Oxidative stress status, clinical outcome, and β-globin gene cluster haplotypes in pediatric patients with sickle cell disease.

Abstract:

OBJECTIVE:To correlate the clinical and hematological features of β-globin gene haplotypes with the oxidative stress status in pediatric patients with sickle cell disease (SCD). METHODS:A total of 95 patients with SCD and 40 healthy children were studied. The β-globin cluster, plasma lipid peroxidation (LPO) and plasma nitrite plus nitrate (NOx), and erythrocyte content of glutathione (GSH) and glutathione disulfide (GSSG), and glutathione peroxidase (GPx), reductase (GRd), and superoxide dismutase (SOD) activities were measured. RESULTS:Plasma LPO (P < 0.001) and NOx (P < 0.05) were significantly higher in patients than in controls. In erythrocytes of patients with SCD, the activities of GRd (P < 0.001) and SOD (P < 0.05) were lower, and the GSSG/GSH ratio (P < 0.001) and GPx activity (P < 0.001) were higher than in controls. High LPO levels and low SOD plus GRd activities were associated with increased severity of clinical manifestations, which correspond mainly to patients with Bantu and Benin haplotypes. LPO levels were reduced in patients with high fetal hemoglobin (HbF) levels, whereas the NOx levels and GRd activity tended to increase in this group. CONCLUSION:Our results detected an important oxidative stress in patients with SCD and suggest that at least three redox markers, i.e., LPO, GRd, and SOD, were related with their clinical outcomes. Moreover, a relationship between high HbF and low LPO, and high HbF and high GRd activity and NOx levels were found.

journal_name

Eur J Haematol

authors

Rusanova I,Escames G,Cossio G,de Borace RG,Moreno B,Chahboune M,López LC,Díez T,Acuña-Castroviejo D

doi

10.1111/j.1600-0609.2010.01528.x

subject

Has Abstract

pub_date

2010-12-01 00:00:00

pages

529-37

issue

6

eissn

0902-4441

issn

1600-0609

journal_volume

85

pub_type

杂志文章
  • Clinical results according to age in patients with chronic myeloid leukemia receiving imatinib frontline: The younger, the later, the worse?

    abstract:OBJECTIVES:To evaluate differences in clinical results according to age among patients with chronic myeloid leukemia (CML). METHODS:207 consecutive CML patients treated with imatinib frontline were revised, dividing them in young adults (>20 < 45 years) (YA), middle-aged adults (≥45 < 65 years) (MA) and elderly (≥65 y...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13110

    authors: Latagliata R,Breccia M,Carmosino I,Cesini L,De Benedittis D,Mohamed S,Vozella F,Molica M,Campanelli M,De Luca ML,Colafigli G,Quattrocchi L,Loglisci MG,Massaro F,Canichella M,Diverio D,Mancini M,Alimena G,Foà R

    更新日期:2018-06-13 00:00:00

  • Simplification of the blood stem cell transplantation (BSCT) procedure: large volume apheresis and uncontrolled rate cryopreservation at -80 degrees C.

    abstract::Very high-dose chemotherapy with autologous blood stem cell (BSC) rescue becomes more and more widely performed. In order to simplify the technique, a large volume apheresis programme combined with an uncontrolled rate cryopreservation at -80 degrees C was developed. Twenty-six patients suffering from multiple myeloma...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章

    doi:10.1111/j.1600-0609.1996.tb00715.x

    authors: Feremans WW,Bastin G,Moine FL,Ravoet C,Delville JP,Pradier O,Wallef G,Dupont E,Capel P,Lambermont M

    更新日期:1996-05-01 00:00:00

  • Hematopoietic stem cell transplantation for sickle cell disease: state of the science.

    abstract::Sickle cell disease (SCD) is an inherited disorder secondary to a point mutation at the sixth position of the beta chain of human hemoglobin resulting in the replacement of valine for glutamic acid. This recessive genetic abnormality precipitates the polymerization of the deoxygenated form of hemoglobin S inducing a m...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/ejh.12447

    authors: Talano JA,Cairo MS

    更新日期:2015-05-01 00:00:00

  • Impact of hemophilia B on quality of life in affected men, women, and caregivers-Assessment of patient-reported outcomes in the B-HERO-S study.

