulation reactions related to the platelet surface and the presence of microparticles were culprit factors.<span class="elsevierStyleSup">11 </span></p><p class="elsevierStylePara">In the light of these data, we hypotezised that PV may cause of FSGS via recurrent thrombosis in microvascular level. Furthermore, it is well known that atheroembolic disease is a cause of FSGS. Thus, our case is important for present to develop of FSGS in the patient with PV. In the existing literature, a small number of cases of FSGS that are thought to be due to PV have been reported.<span class="elsevierStyleSup">4-10</span> In addition, 3.6% (only two PV) incidence of FSGS has been reported in patients with myeloproliferative disease.<span class="elsevierStyleSup">10</span> It has been expressed in these case reports that hyperviscosity from increased hematocrit, hypoperfusion, predisposition to thrombosis related to elevated platelet counts and the continuation of these conditions in recurrent attacks may have a role on the development of FSGS.<span class="elsevierStyleSup">9</span> The emergence of FSGS has been reported average three to seven years after the diagnosis of PV.<span class="elsevierStyleSup">4-10 </span>In the case of the subject of our study, considering that FSGS has been diagnosed with PV six years later is consistent with the literature. The PV was thought to be the possible reason for FSGS. Additionally, FSGS may occur by occlusions due to the long term recurrent microvascular thrombosis and this also could disorder to glomerular hemodynamics.</p><p class="elsevierStylePara">Consequently, the co-existence of PV and FSGS seems to be a cause-effect relationship rather than a random combination. Further studies will be needed to demonstrate for a better understanding of this association.</p><p class="elsevierStylePara"> </p><p class="elsevierStylePara"><span class="elsevierStyleBold">Acknowledgement</span></p><p class="elsevierStylePara"> </p><p class="elsevierStylePara">None of the authors has a financial interest in any of the products or methods mentioned in this article.</p><p class="elsevierStylePara"> </p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflict of interest</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold"><br></br></span>The authors declare that there is no conflict of interest associated with this manuscript.</p><p class="elsevierStylePara"><a href="grande/11562_108_35467_en_t1.jpg" class="elsevierStyleCrossRefs"><img src="11562_108_35467_en_t1.jpg" alt="Haematological, chemistrical, serological and urinalysis findings of patient"></img></a></p><p class="elsevierStylePara">Table 1. Haematological, chemistrical, serological and urinalysis findings of patient</p>" "pdfFichero" => "P1-E544-S3783-A11562-EN.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig1" "etiqueta" => "Tab. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "11562_108_35467_en_t1.jpg" "Alto" => 821 "Ancho" => 1453 "Tamanyo" => 273814 ] ] "descripcion" => array:1 [ "en" => "Haematological, chemistrical, serological and urinalysis findings of patient" ] ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "1.\u{A0}\u{A0}Tefferi A. Polycythemia vera\u{A0}and essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 8 | 9 | 17 |
| 2024 October | 61 | 35 | 96 |
| 2024 September | 59 | 33 | 92 |
| 2024 August | 83 | 60 | 143 |
| 2024 July | 52 | 27 | 79 |
| 2024 June | 53 | 36 | 89 |
| 2024 May | 69 | 39 | 108 |
