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- W2080276626 abstract "Nephrotic syndrome (NS) is one of the chronic kidney diseases in long-term survivors of allogeneic hematopoietic stem cell transplantation (HSCT) and it has been an increasingly reported complication. Recently, Colombo et al. suggested that careful monitoring of renal function in myeloablative HSCT recipients with chronic graft-versus-host disease (cGVHD) may document the incidence of NS (1). Six cases of NS together with extensive cGVHD were reported in that retrospective study. The cumulative incidence of NS was reported to be 8% at day +1681. Additionally, Srinivasan and co-workers reported NS with a cumulative incidence of 6.1% in nonmyeloablative HSCT recipients from a related human leukocyte antigen (HLA)-compatible donor (2). In contrast to previous reports, three of seven NS cases were not associated with cGVHD. It was usually observed that NS is not improved with the reinitiation of aggressive immunosuppression and it results in progressive renal failure necessitating dialysis. We would like to present our experience regarding the incidence and outcome of NS in patients who underwent allogeneic HSCT. We analyzed 70 adult patients with a minimum follow-up of 100 days who underwent myeloablative allogeneic HSCT. Thirty patients were identified as having cGVHD and one patient NS without evidence of cGVHD. This patient was a-34-year-old woman. She received unmanipulated blood HSCT for acute myeloid leukemia from her HLA full-matched brother. The conditioning was with busulfan and cyclophosphamide. Cyclosporine and short course methotrexate were given as GVHD prophylaxis. The major early posttransplant complication was severe hemorrhagic cystitis. She did not experience GVHD. After 4 months of GVHD prophylaxis cessation, pedal edema was detected during routine follow-up. The serum albumin level was 1.9 g/dL with urinary protein excretion rate 6 g/day. Creatinine clearance was within normal limits, but serum lipid levels were increased. She was in hematological remission and DNA analysis revealed complete chimerism. Renal biopsy showed normal architecture of the glomerulus with normal thickness of the basement membrane. Immunofluorescence microscopy revealed a characteristic pattern of membraneous nephropathy (MN); thin subepithelial granular deposits of immunoglobulin G and C3 along the glomerular basement membrane. Hydroxy-methylglutaryl coenzyme A reductase inhibitor and angiotensin-converting enzyme inhibitor treatment were initiated to reduce serum lipid levels and urinary protein excretion, respectively. Prednisone was administered as immunosuppressant. The patient de-veloped bilateral avascular necrosis of the femoral head and refused further therapies. Steroid treatment was withdrawn. Eight years after the diagnosis of NS, the disease did not progress. The proteinuria persisted but decreased to 0.5–1.5 g/day and the serum albumin level rose to 2.8 g/dL. She has still denied any symptom signifying an underlying disorder or GVHD. As conclusion, the risk of NS as a postHSCT MN should be considered and renal functions should be carefully monitored. The evolution of NS does not usually parallel in the clinical course of cGVHD (2–4). MN—the most common finding on renal biopsy—may be underrecognized until it is clinically symptomatic (5). In all series reported to date, the clinicopathologic features of NS demonstrated that there is an immune-mediated process (1–6). We consider that there may be an association between cGVHD and postHSCT MN, but the treatment should be individualized, especially in cases without manifestations of cGVHD. Sevgi Kalayoglu-Besisik Department of Internal Medicine Division of Hematology Istanbul University Istanbul School of Medicine Istanbul, Turkey Alper Yurci Department of Internal Medicine Division of Gastroenterology Erciyes University School of Medicine Kayseri, Turkey Halil Yazici Department of Internal Medicine Division of Nephrology Istanbul University Istanbul School of Medicine Istanbul, Turkey Ipek Yonal Department of Internal Medicine Istanbul University Istanbul School of Medicine Istanbul, Turkey Deniz Sargin Department of Internal Medicine Division of Hematology Istanbul University Istanbul School of Medicine Istanbul, Turkey" @default.
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- W2080276626 date "2007-05-27" @default.
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- W2080276626 title "Long-Term Outcome of Nephrotic Syndrome in an Allogeneic Hematopoietic Stem Cell Recipient Without Typical Features of Graft Versus Host Disease" @default.
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- W2080276626 doi "https://doi.org/10.1097/01.tp.0000263333.65558.8b" @default.
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