For this purpose, new tools are available today. Association between bone mineral density and ... Serum Transferrin Receptors in Children with Hypochromic ... In this study, we have investigated α4β1, α5β1 integrins and CD71 expression on erythroblasts, together with serum soluble transferrin receptor (sTfR) levels in splenectomized, non-transfusion dependent patients with β-thalassemia intermedia. Must rule out other causes. R'zik S, Beguin Y (2001) Serum soluble transferrin receptor transferrin receptor as a marker of erythropoiesis suppression in concentration is an accurate estimate of the mass of tissue patients on chronic transfusion. The diagnosis of isolated IDA, is an easy task but if paralleled with inflammation and β-thalassemia trait (TT), it becomes difficult endeavor in some instance4-6. High-fluorescence reticulocyte counts and soluble transferrin receptor levels were determined in iron-deficiency an … Some inherited blood disorders can also influence ferritin and soluble transferrin receptor (sTfR) concentrations (12, 16- 20) as a result of high RBC turnover, increased or ineffective erythropoiesis, and hemolytic anemia . The transferrin receptor is an integral membrane glycoprotein having a molecular weight of 190 kilodaltons (kD). Soluble transferrin receptors are proteins found in blood that can be elevated with iron deficiency. Together they form a unique fingerprint. Link to publication in Scopus. Soluble Transferrin Receptors in the Diagnosis of Iron Deficiency Anemia, Ss Thalassemia Minor and Ss Thalassemia Minor with Concomitant Iron Deficiency Anemia (Clinical Report) Pakistan Journal of Medical Sciences 2011, March 31, 27, 1 stfr concentrations were significantly higher in both homozygotes (mean 3.1 mg/l, range 2.8-3.4) and heterozygotes … Serum transferrin receptor (sTfR) is the most reliable method for assessment of body iron. Besides, transferrin receptor (CD71) is the main portal for intracellular iron uptake . The most important clinical use of the sTfR determination is in the differential diagnosis between iron deficiency anaemia and the anaemia of chronic . The TDT patients (β-thalassemia major and severe forms of HbE/β-thalassemia) are those who commonly present in early childhood with severe anemia and require lifelong transfusion therapy for survival. It is then internalized through endosomes and the iron released into the cytoplasm. Punnonen K, Suominen P, Kuusinen A, Kuiper-Kramer E. Clinical use of soluble transferrin receptor. | Find, read and cite all the research you . We measured plasma levels of soluble transferrin receptor (sTfR) and leptin by enzyme-linked immunosorbent assay (ELISA) in 40 β-thalassemia patients (21 transfusion dependent and 19 not transfused or sporadically transfused), in 20 β-thalassemia carriers, and . stfr values, a reliable quantitative assessment of the erythropoietic activity, were lowest … 10.1002/fsn3.1780 [ CrossRef ] [ Google Scholar ] The soluble transferrin receptor (sTfR) immunoassay should not be used for the routine clinical evaluation of patients for iron status when ferritin immunoassay (FERR / Ferritin, Serum) would be appropriate, such as in the absence of confounding pathologies (inflammation, infection, chronic disease, or malignancy). The number of sTfR reflects the cellular requirements for iron, and varies with the function and the morphological development of each cell type. 2020; 8 :4601-4610. A second important conclusion of our trial was that sirolimus influences erythropoiesis and reduces biochemical markers associated to ineffective erythropoiesis (I.E.) spherocytosis, sickle cell anemia, thalassemia major or intermedia, megaloblastic . 