| 2024 April | 58 | 36 | 94 |
| 2024 March | 54 | 22 | 76 |
| 2024 February | 45 | 37 | 82 |
| 2024 January | 20 | 23 | 43 |
| 2023 December | 32 | 36 | 68 |
| 2023 November | 57 | 36 | 93 |
| 2023 October | 79 | 35 | 114 |
| 2023 September | 83 | 30 | 113 |
| 2023 August | 63 | 18 | 81 |
| 2023 July | 66 | 30 | 96 |
| 2023 June | 95 | 24 | 119 |
| 2023 May | 121 | 31 | 152 |
| 2023 April | 82 | 24 | 106 |
| 2023 March | 102 | 27 | 129 |
| 2023 February | 72 | 17 | 89 |
| 2023 January | 52 | 23 | 75 |
| 2022 December | 55 | 34 | 89 |
| 2022 November | 48 | 26 | 74 |
| 2022 October | 51 | 36 | 87 |
| 2022 September | 42 | 29 | 71 |
| 2022 August | 44 | 41 | 85 |
| 2022 July | 34 | 48 | 82 |
| 2022 June | 47 | 28 | 75 |
| 2022 May | 41 | 31 | 72 |
| 2022 April | 43 | 43 | 86 |
| 2022 March | 53 | 37 | 90 |
| 2022 February | 51 | 50 | 101 |
| 2022 January | 66 | 30 | 96 |
| 2021 December | 40 | 44 | 84 |
| 2021 November | 43 | 42 | 85 |
| 2021 October | 50 | 39 | 89 |
| 2021 September | 55 | 34 | 89 |
| 2021 August | 47 | 44 | 91 |
| 2021 July | 34 | 37 | 71 |
| 2021 June | 34 | 24 | 58 |
| 2021 May | 39 | 25 | 64 |
| 2021 April | 64 | 31 | 95 |
| 2021 March | 38 | 41 | 79 |
| 2021 February | 53 | 38 | 91 |
| 2021 January | 22 | 19 | 41 |
| 2020 December | 36 | 20 | 56 |
| 2020 November | 31 | 19 | 50 |
| 2020 October | 24 | 13 | 37 |
| 2020 September | 19 | 6 | 25 |
| 2020 August | 37 | 10 | 47 |
| 2020 July | 32 | 17 | 49 |
| 2020 June | 39 | 24 | 63 |
| 2020 May | 46 | 21 | 67 |
| 2020 April | 24 | 18 | 42 |
| 2020 March | 34 | 24 | 58 |
| 2020 February | 41 | 32 | 73 |
| 2020 January | 54 | 29 | 83 |
| 2019 December | 49 | 24 | 73 |
| 2019 November | 37 | 16 | 53 |
| 2019 October | 18 | 8 | 26 |
| 2019 September | 33 | 15 | 48 |
| 2019 August | 22 | 8 | 30 |
| 2019 July | 47 | 25 | 72 |
| 2019 June | 54 | 12 | 66 |
| 2019 May | 65 | 19 | 84 |
| 2019 April | 116 | 19 | 135 |
| 2019 March | 68 | 33 | 101 |
| 2019 February | 44 | 17 | 61 |
| 2019 January | 37 | 15 | 52 |
| 2018 December | 122 | 39 | 161 |
| 2018 November | 102 | 14 | 116 |
| 2018 October | 120 | 19 | 139 |
| 2018 September | 151 | 14 | 165 |
| 2018 August | 90 | 18 | 108 |
| 2018 July | 82 | 11 | 93 |
| 2018 June | 60 | 8 | 68 |
| 2018 May | 68 | 14 | 82 |
| 2018 April | 74 | 10 | 84 |
| 2018 March | 51 | 9 | 60 |
| 2018 February | 44 | 12 | 56 |
| 2018 January | 37 | 9 | 46 |
| 2017 December | 59 | 12 | 71 |
| 2017 November | 45 | 14 | 59 |
| 2017 October | 49 | 8 | 57 |
| 2017 September | 53 | 15 | 68 |
| 2017 August | 69 | 16 | 85 |
| 2017 July | 62 | 22 | 84 |
| 2017 June | 52 | 9 | 61 |
| 2017 May | 50 | 9 | 59 |
| 2017 April | 55 | 13 | 68 |
| 2017 March | 44 | 31 | 75 |
| 2017 February | 45 | 7 | 52 |
| 2017 January | 30 | 10 | 40 |
| 2016 December | 32 | 8 | 40 |
| 2016 November | 42 | 8 | 50 |
| 2016 October | 86 | 3 | 89 |
| 2016 September | 101 | 8 | 109 |
| 2016 August | 135 | 5 | 140 |
| 2016 July | 159 | 11 | 170 |
| 2016 June | 130 | 0 | 130 |
| 2016 May | 137 | 0 | 137 |
| 2016 April | 116 | 0 | 116 |
| 2016 March | 81 | 0 | 81 |
| 2016 February | 112 | 0 | 112 |
| 2016 January | 91 | 0 | 91 |
| 2015 December | 120 | 0 | 120 |
| 2015 November | 87 | 0 | 87 |
| 2015 October | 86 | 0 | 86 |
| 2015 September | 60 | 0 | 60 |
| 2015 August | 71 | 0 | 71 |
| 2015 July | 51 | 0 | 51 |
| 2015 June | 39 | 0 | 39 |
| 2015 May | 44 | 0 | 44 |
| 2015 April | 5 | 0 | 5 |
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