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1.
Hematol., Transfus. Cell Ther. (Impr.) ; 44(1): 95-99, Jan.-Mar. 2022. tab
Article in English | LILACS | ID: biblio-1364881

ABSTRACT

Abstract Hemochromatosis is currently characterized by the iron overload caused by hepcidin deficiency. Large advances in the knowledge on the hemochromatosis pathophysiology have occurred due to a better understanding of the protein of the iron metabolism, the genetic basis of hemochromatosis and of other iron overload diseases or conditions which can lead to this phenotype. In the present review, the main aims are to show updates on hemochromatosis and to report a practical set of therapeutic recommendations for the human factors engineering protein (HFE) hemochromatosis for the p.Cys282Tyr (C282Y/C282Y) homozygous genotype, elaborated by the Haemochromatosis International Taskforce.


Subject(s)
Humans , Male , Female , Iron Metabolism Disorders , Hemochromatosis/diagnosis , Hemochromatosis/therapy , Phlebotomy , Iron Overload , Hepcidins/deficiency , Hemochromatosis Protein
2.
Article in Chinese | WPRIM | ID: wpr-887867

ABSTRACT

Objective To establish a mouse model of exogenous iron overload combined with tuberculosis(TB). Methods C57BL/6N mice were divided into negative control, low-, medium-, and high-dose iron groups and received intraperitoneal injection of iron dextran at 0, 3.75, 7.50, and 15.00 mg/dose(3 times/week for 4 weeks), respectively.After 4 weeks, the organ morphology and body weight of the mice were evaluated.The content of serum iron, ferritin, transferrin, and transferrin receptor was determined by ELISA.Heart, liver, spleen, lung, kidney, and small intestine were analyzed for tissue iron content and iron deposition pathology.


Subject(s)
Animals , Humans , Iron , Iron Overload , Iron-Dextran Complex , Mice , Mice, Inbred C57BL , Tuberculosis
3.
Journal of Experimental Hematology ; (6): 1881-1886, 2021.
Article in Chinese | WPRIM | ID: wpr-922217

ABSTRACT

OBJECTIVE@#To investigate the prevalence rate of hypothyroidism in children with β-thalassemia major (β-TM) and its risk factors.@*METHODS@#A total of 86 children with β-TM treated and followed up in the Department of Pediatrics of the Fifth Affiliated Hospital of Sun Yat-Sen University, Zhuhai Municipal Maternal and Child Health Care Hospital from August 2018 to August 2020 were enrolled. The clinical data of the children were analyzed to investigate the prevalence rate of hypothyroidism in children with β-thalassemia major (β-TM) and its risk factors.@*RESULTS@#The prevalence rate of hypothyroidism in children with β-TM in Zhuhai area was 17.4%. The level of serum ferritin(SF) (4948.27±1225.33 μg/L) in hypothyroidism children was significantly increased(t=10.273,P<0.05). The prevalence rate of hypothyroidism was significantly higher in β-TM children(age ≥10 years old, SF ≥2 500 μg/L and irregular iron removal) (P<0.05). Logistic regression result showed that age ≥10 years old was the independent risk factor affecting the increasing of hypothyroidism rate in the children. The levels of SF(3880.60±1269.17 μg/L), TSH(4.43±1.52 mIU/L) and the prevalence rate of hypothyroidism(37.14%)(P<0.05) were higher for the children in irregular iron removal group.@*CONCLUSION@#The prevalence rate of hypothyroidism in children with β-TM in Zhuhai area is high, and it is related to the age ≥10 years old, SF ≥2 500 μg/L and irregular iron removal of the children.