10.1002/ajh.20804. Food Sci Nutr . Soluble transferrin receptor (sTfR) is inversely related to iron levels in blood. We investigated whether the soluble transferrin receptor (TfR) level was related to the degree of iron overload in 20 patients with thalassaemia intermedia, six patients with congenital dyserythropoietic anaemia type II (CDA II) and four patients with X‐linked . The soluble transferrin receptor (sTfR) immunoassay should not be used for the routine clinical evaluation of patients for iron status when ferritin immunoassay (FERR / Ferritin, Serum) would be appropriate, such as in the absence of confounding pathologies (inflammation, infection, chronic disease, or malignancy). Beta-thalassemia minor, also called carrier or trait, is the heterozygous state that is usually asymptomatic with mild anemia . However, . Diabetes Care 23(9):1384-8, 2000. The soluble transferrin receptor (sTfR) immunoassay should not be used for the routine clinical evaluation of patients for iron status when ferritin immunoassay (FERR / Ferritin, Serum) would be appropriate, such as in the absence of confounding pathologies (inflammation, infection, chronic disease, or malignancy). For example, the life span of sickled RBCs is only ∼17 d compared with ∼90-120 d for healthy RBCs . Keywords Transferrin receptor (TfR), Soluble transferrin receptor (sTfR), Soluble transferrin receptor-log ferritin index (sTfR-F index), Anemia, Iron defi- ciency, Thalassemia J Nutr. To rule out the cause of hypochromic microcytic anaemia is a diagnostic difficulty. Dear Sir, We previously found that the soluble transferrin receptor (sTfR) which fully reflects bone marrow erythropoietic activity, had an attractive diagnostic accuracy in predicting the risk of extramedullary haematopoiesis not only in patients with thalassemia intermedia 1, recently redefined non-transfusion-dependent thalassaemia (NTDT), but also in those with thalassaemia major 2. Aberrant neutrophil function was found in all thalassemia patients. Purpose of this study was to evaluate the role of soluble transferrin receptors in the differentiation of iron deficiency anemia from ss thalassemia minor. 91, no. The groups were compared in terms of levels of erythropoiesis biomarkers [growth differentiation factor 15 (GDF15), erythropoietin (EPO) and soluble transferrin receptor (sTfR)] and of iron overload biomarkers [serum ferritin (SF), liver iron concentration (LIC) and cardiac T2*] and hepcidin. This test measures the amount of soluble transferrin receptors to help detect and evaluate iron deficiency and aid in the diagnosis of iron deficiency anemia. See Transferrin Saturation. Nucleated red blood cells and soluble transferrin receptor in thalassemia syndromes: relationship with global and ineffective erythropoiesis Clin Chem Lab Med. It consists of two identical subunits linked by disulfide bridges. Each of the monomers has an 85 kD C-terminal component which can bind an iron-laden transferrin molecule. IntroductionBeta thalassemia is an inherited disorder characterised by ineffective erythropoiesis leading to anemia and secondary iron overload. P. Ricchi, M. Ammirabile, S. Costantini et al., "A useful relationship between the presence of extramedullary erythropoeisis and the level of the soluble form of the transferrin receptor in a large cohort of adult patients with thalassemia intermedia: A prospective study," Annals of Hematology, vol. Link to publication in Scopus. In thalassemia intermedia (TI), the increase in bone marrow hemopoietic activity frequently leads to extramedullary erythropoeisis (EMH), but its relationship with the soluble form of transferrin receptor (sTfR) which fully reflects the marrow erythropoietic activity, has not yet been explored. skarmoutsou et al 18 measured a series of hematological parameters, including reticulocyte hemoglobin content (chr), soluble transferrin receptor (stfr), reticulocytes and hb a 2 and f levels in 57 iron-replete individuals with heterozygous β thalassemia. We measured plasma levels of soluble transferrin receptor . . Epub 2017 Aug 2. S tudies indicate that the concentra- tion of. Soluble TfR is a truncated monomer of tissue receptor, lacking its first 100 amino acids, which circulates in the form of a . Soluble transferrin receptors (sTfRs) are proteins found in the blood that are cleaved from the membrane-bound transferrin receptors found on nearly all cells [42]. Increased levels of transferrin receptors (TfR) and soluble TfR (sTfR) are reliable indicators of iron deficiency anemia and conditions with enhanced erythropoietic activity. 