The Experts below are selected from a list of 37911 Experts worldwide ranked by ideXlab platform
R Donald Harvey - One of the best experts on this subject based on the ideXlab platform.
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Myelodysplastic syndromes and the role of Iron Overload
American Journal of Health-system Pharmacy, 2010Co-Authors: R Donald HarveyAbstract:Purpose The epidemiology of myelodysplastic syndromes (MDS) and Iron Overload, recent clinical findings that highlight the importance of actively managing Iron Overload, and recommendations for initiating and maintaining Iron chelation therapy (ICT) are summarized. Summary MDS are a variety of hematological disorders with differing time courses. Disease morbidities are primarily due to cytopenias and evolution to acute myeloid leukemia. Iron Overload is a serious complication in patients with MDS due to the long-term use of red blood cell transfusions in patients with symptomatic anemia. Clinical consequences of Iron Overload include end-organ damage and dysfunction, an increased frequency of transplant-related complications, and reduced survival rates. To prevent these complications, recommendations for initiating and maintaining ICT should be followed by clinicians caring for patients with MDS and Iron Overload. Conclusion As current therapeutic options for patients with MDS do not always reduce the transfusion burden, many patients will still need long-term transfusion therapy. Strategies for the management of Iron Overload in MDS should be considered early in the disease course and in appropriate patients in order to prevent negative clinical outcomes associated with excessive Iron accumulation.
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Myelodysplastic syndromes and the role of Iron Overload.
American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists, 2010Co-Authors: R Donald HarveyAbstract:The epidemiology of myelodysplastic syndromes (MDS) and Iron Overload, recent clinical findings that highlight the importance of actively managing Iron Overload, and recommendations for initiating and maintaining Iron chelation therapy (ICT) are summarized. MDS are a variety of hematological disorders with differing time courses. Disease morbidities are primarily due to cytopenias and evolution to acute myeloid leukemia. Iron Overload is a serious complication in patients with MDS due to the long-term use of red blood cell transfusions in patients with symptomatic anemia. Clinical consequences of Iron Overload include end-organ damage and dysfunction, an increased frequency of transplant-related complications, and reduced survival rates. To prevent these complications, recommendations for initiating and maintaining ICT should be followed by clinicians caring for patients with MDS and Iron Overload. As current therapeutic options for patients with MDS do not always reduce the transfusion burden, many patients will still need long-term transfusion therapy. Strategies for the management of Iron Overload in MDS should be considered early in the disease course and in appropriate patients in order to prevent negative clinical outcomes associated with excessive Iron accumulation.
Vinod Pullarkat - One of the best experts on this subject based on the ideXlab platform.
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Iron Overload in patients undergoing hematopoietic stem cell transplantation.
Advances in hematology, 2010Co-Authors: Vinod PullarkatAbstract:Recipients of hematopoietic stem cell transplantation (HSCT) frequently have Iron Overload resulting from chronic transfusion therapy for anemia. In some cases, for example, in patients with myelodysplastic syndromes and thalassemia, this can be further exacerbated by increased absorption of Iron from the gut as a result of ineffective erythropoiesis. Accumulating evidence has established the negative impact of elevated pretransplantation serum ferritin, a surrogate marker of Iron Overload, on overall survival and nonrelapse mortality after HSCT. Complications of HSCT associated with Iron Overload include increased bacterial and fungal infections as well as sinusoidal obstruction syndrome and possibly other regimen-related toxicities. Based on current evidence, particular attention should be paid to prevention and management of Iron Overload in allogeneic HSCT candidates, especially in patients with thalassemia and myelodysplastic syndromes. The pathophysiology of Iron Overload in the HSCT patient and optimum strategies to deal with Iron Overload during and after HSCT require further study.
Pierre Brissot - One of the best experts on this subject based on the ideXlab platform.