Subject(s)
Child , Humans , Hypothyroidism/epidemiology , Iron Overload , Prevalence , Risk Factors , beta-Thalassemia/epidemiology
4.
Article in Chinese | WPRIM | ID: wpr-879516

ABSTRACT

β-thalassemia is a type of inherited hemolytic anemia caused by decreased globin production due to defect of the HBB gene. The pathogenesis of the disease is imbalance of α/β globin chains. The excess of α-globin chains will form hemichromes which can damage red blood cell membranes and lead to hemolysis, ineffective erythropoiesis, and secondary iron overload. Iron overload in turn can cause complications such as growth retardation, liver cirrhosis, cardiac insufficiency, and aggravate the disease phenotype. In recent decades, genes participating in iron metabolism have been discovered, and the mechanism of iron metabolism in the development of thalassemia has gradually been elucidated. Subsequently, by manipulating the expression of key genes in iron metabolism such as hepcidin and transferrin receptor, researchers have revealed that iron restriction can improve ineffective hematopoiesis and iron overload, which may provide a potential approach for the treatment of thalassemia. This article reviews the progress of research on iron metabolism-related genes and related pathways in β-thalassemia.


Subject(s)
Humans , Iron/metabolism , Iron Overload/genetics , Phenotype , Research/trends , beta-Thalassemia/physiopathology
5.
Medicentro (Villa Clara) ; 24(4): 842-849, oct.-dic. 2020. graf
Article in Spanish | LILACS | ID: biblio-1143251

ABSTRACT

RESUMEN La hemocromatosis hereditaria es una enfermedad metabólica infrecuente que afecta primariamente al hígado, y que se caracteriza por un incremento de la absorción intestinal de hierro. Se presentó un paciente de 49 años de edad, evaluado en consulta externa, desde alrededor de dos años atrás, por: astenia, anorexia, artralgias e hiperpigmentación cutánea, asociada a hipertransaminasemia y seronegatividad para virus B y C. Los niveles de saturación de transferrina y ferritina evidenciaron la sobrecarga de hierro, y el estado homocigoto para la mutación C282Y confirmó la sospecha diagnóstica; se descartaron otras condiciones como: hepatitis crónica por virus B y C, esteatohepatitis no alcohólica, anemia hemolítica crónica, anemia sideroblástica, talasemia mayor, u otras enfermedades metabólicas que afectan al hígado. La biopsia hepática mostró hallazgos típicos de esta condición. Las flebotomías semanales fueron bien toleradas y se logró una mejoría clínica del paciente y de los parámetros de laboratorio.


ABSTRACT Hereditary hemochromatosis is an uncommon metabolic disease, primarily affecting the liver in which increased intestinal absorption of iron is seen. We presented a 49- year -old patient who was evaluated in an outpatient clinic for suffering from asthenia, anorexia, arthralgia and skin hyperpigmentation associated with hypertransaminasemia and negative serology for B and C viruses from about two years ago. Serum ferritin, and transferrin saturation levels evidenced iron overload and homozygosity for the C282Y mutation confirmed the suspected diagnosis; other conditions were ruled out such as chronic hepatitis due to B and C viruses, non-alcoholic steatohepatitis, chronic hemolytic anemia, sideroblastic anemia, thalassemia major or some other metabolic diseases affecting the liver. Liver biopsy showed typical findings related to this condition. Weekly phlebotomies were well tolerated, as well as clinical improvement of the patient and laboratory parameters were achieved.


Subject(s)
Iron Overload , Hemochromatosis
6.
Journal of Experimental Hematology ; (6): 1406-1409, 2020.
Article in Chinese | WPRIM | ID: wpr-827104

ABSTRACT

OBJECTIVE@#To explore the possible etiological factors of iron overload through detecting plasma hepcidin level of adult males at Tibet plateau.@*METHODS@#81 Tibetan male adult patients hospitalized in our department during January 2017 - December 2018 were selected, and divided into iron overload group and non-iron overload group. The difference in serum ferritin, serum iron, total iron binding capacity, hemoglobin, HBSAg, ALT, AST, albumin, creatinine and hepcidin of patients in each group were tested. To analyze the differences between groups. The regression analysis was applied to analyze the relationship between laboratory index and hepcidin.@*RESULTS@#The plasma hepcidin of iron overload group was significantly higher than that of the non-iron overload group [93.69 (65.57-133.92) ng/ml vs 63.93 (40.01-90.65) ng/ml] (P=0.005). And there was a positive correlation between plasma hepcidin and ferritin (β=0.03 ng/ml,95%CI 0.01-0.05) (P<0.01) and BMI (β=5.71 ng/ml,95%CI 0.54-10.88) (P<0.05).@*CONCLUSION@#Iron overload at Tibet plateau can not be attributed to hepcidin deficiency in Tibetan adult male patients. Iron metabolism disorders in Tibetan population may be associated with metabolic syndrome.