1 Although the introduction of transfusions improved survival in TDT patients, it did not come without its own side-effect, systemic iron . the ratio of serum iron/total iron binding capacity and serum ferritin coupled with genetic testing and non-invasive magnetic resonance imaging measurements of liver iron content, define the nature and severity of iron loading in both hemochromatosis77 and thalassemia.110 Other useful markers are the level of . We measured plasma levels of soluble transferrin receptor (sTfR) and leptin by enzyme-linked immunosorbent assay (ELISA) in 40 beta-thalassemia patients (21 transfusion dependent and 19 not transfused or sporadically transfused), in 20 beta-thalassemia carriers, and in 30 healthy individuals (HI). Congenital anaemias due to ineffective erythropoiesis may be associated with excessive iron absorption and progressive iron loading. The goal of our study was to evaluate NRBC counts and the concentrations of serum transferrin receptor (sTfR) in patients affected by different thalassemia syndromes and hereditary spherocytosis. 2017 Sep;147(9):1785-1794. doi: 10.3945/jn.117.252635. We studied 57 iron-sufficient erozygous β-thalassemia, although a wide variation adults with heterozygous β-thalassemia, divided into groups from case-to-case has been observed.1,2 according to genotype: group A, βsilent-thalassemia het- unThe level of soluble transferrin receptors (sTfR) in the erozygotes, group B, β+-thalassemia . 10.1002/ajh.20804. Ricchi P, Ammirabile M, Costantini S. et al. (excess of free α-globin chains, bilirubin, soluble transferrin receptor and ferritin). Gebremedhin S. Soluble transferrin receptor level, inflammation markers, malaria, alpha‐thalassemia and selenium status are the major predictors of hemoglobin in children 6-23 months in Malawi. Forty-six children . Hypochromic microcytic anaemia includes iron deficiency, anaemia of chronic disorders, beta thalassemia trait and sideroblastic anaemia. Neutr… Acta 2003, 329, 9-22. Am J Hematol 60:121-125 receptors. spherocytosis, sickle cell anemia, thalassemia major or intermedia, megaloblastic . Soluble transferrin receptor (sTfR) is a truncated form of the cellular transferrin receptor, without its transmembrane and cytoplasmic domains, and circulates bound to transferrin . The concentration of sTfR is an indicator of iron status. The goal of our study was to evaluate NRBC counts and the concentrations of serum transferrin receptor (sTfR) in patients affected by different thalassemia syndromes and hereditary spherocytosis . The transferrin receptor is an integral membrane glycoprotein having a molecular weight of 190 kilodaltons (kD). It consists of two identical subunits linked by disulfide bridges. There was a strong inverse relationship between urinary hepcidin levels and both erythropoietin and soluble transferrin receptor, markers of erythropoietic activity. 2009;47(12):1539-42. doi: 10.1515/CCLM.2009.340. Proteolysis leads to the soluble form of the transferrin . Expression of TfR is. Elevation of the soluble transferrin receptor may be also caused by haemolytic anaemia, polycythaemia and thalassemia while aplastic anaemia and chronic renal failure may result in its decrease. Nucleated red blood cells and soluble transferrin receptor in thalassemia syndromes: relationship with global and ineffective erythropoiesis. Iron is an essential nutrient absorbed from the diet. A soluble form of the TfR (sTfR) has been identified in animal and human serum. Soluble transferrin receptor (sTfR) is a product of the TfR that circulates in plasma, its concentration therefore, is proportional to the total concentration of cellular TfR. Thus, the concentration of sTfR in plasma or serum is an indirect measure of total TfR. In most (7/8) of the patients a decrease of the transfusion demand index was observed. 