    abstract:INTRODUCTION:Health-related quality of life (HRQoL) is impaired in patients with hemophilia; however, the impact in mild/moderate hemophilia B and affected women is not well characterized. OBJECTIVE:To evaluate factors that affect HRQoL in adults with hemophilia B and caregivers of affected children. METHODS:US adult...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13055

    authors: Buckner TW,Witkop M,Guelcher C,Sidonio R,Kessler CM,Clark DB,Owens W,Frick N,Iyer NN,Cooper DL

    更新日期:2018-06-01 00:00:00

  • Cardiac function during iron chelation therapy in adult non-thalassaemic patients with transfusional iron overload.

    abstract::It is well-documented that iron chelation by desferrioxamine protects/improves the cardiac function in blood transfusion-dependent children suffering from beta-thalassaemia. In patients who do not become dependent upon blood transfusion until adulthood (ANT-patients), iron chelation by desferrioxamine may affect the c...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1997.tb00981.x

    authors: Jensen PD,Olsen N,Bagger JP,Jensen FT,Christensen T,Ellegaard J

    更新日期:1997-10-01 00:00:00

  • Low-dose cyclophosphamide effectively mobilizes peripheral blood stem cells in patients with autoimmune disease.

    abstract::For patients with severe and refractory autoimmune diseases, high-dose chemotherapy and autologous hematopoietic stem cell transplantation has been established as a considerable therapeutic option in recent years. In this retrospective single-center analysis, we assessed the feasibility and efficacy of peripheral bloo...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.12686

    authors: Blank N,Lisenko K,Pavel P,Bruckner T,Ho AD,Wuchter P

    更新日期:2016-07-01 00:00:00

  • Infections after CD34-selected or unmanipulated autologous hematopoietic stem cell transplantation.

    abstract::Immune reconstitution may be delayed after CD34-selected compared with unmanipulated autologous peripheral blood stem cell transplantation (PBSCT), resulting in a theoretically increased risk of infections. In a case-control matched study we compared the incidence of infection in 25 recipients of CD34-selected PBSC (C...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2005.00569.x

    authors: Frère P,Pereira M,Fillet G,Beguin Y

    更新日期:2006-02-01 00:00:00

  • Prognostic role of immunohistochemical analysis of 5 mc in myelodysplastic syndromes.

    abstract:BACKGROUND:Aberrant DNA methylation at CpG islands within promoters is increasingly recognised as a common event in human cancers and has been associated with the silencing of important tumour suppressor genes. Epigenetic therapy using hypomethylating agents has demonstrated clinical effectiveness; the drugs azacitidin...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.12145

    authors: Poloni A,Goteri G,Zizzi A,Serrani F,Trappolini S,Costantini B,Mariani M,Olivieri A,Catarini M,Centurioni R,Alesiani F,Giantomassi F,Stramazzotti D,Biagetti S,Alfonsi S,Berardinelli E,Leoni P

    更新日期:2013-09-01 00:00:00

  • VBAMDex chemotherapy in advanced multiple myeloma.

    abstract::In a phase II study 28 patients with advanced multiple myeloma were treated with a five drug regimen consisting of vincristine, BCNU, adriamycin, melphalan and dexamethasone. 11 out of 13 patients without prior chemotherapy showed significant remissions (greater than 25% tumor cells mass reduction), 7 of them had more...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1988.tb00831.x

    authors: Peest D,Schmoll HJ,Schedel I,Glück S,Schumacher K,Deicher H

    更新日期:1988-03-01 00:00:00

  • Use of rituximab to treat refractory Diamond-Blackfan anemia.

    abstract::We report here the first case with Diamond-Blackfan anemia (DBA) who responded to rituximab. The patient is an 8-yr-old Japanese girl with refractory DBA accompanied by complex congenital heart disease. She received two doses of rituximab, 375 mg/m(2)/wk. She became transfusion independent 6 months after the treatment...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2004.00394.x

    authors: Morimoto A,Kuriyama K,Tsuji K,Isoda K,Hibi S,Todo S,Sugimoto T,Imashuku S

    更新日期:2005-05-01 00:00:00

  • Leukemia diagnosis: today and tomorrow.

    abstract::The European LeukemiaNet (ELN) is composed of several work packages, four of them being directly involved in the various aspects of diagnosis. On the occasion of the annual ELN meeting of 2015 in Mannheim, these four work packages collectively examined the current situation and future prospects of cytomorphology, flow...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/ejh.12603

    authors: Béné MC,Grimwade D,Haferlach C,Haferlach T,Zini G,European LeukemiaNet.