Subject(s)
Adult , Ferritins , Hepcidins , Humans , Iron , Iron Overload , Male , Tibet
7.
Autops. Case Rep ; 10(3): e2020180, 2020. graf
Article in English | LILACS | ID: biblio-1131840

ABSTRACT

Hepatic cirrhosis, diabetes mellitus and iron overload can each independently predispose to cryptococcosis. Hereditary hemochromatosis leads to all three of these predispositions. This report is the case of a patient with chronic hepatitis B virus infection and cirrhosis, who had markedly elevated serum ferritin and 99% transferrin saturation, and developed a leukemoid reaction. Autopsy revealed disseminated cryptococcosis for which the leukemoid reaction was a clue and possible hereditary hemochromatosis of which elevated ferritin and transferrin saturation can be clues. Hereditary hemochromatosis is an important diagnosis clinicians should never miss because early treatment with phlebotomy can be life-saving. Disseminated cryptococcosis can be rapidly diagnosed with serum cryptococcal antigen test and is treatable.


Subject(s)
Humans , Male , Middle Aged , Cryptococcosis/pathology , Hemochromatosis/pathology , Autopsy , Transferrin , Fatal Outcome , Iron Overload , Ferritins , Hepatitis , Liver Cirrhosis
8.
Arch. argent. pediatr ; 117(6): 684-687, dic. 2019. tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1051382

ABSTRACT

La xerocitosis hereditaria es un desorden poco frecuente causado por defectos en la permeabilidad eritrocitaria, que se caracteriza por anemia hemolítica de gravedad variable y sobrecarga de hierro. El diagnóstico suele ser tardío y confundirse con otras anemias hemolíticas, lo que puede llevar a indicaciones de procedimientos, como la esplenectomía, contraindicados en estos pacientes. Se reportan las características clínicas, hematológicas y moleculares de dos pacientes pediátricos no relacionados con diagnóstico de xerocitosis hereditaria. Ambos presentaban eritrocitos deshidratados con alta concentración de hemoglobina corpuscular media, frotis no patognomónico, marcadores de hemólisis y una curva de fragilidad osmótica resistente. El diagnóstico se confirmó por la secuenciación del gen PIEZO.Se resalta la importancia de reconocer la causa de la anemia hemolítica para dar un enfoque terapéutico preciso y dar adecuado consejo genético


Hereditary xerocytosis is a rare disorder caused by defects of red blood cell permeability that are characterized by hemolytic anemia of variable degree and iron overload. Diagnosis is usually late and confused with other hemolytic anemias, which can lead to procedural indications, such as splenectomy, contraindicated in these patients. We report the clinical, haematological, and molecular characteristics of two patients from two unrelated families affected by hereditary xerocytosis. Both patients had dehydrated erythrocytes with a high concentration of mean corpuscular hemoglobin, non-pathognomonic smears, markers of hemolysis and a resistant osmotic fragility curve. The diagnosis was confirmed by the sequencing of the PIEZO gene. We emphasize the importance of recognizing the cause of hemolytic anemia to give an accurate therapeutic approach and give adequate genetic counseling.