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Pathophysiology and classification of Iron Overload diseases; update 2018
Transfusion Clinique et Biologique, 2019Co-Authors: Pierre Brissot, Marie-bérengère Troadec, Olivier Loréal, Eolia BrissotAbstract:Iron Overload pathophysiology has benefited from significant advances in the knowledge of Iron metabolism and in molecular genetics. As a consequence, Iron Overload nosology has been revisited. The hematologist may be confronted to a number of Iron Overload syndromes, from genetic or acquired origin. Hemochromatoses, mostly but not exclusively related to the HFE gene, correspond to systemic Iron Overload of genetic origin in which Iron excess is the consequence of hepcidin deficiency, hepcidin being the hormone regulating negatively plasma Iron. Iron excess develops following hypersideremia and the formation of non-transferrin-bound Iron, which targets preferentially parenchymal cells (hepatocytes). The ferroportin disease has a totally different Iron Overload mechanism consisting of defective egress of cellular Iron into the plasma, Iron deposition taking place mostly within the macrophages (spleen). Hereditary aceruloplasminemia is peculiar since systemic Iron Overload involves the brain. Two main types of acquired Iron Overload can be seen by the hematologist, one related to dyserythropoiesis (involving hypohepcidinemia ), the other related to multiple transfusions (thalassemias, myelodysplasia, hematopoietic stem cell transplantation). Congenital sideroblastic anemias, either monosyndromic (anemia) or polysyndromic (anemia plus extra-hematological syndromes), develop both compartimental Iron excess within the erythroblast mitochondria, and systemic Iron Overload (through dyserythropoiesis and/or transfusions).
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Iron Overload: Diagnosis, Complications, and Management
Nonmalignant Hematology, 2016Co-Authors: Pierre BrissotAbstract:International audienceChronic Iron Overload corresponds to a variety of genetic and acquired diseases. Genetic Iron Overload encompasses HFE and non-HFE-related hemochromatoses. Acquired Iron Overload is mainly due to multiple transfusions and to dyserythropoiesis. The damaging effect of Iron excess concerns numerous organs and can therefore lead to miscellaneous clinical features, related especially to the liver, heart, pancreas, and rheumatological complications. Those complications alter not only the quality of life but also life expectancy. The diagnostic approach has become essentially noninvasive. It rests – beside clinical examination – on serum Iron parameters (especially ferritin and transferrin saturation), on Iron MRI, and, whenever indicated, on genetic testing. The treatment, in hepcidin deficiency-related Iron Overload, remains based on venesection therapy but should resort in the future to hepcidin supplementation. Posttransfusional Iron Overload has greatly benefited from the design of oral Iron chelators
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Diagnosis and current treatments for primary Iron Overload.
American journal of hematology, 2007Co-Authors: Pierre BrissotAbstract:Primary Iron Overload encompasses a variety of genetic Iron Overload syndromes, dominated in frequency by HFE-related, or Type 1 hemochromatosis, for which French diagnostic and therapeutic guidelines have been recently proposed. Differential diagnosis of Type 1 hemochromatosis can be made from both clinical data and genetic studies. Venesection therapy and family screening remain the basis for the curative and preventive management of most genetic Iron Overload diseases.
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insulin resistance associated hepatic Iron Overload
Gastroenterology, 1999Co-Authors: Michelhenry Mendler, Dominique Guyader, Pierre Brissot, Bruno Turlin, Romain Moirand, Annemarie Jouanolle, Thierry Sapey, Jeanyves Le Gall, Veronique David, Yves DeugnierAbstract:Abstract Background & Aims: Hepatic Iron Overload has been reported in various metabolic conditions, including the insulin-resistance syndrome (IRS) and nonalcoholic steatohepatitis (NASH). The aim of this study was to show that such hepatic Iron Overload is part of a unique and unrecognized entity. Methods: A total of 161 non–C282Y-homozygous patients with unexplained hepatic Iron Overload were included. We determined the age; sex; presence of IRS (1 or more of the following: body mass index of >25, diabetes, or hyperlipidemia); serum Iron tests and liver Iron concentration (LIC; reference value, HFE mutations; and liver histological status. Results: Patients were predominantly male and middle-aged. Most (94%) had IRS. Transferrin saturation was increased in 35% (median, 42%; range, 13%–94%). LIC ranged from 38 to 332 μmol/g (median, 90 μmol/g), and LIC/age ratio ranged from 0.5 to 4.8 (median, 1.8). Allelic frequencies of both HFE mutations were significantly increased compared with values in normal controls (C282Y, 20% vs. 9%; H63D, 30% vs. 17%), only because of a higher prevalence of compound heterozygotes. Patients with no HFE mutations had similar degrees of Iron Overload as those with other genotypes, except for compound heterozygotes, who had slightly more Iron burden. Steatosis was present in 25% of patients and NASH in 27%. Portal fibrosis (grades 0–3) was present in 62% of patients (grade 2 or 3 in 12%) in association with steatosis, inflammation, and increased age. Sex ratio, IRS, transferrin saturation, and LIC did not vary with liver damage. Serum ferritin concentration, liver function test results, and fibrosis grade were more elevated in patients with steatosis and NASH than in others, but LIC and allelic frequencies of HFE mutations were similar. Conclusions: This study shows that patients with unexplained hepatic Iron Overload are characterized by a mild to moderate Iron burden and the nearly constant association of an IRS irrespective of liver damage. GASTROENTEROLOGY 1999;117:1155-1163
Gavin Y. Oudit - One of the best experts on this subject based on the ideXlab platform.