    更新日期:2015-10-01 00:00:00

  • Accumulation of vincristine and doxorubicin in malignant lymphocytes with different immunophenotypes.

    abstract::Cellular accumulation of vincristine (VCR) and doxorubicin (DOX) was studied in 15 patients with low-grade B-cell malignancies. Their leukemic lymphocytes were isolated and the effect of verapamil on drug uptake was studied. The immunophenotype was characterized using anti-IgM, -IgD, -B 1, monoclonal antibodies. The m...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1989.tb00334.x

    authors: Reizenstein P,Kapoor R,Gruber A,Peterson C

    更新日期:1989-11-01 00:00:00

  • Intensive remission induction therapy for chronic myeloid leukemia in blast phase with a goal of post-remission bone marrow transplant--a pilot study.

    abstract::An intensive protocol utilizing mitoxantrone, high-dose cytarabine, vincristine, etoposide and methylprednisolone as induction therapy for chronic myeloid leukemia in blast transformation is described. Fourteen patients were treated, with a remission/second chronic phase achieved in 64%. None of the 3 patients older t...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1996.tb00488.x

    authors: Lipton JH,Messner HA,Curtis JE,Atkins HL,Minden MD

    更新日期:1996-07-01 00:00:00

  • Thirty-year experience in preventing haemoglobinopathies in Greece: achievements and potentials for optimisation.

    abstract:OBJECTIVES:Beta thalassaemia major (β-TM) and sickle-cell disease (SCD) are severe haemogobinopathies requiring life-lasting, advanced medical management. In the Mediterranean region, both conditions occur with high frequency. We assessed the efficacy of the National Program for the Prevention of Haemoglobinopathies in...

    journal_title:European journal of haematology

    pub_type: 历史文章,杂志文章

    doi:10.1111/ejh.12076

    authors: Ladis V,Karagiorga-Lagana M,Tsatra I,Chouliaras G

    更新日期:2013-04-01 00:00:00

  • Clinical and microbiological impact of discontinuation of fluoroquinolone prophylaxis in patients with prolonged profound neutropenia.

    abstract:BACKGROUND:Infections remain a leading cause of morbidity and mortality in patients with reduced immunity caused by haematological disease and chemotherapy-induced neutropenia. We evaluated the clinical and microbiological impact of discontinuing fluoroquinolone prophylaxis in these patients. METHODS:We analysed 154 a...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.12345

    authors: Verlinden A,Jansens H,Goossens H,van de Velde AL,Schroyens WA,Berneman ZN,Gadisseur AP

    更新日期:2014-10-01 00:00:00

  • Changes in serum erythropoietin and the reticulocyte count during chemotherapy for leukemias.

    abstract::We serially determined serum erythropoietin (Epo) and the reticulocyte count in patients with various types of leukemia during chemotherapy. Serum Epo increased soon after the initiation of chemotherapy and decreased after the termination of therapy irrespective of the types of leukemia or treatment regimen. However, ...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1996.tb01397.x

    authors: Sawabe Y,Kikuno K,Iseki T,Lida S,Tabata Y,Yonemitsu H

    更新日期:1996-11-01 00:00:00

  • Hepatic and extrahepatic clearance of circulating human lactoferrin: an experimental study in rat.

    abstract::Lactoferrin, unlabelled or 125I-labelled by 2 different methods, was given intravenously to rats. Blood, tissue and liver cell radioactivity was measured. Both of the radiolabelled preparations were eliminated by the liver, and some deposited extrahepatically. One preparation formed large aggregates--here 90% of the h...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1998.tb01078.x

    authors: Peen E,Johansson A,Engquist M,Skogh T

    更新日期:1998-09-01 00:00:00

  • Acquired Factor VIII autoantibody: four cases demonstrating the heterogenous nature of this condition and problems involved in diagnosis and treatment.