Subject(s)
Humans , Male , Female , Child , Adolescent , Hydrops Fetalis/diagnosis , Anemia, Hemolytic, Congenital/diagnosis , Mutation , Pedigree , Hemoglobins/analysis , Iron Overload , Erythrocyte Indices , Anemia, Hemolytic, Congenital/complications , Anemia, Hemolytic, Congenital/genetics , Anemia, Hemolytic, Congenital/blood , Jaundice, Neonatal
9.
Rev. Assoc. Med. Bras. (1992) ; 65(9): 1216-1222, Sept. 2019. tab, graf
Article in English | LILACS | ID: biblio-1041073

ABSTRACT

SUMMARY INTRODUCTION Iron overload is a broad syndrome with a large spectrum of causative etiologies that lead to iron deposition. When iron exceeds defenses, it causes oxidative damage and tissular disfunction. Treatment may prevent organ dysfunction, leading to greater life expectancy. METHODS Literature from the last five years was reviewed through the use of the PubMed database in search of treatment strategies. DISCUSSION Different pharmacological and non-pharmacological strategies are available for the treatment of iron overload and must be used according to etiology and patient compliance. Therapeutic phlebotomy is the basis for the treatment of hereditary hemochromatosis. Transfusional overload patients and those who cannot tolerate phlebotomy need iron chelators. CONCLUSION Advances in the understanding of iron overload have lead to great advances in therapies and new pharmacological targets. Research has lead to better compliance with the use of oral chelators and less toxic drugs.


RESUMO INTRODUÇÃO A síndrome de sobrecarga de ferro engloba um grande espectro de etiologias que levam a um aumento da quantidade de ferro nos tecidos. Esse ferro excede a capacidade de proteção dos tecidos, levando a dano oxidativo e lesão tissular. Tratamento pode prevenir esse dano, levando à melhor sobrevida. METODOLOGIA A literatura dos últimos cinco anos foi revisada por meio de pesquisa na base de dados PubMed buscando identificar estratégias de tratamento. DISCUSSÃO Medidas farmacológicas e não farmacológicas estão disponíveis para o tratamento da síndrome de sobrecarga de ferro e devem ser utilizadas de acordo com a etiologia e a aceitação do paciente. A flebotomia terapêutica é base do tratamento dos pacientes com hemocromatose hereditária. Pacientes com sobrecarga transfusional ou aqueles que não toleram flebotomias devem utilizar quelantes de ferro. CONSIDERAÇÕES FINAIS Avanços no entendimento da síndrome de sobrecarga de ferro têm levado a grandes progressos na terapêutica, com promessas de abordagem de novos alvos farmacológicos. A evolução da pesquisa tem possibilitado melhor aderência com o uso de quelantes orais e com possibilidade de drogas menos tóxicas.


Subject(s)
Humans , Iron Chelating Agents/therapeutic use , Iron Overload/therapy , Syndrome , Patient Compliance , Phlebotomy/methods , Hemochromatosis/therapy
10.
Hematol., Transfus. Cell Ther. (Impr.) ; 41(2): 139-144, Apr.-June 2019. tab
Article in English | LILACS | ID: biblio-1012188

ABSTRACT

ABSTRACT Objective: This is a quantitative transversal study that aimed to analyze the sociodemographic and clinical characteristics of thalassemia major patients with and without diabetes mellitus. Method: The cohort consisted of 31 thalassemia major patients from a reference center of treatment in Brazil in 2016. The data were obtained from an interview using a questionnaire containing demographic and clinical variables. The results show that 16.1% of the participants with thalassemia major had diabetes mellitus. The participants' ages ranged from 20 to 48 years, with an average of 35 years, mostly students and starting in the formal job market. The most commonly used treatment was the oral desferasirox and the transfusion treatment interval was 15-22 days. Results: Patients with thalassemia major and diabetes mellitus presented altered values of fasting glycemia, serum alanine transaminase, magnetic resonance imaging and bone densitometry. Conclusion: It was concluded that knowledge of the characteristics of this population contributes in the proposal of effective educational strategies in light of the complexity of care and the progression of the disease.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Thalassemia , beta-Thalassemia , Iron Overload , Diabetes Mellitus
11.
Blood Research ; : 87-101, 2019.
Article in English | WPRIM | ID: wpr-763065