6, pp. In contrast, hepcidin levels were elevated in thalassemia major, presumably due to transfusions that reduce erythropoietic drive and deliver a large iron load. sTfR values, a reliable quantitative assessment of the erythropoietic activity, were . Correlation between soluble transferrin receptor and serum ferritin levels following bone marrow transplantation for thalassemia Abstract This study analyzes the serum transferrin receptor (sTfR) levels in a series of 230 ex-thalassemics with a follow-up of 1 to 9 years after bone marrow transplantation (BMT) for homozygous beta thalassemia. The soluble transferrin receptor is a trun- cated form of tissue receptor that circulates in serum as a complex of transferrin and its receptor. Overall, these changes were not accompanied by a significant increase in soluble TfR. TfR is abundant on the surface of many newly formed cells, but the erythroid marrow cells account for 70 to 80 % of the total body TfR content. High sTfR level indicates iron deficiency even if ferritin elevated. The mean soluble transferrin receptor concentration was 1.9 ± .8 mg/L, and the mean TfR-F index was 1.21 ± .57. We retrospectively evaluated the relationship between serum transferrin receptor-1 (sTfR1) and some fundamental events in the life and the management (the age at diagnosis, the age at the first red blood cells transfusion, the age at splenectomy, and the overall need of chelation therapy) of 111 patients with non-transfusion-dependent thalassemia (NTDT) subdivided in four genetic entities . 2017 Sep;147(9):1785-1794. doi: 10.3945/jn.117.252635. It is NOT sensitive to inflammation. sTfR is elevated in patients with thalassemia and sickle cell disease. In this study wemeasured sTfR levels in the cases of beta-thalassemia trait (βTT) and IDA to see whether it would be a diagnostic tool for the differentiation of βTT and IDA. Soluble Transferrin Receptor (sTfR) Soluble Transferrin Receptor (sTfR) « Find Another Test . The aim of the study was to investigate the association of leptin with hematological parameters in β-thalassemia patients in Greece. In stratified, adjusted analyses, only parity was associated with anemia (odds ratio, OR (95% CI): 11.34 (1.94, 66.15)) for those with thalassemia (28% prevalent). 905-909, 2012. beta-Thalassemia / therapy Substances Receptors, Transferrin . Chim. The transferrin receptor (TfR) is the gateway for transferrin-bound-iron entering all body cells. Authors Paolo Danise 1 . Soluble transferrin receptor had negative association with DMT1A (containing IRE region) and IRP2 respectively (r=-0.900; p=0.037 and r=-0.750; p=0.052). Whether the soluble transferrin receptor (sTfR), a marker of erythropoietic activity, correlates with the bone mineral density (BMD) in thalassemic patients has not previously been addressed. Serum soluble transferrin receptor concentrations are elevated in Congolese children with glucose-6-phosphate dehydrogenase variants, but not sickle cell variants or α-thalassemia. Plasma membrane-bound transferrin receptor is released by proteolytic cleavage of the extracellular domain, resulting in the formation of a truncated soluble transferrin receptor (sTfR) that circulates freely in the blood. Extensively studied, . membrane receptor, the transferrin receptor (TfR). Soluble TfR is a truncated monomer of tissue receptor, lacking its first 100 amino acids, which circulates in the form of a . High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin. to study this problem, we have measured the concentrations of soluble transferrin receptor (stfr) and ferritin in 181 children from vanuatu with heterozygous (68) and homozygous (46) α + thalassaemia, and normal controls (67). Soluble transferrin receptor arises from proteolysis of the intact protein on the cell surface, leading to monomers that can be measured in plasma and serum. Another concern is that the reference values could be affected by hematological disorders, such as thalassemia or hemochromatosis. Each of the monomers has an 85 kD C-terminal component which can bind an iron-laden transferrin molecule. In order for iron to be internalized into cells, the iron-transferrin complex binds to these receptors. Iron deficiency and heterozygous beta-thalassemia are important causes of