Cases reported "Polycythemia"

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1/26. adult Wilms' tumor associated with polycythemia--a case report.

    Both Wilms' tumor (nephroblastoma) in adults and polycythemia in Wilms' tumor are rare. Herein we report an extremely rare case of adult Wilms' tumor associated with polycythemia. A 41-year-old female was incidentally found to have right renal mass by abdominal sonography in a routine health examination. Laboratory examination revealed polycythemia (hemoglobin 20.2 g/dL). Although physical examination was unremarkable, CT scan revealed an homogeneous mass at the middle pole of right kidney, and chest x-ray revealed no metastatic lesions. Right radical nephrectomy was performed smoothly. Grossly, the tumor of 5 x 4.5 x 4.5 cm in size was well circumscribed, and had no vascular structure or collecting system involvement. Microscopic features were consistent with adult nephroblastoma. The post-operative course was uneventful. At follow-up, the patient was well, showed no evidence of recurrence and her hemoglobin level had returned to normal (hemoglobin 14.5 g/dl). We suggest that the relationship between polycythemia and Wilms' tumor should be carefully evaluated before surgical treatment.
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2/26. EPO-producing gastric carcinoma in a hemodialysis patient.

    A case of erythrocytosis caused by gastric cancer that produced erythropoietin is described. To the authors' knowledge, no case of erythropoietin-producing gastric cancer has been reported until now. A 73-year-old man with a 4-year history of maintenance hemodialysis for diabetic nephropathy required phlebotomy. serum erythropoietin level was 181 mU/mL (181 IU/L). gastroscopy results showed rough mucosa with hemorrhaging caused by gastric cancer. The patient underwent distal gastrectomy, and serum erythropoietin level decreased to 27.1 mU/mL (27.1 IU/L) by postoperative day 8. Existence of erythropoietin in the tumor tissue was confirmed immunohistochemically. The presence of severe acquired cystic disease of the kidney, renal cell carcinoma, and other malignant tumors should be investigated in hemodialysis patients displaying erythrocytosis.
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3/26. Erythrocytosis in renal allograft recipients. Benefit of staggered venous erythropoietin measurements.

    Two adult renal allograft recipients experienced erythrocytosis--one in acute form--within 3 months of grafting. Involvement of well functioning transplanted kidneys was unlikely whereas staggered erythropoietin measurements detected a high gradient in front of venous remnant kidneys. Because of these results bilateral nephrectomy was performed, which cured polycythaemia. Multiple events leading to polycythaemia after renal transplantation are reviewed and diagnosis and therapeutic schedules are proposed.
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4/26. Post-renal transplant erythrocytosis in a child.

    We report the case of a 7-year-old boy who developed severe erythrocytosis 4 months after successful kidney transplantation, with a well-functioning graft. When the haematocrit rose above 60%, phlebotomy had to be performed once to twice a week in order to keep the haematocrit below 50%. A 3-month course of theophylline therapy did not influence the erythrocytosis significantly. There were 5 further patients with erythrocytosis out of 186 children who had undergone kidney transplantation at our centre.
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5/26. A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis.

    The number of red blood cells is normally tightly regulated by a classic homeostatic mechanism based on oxygen sensing in the kidney. Decreased oxygen delivery resulting from anemia induces the production of erythropoietin, which increases red cell production and hence oxygen delivery. Investigations of erythropoietin regulation identified the transcription factor hypoxia-inducible factor (HIF). HIF is now recognized as being a key regulator of genes that function in a comprehensive range of processes besides erythropoiesis, including energy metabolism and angiogenesis. HIF itself is regulated through the alpha-subunit, which is hydroxylated in the presence of oxygen by a family of three prolyl hydroxylase domain proteins (PHDs)/HIF prolyl hydroxylases/egg-laying-defective nine enzymes. hydroxylation allows capture by the von Hippel-Lindau tumor suppressor gene product, ubiquitination, and destruction by the proteasome. Here we describe an inherited mutation in a mammalian PHD enzyme. We show that this mutation in PHD2 results in a marked decrease in enzyme activity and is associated with familial erythrocytosis, identifying a previously unrecognized cause of this condition. Our findings indicate that PHD2 is critical for normal regulation of HIF in humans.
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6/26. A rare case of renal oncocytoma associated with erythrocytosis: case report.