ABSTRACT

An increase in biochemical concentrations of non-transferrin bound iron (NTBI) within the patients with an increase in serum iron concentration was evaluated with the following objectives: (a) Iron overloading diseases/conditions with free radicle form of ‘iron containing’ reactive oxygen species (ROS) and its imbalance mediated mortality, and (b) Intervention with iron containing drugs in context to increased redox iron concentration and treatment induced mortality. Literature search was done within Pubmed and cochrane review articles. The Redox iron levels are increased during dys-erythropoiesis and among transfusion recipient population and are responsive to iron-chelation therapy. Near expiry ‘stored blood units’ show a significant rise in the ROS level. Iron mediated ROS damage may be estimated by the serum antioxidant level, and show reduction in toxicity with high antioxidant, low pro-oxidant levels. Iron drug therapy causes a significant increase in NTBI and labile iron levels. Hospitalized patients on iron therapy however show a lower mortality rate. Serum ferritin is a mortality indicator among the high-dose iron therapy and transfusion dependent population. The cumulative difference of pre-chelation to post chelation ROS iron level was 0.97 (0.62; 1.32; N=261) among the transfusion dependent subjects and 2.89 (1.81–3.98; N=130) in the post iron therapy ‘iron ROS’ group. In conclusion, iron mediated mortality may not be mediated by redox iron among multi-transfused and iron overloaded patients.


Subject(s)
Drug Therapy , Ferritins , Hepcidins , Humans , Iron Overload , Iron , Mortality , Oxidation-Reduction , Reactive Oxygen Species
12.
Article in English | WPRIM | ID: wpr-785874

ABSTRACT

Hemosiderosis is characterized by the deposition of excess iron in body tissues. The choroid plexus is an important part of the central nervous system that can be the primary site of iron overload. T2*-weighted gradient echo (GRE) sequence provides high sensitivity for demonstrating cerebral microhemorrhagic foci and iron deposition. In the present study, we describe the case of a 15-year-old boy with acute lymphoblastic leukemia, in whom repeated transfusion led to iron accumulation in the brain. GRE sequence effectively demonstrated hemosiderin deposition in the choroid plexus.


Subject(s)
Adolescent , Brain , Central Nervous System , Choroid Plexus , Choroid , Hemosiderin , Hemosiderosis , Humans , Iron Overload , Iron , Magnetic Resonance Imaging , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma
13.
Acta Physiologica Sinica ; (6): 689-697, 2019.
Article in English | WPRIM | ID: wpr-777142

ABSTRACT

The aim of the present study was to investigate the role of ferroptosis in acute lung injury (ALI) mouse model induced by oleic acid (OA). ALI was induced in the mice via the lateral tail vein injection of pure OA. The histopathological score of lung, lung wet-dry weight ratio and the protein content of bronchoalveolar lavage fluid (BALF) were used as the evaluation indexes of ALI. Iron concentration, glutathione (GSH) and malondialdehyde (MDA) contents in the lung tissues were measured using corresponding assay kits. The ultrastructure of pulmonary cells was observed by transmission electron microscope (TEM), and the expression level of prostaglandin-endoperoxide synthase 2 (PTGS2) mRNA was detected by quantitative polymerase chain reaction (q-PCR). Protein expression levels of glutathione peroxidase 4 (GPX4), ferritin and transferrin receptor 1 (TfR1) in lung tissues were determined by Western blot. The results showed that histopathological scores of lung tissues, lung wet-dry weight ratio and protein in BALF in the OA group were higher than those of the control group. In the OA group, the mitochondria of pulmonary cells were shrunken, and the mitochondrial membrane was ruptured. The expression level of PTGS2 mRNA in the OA group was seven folds over that in the control group. Iron overload, GSH depletion and accumulation of MDA were observed in the OA group. Compared with the control group, the protein expression levels of GPX4 and ferritin in lung tissue were down-regulated in the OA group. These results suggest that ferroptosis plays a potential role in the pathogenesis of ALI in our mouse model, which may provide new insights for development of new drugs for ALI.