hypochromic-microcytic anemia. Caution should be exercised in managing anemia in these individuals based on sTfR test . Capillary blood was analyzed for iron (plasma ferritin, soluble transferrin receptor), inflammation (C-reactive protein) and thalassemia (β thalassemia and Hb E) status. to study this problem, we have measured the concentrations of soluble transferrin receptor (stir) and ferritin in 181 children from vanuatu with heterozygous (68) and homozygous (46) alpha+ thalassaemia, and normal controls (67). Transferrin saturation (Tsat), i.e. Nucleated red blood cells and soluble transferrin receptor in thalassemia syndromes: relationship with global and ineffective erythropoiesis Paolo Danise 1 , Giovanni Amendola 2 , Rosanna Di Concilio 2 , Enrico Cillari 3 , Maria Gioia 3 , Anna Di Palma 1 , Daniela Avino 1 , Paolo Rigano 4 and Aurelio Maggio 4 The aim of the study was to investigate the association of leptin with hematological parameters in beta-thalassemia patients in Greece. 2007;13:1096-101 8. Laboratory findings in iron deficiency anemia, thalassemia, and anemia of chronic disease/inflammation. 1993 Oct 23;342(8878):1058. doi: 10.1016/0140-6736(93)92916-h. . Other files and links. The soluble Transferrin Receptor (sTfR) has been suggested to be less influenced by the infectious and inflammatory status. PDF | Introduction: The β-thalassemias are due to autosomal mutations of the β-globin gene, inducing absence or low-level synthesis of β-globin in. A soluble form of the TfR (sTfR) has been identified in animal and human serum. Soluble transferrin receptor; Thalassemia; Access to Document. Tancabelic J, Sheth S, Paik M, Piomelli S (1999) Serum 22. The soluble transferrin receptor (sTfR) immunoassay should not be used for the routine clinical evaluation of patients for iron status when ferritin immunoassay (FERR / Ferritin, Serum) would be appropriate, such as in the absence of confounding pathologies (inflammation, infection, chronic disease, or malignancy). Transferrin receptors are present on the external surface of the plasma membrane. Beguin, Y. Soluble transferrin receptor for the evaluation of erythropoiesis and iron status. Nucleated red blood cells and soluble transferrin receptor in thalassemia syndromes: relationship with global and ineffective erythropoiesis Paolo Danise1,*, Giovanni Amendola2, Rosanna Di Concilio2, Enrico Cillari3, Maria Gioia3, Anna Di Palma1, Daniela Avino1, Paolo Rigano4 and Aurelio Maggio4 1 Department of Laboratory of Hematology, From January 2007 to December 2010, all TI patients attending at our center were prospectively . A second important conclusion of our trial was that sirolimus influences erythropoiesis and reduces biochemical markers associated to ineffective erythropoiesis (I.E.) stfr concentrations were significantly higher in both homozygotes (mean 3.1 mg/l, range 2.8-3.4) and heterozygotes … For instance, soluble transferrin receptor protein is a peptide deriving from transmembrane transferrin receptor, which is expressed in iron requiring cells; therefore, soluble transferring receptor concentration is directly proportional with cellular receptor density, revealing total iron demand. Thus, the concentration of sTfR in plasma or serum is an indirect measure of total TfR. Alpha thalassemia is associated with increased soluble transferrin receptor levels Br J Haematol , 103 ( 1998 ) , pp. Soluble transferrin receptor in beta-thalassaemia Lancet. Figure 2: GDF15 concentration in human blood and correlation with concentrations of hemoglobin, erythropoietin, soluble transferrin receptor and ferritin in blood from thalassemia patients. SOLUBLE TRANSFERRIN RECEPTOR (STFR), SERUM. Fingerprint Dive into the research topics of 'Hypocholesterolemia in chronic anemias with increased erythropoietic activity'. The serum level of sTfR reflects either the cellular need for iron or the rate of erythropoiesis. cells isolated from -thalassemia patients treated with low-dose sirolimus. serum soluble transferrin receptor (sTfR) con-centration until the appearance of anemia in which most of the laboratory parameters become abnormal2,3. Two laboratory parameters are suggested for the differentiation of such anemia. Clin. Patients with hemolysis and recent blood loss may have falsely elevated sTfR levels. Soluble form of transferrin receptor as a biomarker of overall morbidity in patients with non-transfusion-dependent thalassaemia: a cross-sectional study. Methodology: A total of 80 subjects were enrolled in this study. 