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Iron-Overload Cardiomyopathy: Pathophysiology, Diagnosis, and Treatment
Journal of cardiac failure, 2010Co-Authors: Colm J. Murphy, Gavin Y. OuditAbstract:Abstract Background The prevalence of primary (hereditary) hemochromatosis and secondary Iron Overload (hemosiderosis) is reaching epidemic levels worldwide. Iron-Overload leads to excessive Iron deposition in a wide variety of tissues, including the heart and endocrine tissues. Methods and Results Iron-Overload cardiomyopathy is the primary determinant of survival in patients with secondary Iron Overload, while also being a leading cause of morbidity and mortality in patients with primary hemochromatosis. Iron-induced cardiovascular injury also occurs in acute Iron toxicosis (Iron poisoning), myocardial ischemia-reperfusion injury, cardiomyopathy associated with Friedreich ataxia, and vascular dysfunction. The mainstay therapies for Iron Overload associated with primary hemochromatosis and secondary Iron Overload is phlebotomy and Iron chelation therapy, respectively. L-type Ca 2+ channels provide a high-capacity pathway for ferrous (Fe 2+ ) uptake into cardiomyocytes in Iron-Overload conditions; calcium channel blockers may represent a new therapeutic tool to reduce the toxic effects of excess Iron. Conclusions Iron-Overload cardiomyopathy is a an important and potentially reversible cause of heart failure at an international scale and involves diastolic dysfunction, increased susceptibility to arrhythmias and a late-stage dilated cardiomyopathy. The early diagnosis of Iron-Overload cardiomyopathy is critical since the cardiac dysfunction is reversible if effective therapy is introduced before the onset of overt heart failure.
Robert J Soiffer - One of the best experts on this subject based on the ideXlab platform.
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does Iron Overload really matter in stem cell transplantation
American Journal of Hematology, 2012Co-Authors: Philippe Armand, Mariemichele Sainvil, Haesook T Kim, Joanna Rhodes, Corey Cutler, John Koreth, Edwin P Alyea, Ellis J Neufeld, Raymond Y Kwong, Robert J SoifferAbstract:A growing body of evidence suggests that Iron Overload is associated with inferior outcomes after myeloablative allogeneic hematopoietic stem cell transplantation (HSCT). However, all of those studies used surrogate markers of Iron Overload, especially serum ferritin, and most had a retrospective design. We conducted a prospective observational study in patients with MDS or acute leukemia undergoing myeloablative HSCT. Forty-five patients who were followed for over 1 year, with serial measurements of serum Iron parameters, as well as liver and cardiac MRI. There was no significant increase in ferritin, liver or cardiac Iron content in the 12 months following HSCT. While serum ferritin still appeared to have prognostic significance, as previously reported, pre-HSCT Iron Overload (as reflected in liver Iron content) was not associated with increased mortality, relapse, or GVHD. These results raise the possibility that the adverse prognostic impact of pre-HSCT hyperferritinemia may be related to factors independent of Iron Overload.