Subject(s)
Acute Lung Injury , Pathology , Animals , Apoptosis , Bronchoalveolar Lavage Fluid , Chemistry , Cyclooxygenase 2 , Metabolism , Ferritins , Metabolism , Glutathione , Glutathione Peroxidase , Metabolism , Iron , Iron Overload , Lung , Cell Biology , Pathology , Malondialdehyde , Mice , Microscopy, Electron, Transmission , Mitochondrial Membranes , Oleic Acid
14.
Article in Chinese | WPRIM | ID: wpr-771910

ABSTRACT

OBJECTIVE@#The explore the molecular basis of iron-overload in Tibet nationality population of Tibet.@*METHODS@#The inpatients with iron-overload in our department from Dec. 1st 2014 to Jul.31st 2016 were enrolled in this study. Abdominal MRI and the mutation sites C282Y and H63D in HFE exon were examined. For HFE mutation-negative patients, the non-HFE mutation was detected, including 5 HJV mutations of G320V, p.Q312X, p.D249H, p.I281T, p.C321X and 2 TFR2 mutations: (Y250X, I238M), and 2 SLC40A1 mutations: (V162del, N144H).@*RESULTS@#Among 113 iron overload patients, only one showed homozygous p.H63D mutation, and one showed heterozygosis p.H63D mutation. In 73 patients accepted non-HFE gene detection, only one was heterozygosis p.D249N mutation in HJV, and one was heterozygosis p.I238M mutation in TFR2.@*CONCLUSION@#Currently, the pathogenic gene for Tibetan iron-overload has not yet been found.


Subject(s)
Genotype , Hemochromatosis Protein , Histocompatibility Antigens Class I , Humans , Iron Overload , Mutation , Tibet
15.
São Paulo; s.n; s.n; 2019. 60 p. graf, tab.
Thesis in Portuguese | LILACS | ID: biblio-1023080

ABSTRACT

A sobrecarga de ferro é uma condição prejudicial para os pacientes, que apresentam uma diminuição significativa na qualidade de vida. Os fármacos quelantes são moléculas que têm capacidade de uso clínico para atuar como atenuadores da sobrecarga de metais. Neste trabalho apresentamos uma análise de sideróforos do tipo hidroxamato e quinona, com o objetivo de ampliar a gama de terapia de sobrecarga de ferro. Para cada composto foi realizado um ensaio competitivo com a sonda calce- ína para verificar a capacidade de ligação do ferro, e um ensaio antioxidante baseado na supressão da oxidação dependente de ferro da dihidrorrodamina (DHR) sob ascorbato. Foi observado que o hidroxamato cíclico piridoxatina apresentou capacidade de sequestrar ferro de substratos de alta afinidade, tanto em meio tamponado quanto em meio intracelular. Em ambas as situações também se mostrou um antioxidante eficiente. Entretanto, parece ser o mais tóxico do grupo dos hidroxamatos (que ainda continha o hidroxamato linear desferricoprogênio e o aromático desferriastercromo). Outros compostos naturais também foram estudados como possíveis candidatos a fármacos para sobrecarga de ferro. Complexos de ferro foram caracterizados por espectrofotometria para avaliar a estequiometria possível, considerando os sítios de ligação para cada composto. Ensaios de fluorescência revelaram que entre os quatro compostos em estudo (ácido clorogênico, lapachol, hemateína e hematoxilina), o complexo entre ferro e hemateína apresenta maior estabilidade relativa do que outros