365 - 369 View Record in Scopus Google Scholar Skarmoutsou et al 18 measured a series of hematological parameters, including reticulocyte hemoglobin content (CHr), soluble transferrin receptor (sTfR), reticulocytes and Hb A 2 and F levels in 57 iron-replete individuals with heterozygous β thalassemia. The serum level of sTfR reflects either the cellular need for iron or the rate of erythropoiesis. Soluble transferrin receptor in beta-thalassaemia. 1. (excess of free -globin chains, bilirubin, soluble transferrin receptor and ferritin). Increased marrow erythropoiesis in patients with thalassemia syndromes results in the expansion of bone marrow cavities and consequently decreases bone tissues, leading to osteoporosis. Serum Soluble Transferrin Receptor Concentrations Are Elevated in Congolese Children with Glucose-6-Phosphate Dehydrogenase Variants, but Not Sickle Cell Variants or α-Thalassemia J Nutr . Soluble transferrin receptor in beta-thalassaemia. We measured plasma levels of soluble transferrin receptor (sTfR) and leptin by enzyme-linked immunosorbent assay (ELISA) in 40 β-thalassemia patients (21 transfusion dependent and 19 not transfused or sporadically transfused), in 20 β-thalassemia carriers, and . Serum Soluble Transferrin Receptor Concentrations Are Elevated in Congolese Children with Glucose-6-Phosphate Dehydrogenase Variants, but Not Sickle Cell Variants or α-Thalassemia. However, . Refer to UpToDate topic on iron deficiency anemia for early changes associated with iron deficiency (before anemia develops). Refer to UpToDate topics on anemia for further details of the evaluation and interpretation. Soluble transferrin receptor; Thalassemia; Access to Document. Together they form a unique fingerprint. Anemia of CKD: Hypoproliferative, normocytic (usually), and normochromic anemia. Iron deficiency causes overexpression of transferrin receptor and . Thalassemia (thal) is an autosomal recessive hereditary chronic hemolytic anemia due to a partial or complete deficiency in the synthesis of α-globin chains (α-thal) or β-globin chains (β-thal) which compose the major adult hemoglobin (HbA), a tetramer of α 2β It is caused by one or more of several hundred mutations in the corresponding genes. membrane receptor, the transferrin receptor (TfR). Clinical Chemistry & Laboratory Medicine 38(4):377, 2000. Other files and links. The aim of the study was to investigate the association of leptin with hematological parameters in β-thalassemia patients in Greece. Conclusions: Nearly 19% of the study subjects had mild anemia at recruitment, and depletion of iron stores was observed among 18%. Nat Med. Determination of soluble transferrin receptors is a helpful laboratory test for the diagnosis with certainty. Journal of Nutrition , 147 (9), 1785-1794. Proteolysis leads to the soluble form of the transferrin . The soluble (or serum) transferrin receptor (sTfR) is a circulating truncated form of the membrane . Soluble transferrin receptor level: a new marker of iron deficiency anemia, a common manifestation of gastric autoimmunity in type 1 diabetes. Soluble transferrin receptor arises from proteolysis of the intact protein on the cell surface, leading to monomers that can be measured in plasma and serum. The serum concentration of sTfR is related to tissue receptor expression and therefore provides a clinical measure of the potential for cell proliferation (erythropoiesis) [42]. The conventional laboratory tests used for diagnosis have few disadvantages. Fingerprint Dive into the research topics of 'Hypocholesterolemia in chronic anemias with increased erythropoietic activity'. 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