    abstract::The development of an autoantibody to human Factor VIII is rare and presents many problems for diagnosis and treatment. We have seen several cases at our institution recently with widely heterogenous clinical and laboratory presentations. A wide range of treatment modalities were used in these cases with no gold stand...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1034/j.1600-0609.2001.00383.x

    authors: Hall RL,Leahy MF

    更新日期:2001-03-01 00:00:00

  • Interferon-alpha-induced changes in surface antigens in a hairy-cell leukemia (JOK-1), and a Burkitt's lymphoma cell line (Daudi) during in vitro culture.

    abstract::In further studying the mechanism of action of IFN-alpha in HCL, we cultured the HCL cell line JOK-1 and the IFN-sensitive Burkitt cell line Daudi with and without IFN-alpha and investigated the changes in density of a number of surface antigens by use of mAb and flow cytometry analyses. During culture with IFN-alpha,...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1992.tb00586.x

    authors: Nielsen B,Madsen PS,Jensen AW,Hokland P,Hokland M

    更新日期:1992-03-01 00:00:00

  • Long-term complication in follicular lymphoma: assessing the risk of secondary neoplasm in 242 patients treated or not with 90-yttrium-ibritumomab-tiuxetan.

    abstract:BACKGROUND:Non-Hodgkin lymphoma patients have a 25% increased risk of secondary primary neoplasms (SPNs). Regarding the controversy about the increased risk of SPN in patients exposed to radioimmunotherapy (RIT), we have analyzed this issue in a cohort of follicular lymphoma (FL) patients treated with/without RIT. PAT...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.12775

    authors: Andrade-Campos MM,Liévano P,Espinosa-Lara N,Soro-Alcubierre G,Grasa-Ulrich JM,López-Gómez L,Baringo T,Giraldo P

    更新日期:2016-12-01 00:00:00

  • Bone marrow WT1 levels in patients with myeloid neoplasms treated with 5-azacytidine: Identification of responding patients.

    abstract:INTRODUCTION:Increased levels of Wilms' tumor (WT1) mRNA have been used to establish risk categories in patients with acute myeloid leukemia (AML). Raised values of WT1 have been associated with progression in myelodysplastic syndrome (MDS). METHODS:We retrospectively analyzed the available bone marrow (BM) samples fr...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13275

    authors: Santaliestra M,Garrido A,Carricondo M,Bussaglia E,Pratcorona M,Blanco ML,Gich I,Hoyos M,Esquirol A,García-Cadenas I,Brunet S,Martino R,Sierra J,Nomdedéu JF

    更新日期:2019-09-01 00:00:00

  • Direct oral anticoagulants for atrial fibrillation in patients with congenital factor VII deficiency.

    abstract::The management of anticoagulant therapy (OAT) in patients with factor VII (FVII) deficiency is a very challenging clinical issue, as warfarin further reduces FVII levels, thus potentially increasing bleeding risk. On the other hand, the International Normalized Ratio test is misleading in such patients, as they do not...

    journal_title:European journal of haematology

    pub_type: 杂志文章,meta分析

    doi:10.1111/ejh.13246

    authors: Arletti L,Coluccio V,Romagnoli E,Luppi M,Marietta M

    更新日期:2019-07-01 00:00:00

  • Identification and characterization of the first large deletion of the MYH9 gene associated with MYH9 disorders.

    abstract::MYH9 disorders are autosomal dominant macrothrombocytopenias with leukocyte inclusion bodies. Single point mutations in the protein-coding sequence of the MYH9 gene are the most common cause. So far no large gene deletion/insertion and splicing defects have been reported. Conventional DNA sequencing of each MYH9-codin...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2008.01046.x

    authors: Kunishima S,Matsushita T,Hamaguchi M,Saito H

    更新日期:2008-06-01 00:00:00

  • A plea to overcome the concept of "staging" and related inadequacy in multiple myeloma.