    BACKGROUND: Oncocytomas are benign tumors of the kidney that are usually diagnosed postoperatively due to differential diagnostic problems from renal cell carcinoma. Although the latter are neoplasms that have been associated with erythrocytosis in 3.5% of cases, there are no reports in the literature about a similar occurrence in oncocytomas. CASE PRESENTATION: In this case report we present a unique case of a right lower pole oncocytoma associated with erythrocytosis. Erythrocytosis subsided after partial nephrectomy. CONCLUSION: Erythrocytosis can sometimes occur in association with renal oncocytomas.
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7/26. Hepatic hemangioblastoma. An unusual presentation in a patient with von hippel-lindau disease.

    We report a case of multiple capillary hemangioblastomas of the liver occurring in a patient with von hippel-lindau disease and a history of previous cerebellar and spinal hemangioblastomas. Although rare examples of this tumor have previously been recorded in the pancreas, kidney, and urinary bladder, this appears to be the first recorded case with hepatic involvement. The histology and immunohistochemical appearance of this neoplasm are identical with those of the cerebellar tumor. We believe it represents a separate primary neoplasm rather than metastatic disease.
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8/26. Erythrocytosis associated with renal artery thrombosis in a patient with polycystic kidney disease on hemodialysis.

    We report a case of erythrocytosis in a patient with end-stage renal failure on chronic hemodialysis. The patient with polycystic kidney disease had an average Hb level of 10 g/dl while on hemodialysis for 3 years. He developed erythrocytosis (Hb 17.6 g/dl) following a cadaveric renal transplantation. No signs suggesting polycythemia vera were found. Nonrenal causes of secondary erythrocytosis such as anoxia, hemoglobinopathies or tumors were excluded. angiography showed renal artery occlusion of the native kidney. serum erythropoietin level was 85 U/l (normal 52 /- 31 U/l) as measured by 3H-thymidine uptake. It is suggested that ischemia caused by the renal artery thrombosis stimulated the erythropoietin production in the native polycystic kidney.
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ranking = 77.476103615407
keywords = kidney disease, kidney
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9/26. erythropoietin-induced secondary polycythemia in a patient with a renal cell carcinoma. A case report.

    This study provides clear documentation of in vivo biogenesis of erythropoietin (Epo) by a human renal carcinoma. A middle-aged woman with a clear cell renal carcinoma of the left kidney developed severe polycythemia. This polycythemia was accompanied by markedly elevated levels of immunoreactive erythropoietin both in the peripheral venous blood, and in blood derived from the left renal vein during nephrectomy. Exstirpation of the non-invasive renal carcinoma was followed by complete restoration of both hematocrit and erythropoietin plasma concentration to normal levels. The fall in plasma erythropietin concentration immediately after nephrectomy (T/2 less than or equal to 3 hours) was probably a valid representation of plasma erythropoietin metabolism in this patient. Direct evidence of erythropoietin production in individual renal carcinoma cells was provided by immunoperoxidase studies demonstrating focal cytoplasmatic accumulation of immunoreactive erythropoietin in the tumor cells.
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10/26. Polycythemia after kidney transplantation: influence of the native kidneys on the production of hemoglobin.

    3 patients with renal transplantation who developed polycythemia presented normalization of the hemoglobin levels immediately after nephrectomy of the native kidneys. This observation induced the authors to study the role of the native kidneys in the genesis of polycythemia in recipients of renal allografts. Comparison was made among 32 patients submitted to renal transplantation, with maintenance of native kidneys (group I) and among 31 under the same conditions, but without the native kidneys (group II). Both groups were comparable according to age, sex, rejection crisis incidence and immunosuppressive therapy. It was observed that the hemoglobin levels of group I were significantly higher (p less than 0.05 to p less than 0.005) than those observed in group II, from the 3rd to the 30th posttransplantation month, becoming comparable from the 36th to the 54th months. The hemoglobin production, measured by the kinetics of labeled iron (59Fe), was higher in patients of group I. The authors concluded that the native kidneys are responsible for the observed polycythemia after a kidney transplantation.
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