Iron overload is a harmful condition for patients, who have a significant decrease in life quality. Chelating drugs are molecules that have the capacity for clinical use to act as attenuators of metal overload. In this work we present an analysis of hydroxamate and quinone-type siderophores, intending to broaden the range of iron overload therapy. For each compound it was conducted a competitive assay with the fluorescent probe calcein to verify the iron binding ability, and an antioxidant assay based on suppression of the iron-dependent oxidation of dihydrorhodamine (DHR) under ascorbate. It was observed that cyclic hydroxamate pyridoxatin displayed good ability to scavenge iron from high affinity substrates both in buffer and in intracellular medium. It was also an efficient antioxidant in both setups. However, pyridoxatin seems to be the most toxic from the hydroxamate group (composed also by the linear desferricoprogen and the aromatic desferriasterchrome). Other natural compounds have also been studied as possible candidates for iron-overload drug therapy. Iron complexes were characterized by spectrophotometry to assess the possible stoichiometry considering the binding sites for each compound. Fluorescence assays revealed that among the four compounds in study (chlorogenic acid, lapachol, hematein and hematoxylin), the complex between iron and hematein has higher relative stability than others


Subject(s)
Siderophores/analysis , Iron Overload/therapy , Fluorescence , Spectrophotometry/instrumentation , Chelation Therapy , Deferoxamine/classification , Iron/adverse effects , Antioxidants
16.
Rev. cuba. hematol. inmunol. hemoter ; 34(4): 1-8, oct.-dic. 2018. ilus, tab
Article in Spanish | LILACS, CUMED | ID: biblio-1003875

ABSTRACT

La sobrecarga de hierro es común en pacientes receptores de trasplante de progenitores hematopoyéticos. En estos pacientes, la ferritina y la saturación de transferrina, pre- y postrasplante, están aumentadas debido a las frecuentes transfusiones de sangre. Además, la sobrecarga de hierro postrasplante puede relacionarse con otras complicaciones como las infecciones, las mucositis y la enfermedad injerto contra hospedero. Una ferritina elevada antes del trasplante alogénico es un factor de mal pronóstico para la sobrevida y la mortalidad no relacionada. Para el monitoreo y diagnóstico de la sobrecarga de hierro se cuenta con diversa herramientas entre las que la ferritina continúa siendo una de las más utilizadas(AU)


Iron overload is common in hematopietic stem cell transplantation (HSCT) recipients. In these patients, pre- and early post-transplant ferritin and transferrin saturation were found to be highly elevated due to high transfusion requirements. In addition to that, post-HSCT iron overload may be related to other complications such as infections, mucositis, and acute graft-versus-host disease. An elevated ferritin level before allogeneic HSCT is an adverse prognostic factor for overall and nonrelapse mortality. There are several diagnostic tools for iron overload but ferritinis still one of the most used(AU)


Subject(s)
Humans , Hematopoietic Stem Cell Transplantation/adverse effects , Iron Overload/complications , Transfusion Reaction , Health Evaluation , Prospective Studies , Retrospective Studies
17.
Journal of Experimental Hematology ; (6): 1129-1136, 2018.
Article in Chinese | WPRIM | ID: wpr-689517

ABSTRACT

<p><b>OBJECTIVE</b>To establish a MDS mouse model with iron overload and to study the effect of iron overload on MDS.</p><p><b>METHODS</b>The exogenous mutant gene RUNX1-S291fs was inserted into the mice bone marrow mononuclear cell's genome in mice by retrovirus and transplanted into C57BL/6 mice irradiated by Co γ-ray. After 8 weeks,intraperitoneal injection of iron was performed to establish an MDS mouse model with iron overload. After 24 weeks of transplantation, the peripheral blood, bone marrow, femur, liver and spleen of mice were taken, then the morphological characteristics of peripheral blood and bone marrow cells were observed by Wright's staining; the liver, spleen and bone marrow were stained with Prussian blue to observe the iron deposition. The surface antigens of bone marrow cells were detected by flow cytometry. Bone marrow mononuclear cells and spleen tissue proteins were detected by Western blot to confirm the transfection of RUNX1-S291fs gene and expression of protein. The blood routine and transplanted cell chimeric rate of mice were monitored periodically.</p><p><b>RESULTS</b>Compared with the empty plasmid control mice, levels of leukocyte and hemoglobin as well as platelet were decreased in RUNX1-S291fs mutant mice; the peripheral blood cells and bone marrow cells showed pathological hematopoiesis; the liver and spleen enlarged significantly; the tissue structure of femur, liver and spleen was abnormal; the expression of bone marrow cell surface antigens was abnormal. Bone marrow cells and spleen tissue expressed the RUNX1-S291fs protein. Compared with the controlled mice injected with normal saline, iron deposition occurred in the bone marrow, liver and spleen stained with Prussian blue in the mice injected with iron agent.</p><p><b>CONCLUSION</b>Mice engineered to carry exogenous mutant gene RUNX1-S291fs and injected with iron showed pathologic features of MDS and iron overload, resulting in establishing MDS iron overloaded mouse model successfully, which lays a foundation for studying the effect of iron overload on MDS.</p>


