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      Anemia microcítica-hipocrómica: anemia ferropénica versus b talasemia menor Translated title: Anemia microcitica-hipocrômica: anemia ferropênica versus b talassemia menor Translated title: Microcytic and hypochromic anemia: iron deficiency anemia versus b thalassemia trait

      research-article
      Acta bioquímica clínica latinoamericana
      Federación Bioquímica de la Provincia de Buenos Aires
      Microcitosis, Deficiencia de hierro, Volumen corpuscular medio, Amplitud de la dispersión eritrocitaria, Hipocromía, Talasemia, Índices de microcitosis, Receptor de transferrina, Electroforesis de hemoglobina, Estrés oxidativo, Microcitose, Deficiência de ferro, Volume corpuscular médio, Amplitude da dispersão eritrocitária, Hipocromia, Talassemia, Índices de microcitose, Receptor de transferrina, Eletroforese de hemoglobina, Estresse oxidativo, Microcytosis, Iron deficiency, Mean corpuscular volume, blood cell distribution width, Hypochromia, Thalassemia, Microcytosis discriminant indices, Transferrin receptor, Hemoglobin electrophoresis, Oxidative stress

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          Abstract

          Las anemias microcíticas hipocrómicas (m-H) presentan VCM<80 fL y HCM<27 pg. Son producto de la baja biodisponibilidad del hierro (Fe), o del defecto de la síntesis de globinas o del HEMO. La más frecuente es la anemia por deficiencia de hierro (ADH), seguida por las talasemias y las anemias de procesos crónicos. Menos frecuentes son aquellas por defectos en el HEMO o por causas genéticas del metabolismo del Fe. El objetivo del trabajo es revisar, por medio de parámetros de distinta complejidad, diferencias entre ADH y b talasemia heterocigota (b-Tal-het), las m-H de mayor prevalencia en nuestro medio. Los recuentos de eritrocitos y reticulocitos, hemoglobina, ferremia, ferritina, saturación de la transferrina, HbA2, porcentaje de alteraciones morfológicas son menores en la ADH. El VCM, el HCM, la ADE, los índices de microcitosis, transferrina, y los receptores solubles de transferrina son menores en b-Tal-het. El estrés oxidativo está aumentado en ambas patologías. En el análisis de estos parámetros se discute el grado de deficiencia de Fe y/o la mutación de b-Tal-het. Se aplica un algoritmo para m-H a partir del Fe sérico. Una vez descartadas las m-H más comunes, se debe investigar a-Tal-het, la cual se considera la causa de la mayoría de m-H inexplicadas.

          Translated abstract

          As anemias microcíticas hipocrômicas (m-H) apresentam VCM<80 fL e HCM<27 pg. São produto da baixa biodisponibilidade do ferro (Fe), ou do defeito da síntese de globinas ou do HEMO. A mais frequente é a anemia por deficiência de ferro (ADH), seguida pelas talassemias e as anemias de processos crônicos. Menos frequentes são aquelas por defeitos no HEMO ou por causas genéticas do metabolismo do Fe. O objetivo do trabalho é revisar, através de parâmetros de diversa complexidade, diferenças entre ADH e b talassemia heterocigota (b-Tal-het), as m-H de maior prevalência no nosso meio. As contagens de eritrócitos e reticulócitos, hemoglobina, ferremia, ferritina, saturação da transferrina, HbA2, percentagem de alterações morfológicas são menores em ADH. O VCM, o HCM, a ADE, os índices de microcitose, transferrina, receptores solúveis de transferrina são menores em b-Tal-het. O estresse oxidativo está aumentado em ambas as patologias. Na análise destes parâmetros é discutido o grau de deficiência de Fe e/ou a mutação de b-Tal-het. Aplica-se um algoritmo para m-H a partir do Fe sérico. Depois de serem descartadas as m-H mais comuns, deve investigar-se a-Tal-het, a qual é considerada a causa da maior parte de m-H inexplicadas.

          Translated abstract

          Microcytic hypochromic anemia (m-H) presents MCV<80 fL and MCH<27 pg. m-H can result from iron availability, defects in globin or HEMO synthesis. The most frequent m-H is iron deficiency anemia (IDA), followed by thalassemias and anemia chronic disease. Rare m-H are a consequence of HEME defects or iron metabolism genetic defects. The aim of this study is to review the differential diagnosis between IDA and b thalassemia trait (b thal trait), the most frequent in our environment. Results of laboratory tests are analysed. Erythrocytes, hemoglobin, reticulocytes, iron, ferritin, transferrin saturation, HbA2 and percentage of morphologic changes are lower in IDA compared with b Thal trait. MCV, MCH, RDW, microcytic index, transferrin and soluble transferrin receptor are higher in IDA compared with b Thal trait. Oxidative stress is increased in the two forms of microcytoses. Degree iron deficiency in IDA and b Thal trait mutation must be considered in the analysis of the parameters. A flowchart is proposed to evaluate m-H stemming from serum iron value. After excluding the most frequent causes of microcytic anemia, a thalassemia trait must be considered.