    abstract::From a retrospective multivariate study on 107 multiple myeloma (MM) patients, serum beta 2-microglobulin (beta 2M) proved to be the best prognostic discriminator, better than each of the currently used staging systems (Durie and Salmon's [DS], Merlini, Waldenström and Jayakar's [MWJ] and the British Medical Research ...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1991.tb01273.x

    authors: Gobbi PG,Bertoloni D,Grignani G,Pieresca C,Rossi A,Rutigliano L,Merlini G,Riccardi A,Ascari E

    更新日期:1991-03-01 00:00:00

  • Circulating dendritic cells subsets and CD4+Foxp3+ regulatory T cells in adult patients with chronic ITP before and after treatment with high-dose dexamethasome.

    abstract::Immune thrombocytopenic purpura (ITP) is an autoimmune disorder, and high-dose dexamethasome (HD-DXM) has been used as a first-line therapy for patients with ITP. However, little is known about the role of dendritic cells (DCs) and CD4(+)Foxp3(+) regulatory T (Treg) cells in the pathogenesis of chronic ITP and the eff...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章

    doi:10.1111/j.1600-0609.2007.00917.x

    authors: Ling Y,Cao X,Yu Z,Ruan C

    更新日期:2007-10-01 00:00:00

  • Patient outcomes in light chain (AL) amyloidosis: The clock is ticking from symptoms to diagnosis.

    abstract:INTRODUCTION:Symptomology of AL amyloidosis can be vague, with a broad range of manifestations and potential etiologies. We sought to determine whether time from initial patient-reported symptom onset to diagnosis was associated with survival. METHODS:The Boston University Amyloidosis Patient Database was queried for ...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13472

    authors: Schulman A,Connors LH,Weinberg J,Mendelson LM,Joshi T,Shelton AC,Sanchorawala V

    更新日期:2020-10-01 00:00:00

  • Central nervous system myelomatosis: review of the literature.

    abstract::Involvement of the central nervous system (CNS) in multiple myeloma (MM) is very uncommon; it has been observed in approximately 1% of the MM patients. This review summarizes the clinical and laboratory characteristics and treatment modalities of 109 patients with CNS myelomatosis (CNS MM) reported in the literature. ...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1600-0609.2007.00956.x

    authors: Nieuwenhuizen L,Biesma DH

    更新日期:2008-01-01 00:00:00

  • Causes of hypereosinophilia in 100 consecutive patients.

    abstract:BACKGROUND:Hypereosinophilia (HE, persistent peripheral blood eosinophilia > 1.5 × 109 /L) and hypereosinophilic syndrome (HES, HE with end-organ damage) are classified as primary (due to a myeloid clone), secondary (due to a wide variety of reactive causes), or idiopathic. Diagnostic evaluation of eosinophilia is chal...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13437

    authors: Moller D,Tan J,Gauiran DTV,Medvedev N,Hudoba M,Carruthers MN,Dehghan N,van den Berghe J,Bruyère H,Chen LYC

    更新日期:2020-09-01 00:00:00

  • Porcine platelets in vitro and in vivo studies: relevance to human thrombosis research.

    abstract::This review summarizes present knowledge on porcine platelets in vitro and recent studies on in vivo activation of platelets in the pig. There are certain differences compared to human platelets: Platelet aggregation and secretion cannot be achieved by epinephrine, and the arachidonate pathway seems poorly developed i...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1600-0609.1992.tb00043.x

    authors: Søfteland E,Framstad T,Thorsen T,Holmsen H

    更新日期:1992-10-01 00:00:00

  • An unusual case of primary effusion lymphoma in a HIV-negative patient not pathogenetically associated with HHV8.

    abstract::The development of an unusual case of primary pleural effusion in a 90-year-old human immunodeficiency virus (HIV)-negative Japanese woman with no identifiable tumor mass has been described. Pleural effusion specimens contained large diffuse lymphoma cells, with the phenotype and genotype of a B-cell lineage (positive...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1034/j.1600-0609.2003.00083.x

    authors: Shimazaki M,Fujita M,Tsukamoto K,Matsuki T,Iwata M,Takahashi H,Doi A,Hyakkoku M,Yamauchi K,Genda S,Kikuiri T,Sakamoto T,Nojiri S,Ashie T

    更新日期:2003-07-01 00:00:00