Subject(s)
Animals , Bone Marrow , Disease Models, Animal , Iron Overload , Mice , Mice, Inbred C57BL , Spleen
18.
Blood Research ; : 314-319, 2018.
Article in English | WPRIM | ID: wpr-718478

ABSTRACT

BACKGROUND: Iron overload is a risk factor affecting all patients with thalassemia intermedia (TI). We aimed to determine whether there is a relationship of serum ferritin (SF) and alanine aminotransferase (ALT) with liver iron concentration (LIC) determined by R2 magnetic resonance imaging (R2-MRI), to estimate the most relevant degree of iron overload and best time to chelate in patients with TI. METHODS: In this cross-sectional study, 119 patients with TI (mean age years) were randomly selected and compared with 120 patients who had a diagnosis of thalassemia major (TM). Correlations of LIC, as determined by R2-MRI, with SF and ALT levels, were assessed in all participants. A P-value 5 mg Fe/g dry weight (P < 0.0001). A significant relationship was also found for patients with TI who had elevated ALT level (63.5 U/L), of 3.15 times the upper normal laboratory limit, using a cut-off for LIC ≥5 mg Fe/g dry weight. CONCLUSION: We determined the cut-off values for ALT and SF indicating the best time to start iron chelation therapy in patients with TI, and found significant correlations among iron overload, SF, and ALT.


Subject(s)
Adolescent , Alanine Transaminase , beta-Thalassemia , Chelation Therapy , Cross-Sectional Studies , Diagnosis , Ferritins , Humans , Iron Overload , Iron , Liver , Magnetic Resonance Imaging , Puberty , Risk Factors , Splenectomy , Thalassemia
19.
Article in Korean | WPRIM | ID: wpr-718428

ABSTRACT

BACKGROUND: Ferritin is used to detect iron overload in patients with chronic red blood cell transfusions. Although ferritin reflects the amount of iron storage in the body, it may increase nonspecifically in inflammation and infection. This study analyzed the cause of increased ferritin and the association with a red blood cell (RBC) transfusion. METHODS: The medical records of patients who visited the authors' hospital from January to December 2017 and underwent a ferritin test were reviewed retrospectively. Hyperferritinemia was defined as a ferritin level more than 1,000 ng/mL. The causes of hyperferritinemia were investigated by examining the laboratory findings and medical records. RESULTS: The results revealed 417 cases of hyperferritinemia in 238 patients during the period. The most common diseases were hematologic malignancies from 125 cases (30.0%) in 31 patients and infectious diseases were the second most common. Iron overload was suspected in 119 cases in 33 patients, and 12 patients (76 cases) were transfused with more than 8 units of RBC for 1 year before the test. CONCLUSION: In hyperferritinemia, the rate of iron overload is high considering the underlying diseases and chronic RBC transfusion. To determine iron storage status accurately, it will be helpful to measure the C-reactive protein (CRP) and iron saturation in the ferritin test. Careful attention should be paid to habitual iron formulations and frequent transfusions due to the possibility of iron overload.


Subject(s)
C-Reactive Protein , Communicable Diseases , Erythrocyte Transfusion , Erythrocytes , Ferritins , Hematologic Neoplasms , Humans , Inflammation , Iron , Iron Overload , Medical Records , Retrospective Studies
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