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          The complex interplay of iron metabolism, reactive oxygen species, and reactive nitrogen species: insights into the potential of various iron therapies to induce oxidative and nitrosative stress.

          Production of minute concentrations of superoxide (O2(*-)) and nitrogen monoxide (nitric oxide, NO*) plays important roles in several aspects of cellular signaling and metabolic regulation. However, in an inflammatory environment, the concentrations of these radicals can drastically increase and the antioxidant defenses may become overwhelmed. Thus, biological damage may occur owing to redox imbalance-a condition called oxidative and/or nitrosative stress. A complex interplay exists between iron metabolism, O2(*-), hydrogen peroxide (H2O2), and NO*. Iron is involved in both the formation and the scavenging of these species. Iron deficiency (anemia) (ID(A)) is associated with oxidative stress, but its role in the induction of nitrosative stress is largely unclear. Moreover, oral as well as intravenous (iv) iron preparations used for the treatment of ID(A) may also induce oxidative and/or nitrosative stress. Oral administration of ferrous salts may lead to high transferrin saturation levels and, thus, formation of non-transferrin-bound iron, a potentially toxic form of iron with a propensity to induce oxidative stress. One of the factors that determine the likelihood of oxidative and nitrosative stress induced upon administration of an iv iron complex is the amount of labile (or weakly-bound) iron present in the complex. Stable dextran-based iron complexes used for iv therapy, although they contain only negligible amounts of labile iron, can induce oxidative and/or nitrosative stress through so far unknown mechanisms. In this review, after summarizing the main features of iron metabolism and its complex interplay with O2(*-), H2O2, NO*, and other more reactive compounds derived from these species, the potential of various iron therapies to induce oxidative and nitrosative stress is discussed and possible underlying mechanisms are proposed. Understanding the mechanisms, by which various iron formulations may induce oxidative and nitrosative stress, will help us develop better tolerated and more efficient therapies for various dysfunctions of iron metabolism. © 2013 Elsevier Inc. All rights reserved.
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            Hemojuvelin is essential for dietary iron sensing, and its mutation leads to severe iron overload.

            Iron homeostasis plays a critical role in many physiological processes, notably synthesis of heme proteins. Dietary iron sensing and inflammation converge in the control of iron absorption and retention by regulating the expression of hepcidin, a regulator of the iron exporter ferroportin. Human mutations in the glycosylphosphatidylinositol-anchored protein hemojuvelin (HJV; also known as RGMc and HFE2) cause juvenile hemochromatosis, a severe iron overload disease, but the way in which HJV intersects with the iron regulatory network has been unclear. Here we show that, within the liver, mouse Hjv is selectively expressed by periportal hepatocytes and also that Hjv-mutant mice exhibit iron overload as well as a dramatic decrease in hepcidin expression. Our findings define a key role for Hjv in dietary iron sensing and also reveal that cytokine-induced inflammation regulates hepcidin expression through an Hjv-independent pathway.
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              Improved classification of anemias by MCV and RDW.

              New automated blood cell analyzers provide an index of red cell volume distribution width (RDW) or heterogeneity and a histogram display of red cell volume distribution. We have developed a classification of red cell disorders, based on mean corpuscular volume (MCV) or red cell size, heterogeneity, and histograms, to guide diagnosis from the peripheral blood analysis. The distinction of iron deficiency anemia from heterozygous thalassemia or the anemia of chronic disease and the detection of early iron and folate deficiency is improved. Red cell volume distribution histograms identify red cell fragmentation or agglutination, dimorphic populations, and artifactual counting of lymphocytes as red cells. We recommend the use of these new variables in the initial classification of anemia by the practicing physician.
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                Author and article information

                Contributors
                Role: ND
                Journal
                abcl
                Acta bioquímica clínica latinoamericana
                Acta bioquím. clín. latinoam.
                Federación Bioquímica de la Provincia de Buenos Aires (La Plata, Buenos Aires, Argentina )
                0325-2957
                1851-6114
                September 2017
                : 51
                : 3
                : 291-305
                Affiliations
                [01] Ciudad Autónoma de Buenos Aires orgnameSociedad Argentina de Hematología orgdiv1Subcomisión de Eritropatías Argentina
                Article
                S0325-29572017000300004
                af1f8555-84f8-4d2d-b846-f33e7514a08e

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 18 July 2016
                : 20 July 2017
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 91, Pages: 15
                Product

                SciELO Argentina


                Talassemia,Receptor de transferrina,Índices de microcitosis,Talasemia,Hipocromía,Amplitud de la dispersión eritrocitaria,Volumen corpuscular medio,Deficiencia de hierro,Microcitosis,Oxidative stress,Hemoglobin electrophoresis,Transferrin receptor,Microcytosis discriminant indices,Thalassemia,Hypochromia,blood cell distribution width,Mean corpuscular volume,Iron deficiency,Microcytosis,Estresse oxidativo,Eletroforese de hemoglobina,Índices de microcitose,Estrés oxidativo,Electroforesis de hemoglobina,Hipocromia,Amplitude da dispersão eritrocitária,Volume corpuscular médio,Deficiência de ferro,Microcitose

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