Four-year evaluation of myocardial and liver iron assessed prospectively with serial MRI scans in young patients with beta-thalassaemia major: comparison between different chelation regimens.

Abstract:

:This study was conducted in order to assess myocardial and liver iron concentrations (LICs) using serial magnetic resonance imaging (MRI) scans in patients with beta-thalassaemia major, over a 4-yr period, and consequently to compare the effectiveness of different chelation regimens. Fifty children and young adults with beta-thalassaemia major (27 boys and 23 girls) were recruited (mean age: 14.74 +/- 3.67 yr). All patients underwent detailed clinical examination, electrocardiography, echocardiography, myocardial and liver MRI at the beginning of the study, 2 and 4 yr after. Additionally, serum ferritin levels were regularly measured and data regarding LICs assessed by percutaneous liver biopsy were available in 26 patients. Both myocardial and liver MRI values showed a moderate inverse correlation with age (r = -0.379, P < 0.001 and r = -0.376, P < 0.001, respectively). Liver MRI was better correlated with serum ferritin concentrations (r = -0.342, P < 0.001) than myocardial MRI (r = -0.186, P = 0.011). Liver MRI values were highly correlated with LICs derived from percutaneous liver biopsy (r = -0.863, P < 0.001), whereas myocardial MRI values did not correlate at all with measurements derived from echocardiography. Regarding iron chelation treatment, patients receiving combined therapy with deferiprone and deferoxamine (DFO) significantly reduced myocardial iron overload during the 4-yr study period, whilst patients in monotherapy with DFO showed a significant increase in LIC.

journal_name

Eur J Haematol

authors

Christoforidis A,Haritandi A,Tsatra I,Tsitourides I,Karyda S,Athanassiou-Metaxa M

doi

10.1111/j.0902-4441.2006.t01-1-EJH3013.x

subject

Has Abstract

pub_date

2007-01-01 00:00:00

pages

52-7

issue

1

eissn

0902-4441

issn

1600-0609

pii

EJH768

journal_volume

78

pub_type

临床试验,杂志文章
  • ZAP-70 mRNA quantification in B-cell chronic lymphocytic leukaemia.

    abstract:OBJECTIVE:The mutational status of the immunoglobulin (Ig) V(H) gene in B-cell chronic lymphocytic leukaemia (B-CLL) identifies two subgroups of patients with significantly different outcomes. We investigated the association of ZAP-70 expression with IgVH mutational status in B-CLL by quantifying ZAP-70 mRNA, to evalua...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章

    doi:10.1111/j.1600-0609.2005.00619.x

    authors: Catherwood MA,Matthews C,Niblock R,Dobbin E,Morris TC,Alexander HD

    更新日期:2006-04-01 00:00:00

  • Challenges of multiple mutations in individual patients with haemophilia.

    abstract::Multiple mutations in the same gene within a haemophilia family are being increasingly reported and raise many issues with regard to the specificity of the mutations in causing the disease. In a proportion of families with multiple mutations, discordant phenotypic severity is often observed among the affected members....

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1600-0609.2010.01564.x

    authors: Shetty S,Bhave M,Ghosh K

    更新日期:2011-03-01 00:00:00

  • Multicenter validation of a simplified method for paroxysmal nocturnal hemoglobinuria screening.

    abstract:BACKGROUND:Paroxysmal nocturnal hemoglobinuria (PNH) diagnostic guidelines recommend single-tube five- to six-color or two-tube four-color assays. PNH clones are detectable in only a fraction of patients at risk, and screening for new PNH cases can be complex and expensive. In this multicenter study, we have validated ...

    journal_title:European journal of haematology

    pub_type: 杂志文章,多中心研究

    doi:10.1111/ejh.12885

    authors: Gatti A,Del Vecchio L,Geuna M,Della Porta MG,Brando B

    更新日期:2017-07-01 00:00:00

  • Expression of Foxo3a in non-Hodgkin's lymphomas is correlated with cell cycle inhibitor p27.

    abstract:OBJECTIVE:Cell cycle arrest by FOXO transcription factors involves in transcriptional activation of p27(kip1), although the exact mechanism remains unclear. And it has been evidenced that reduced level of p27(kip1) which is frequently occurred in human cancers has been associated with poor prognosis. In this study, our...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2008.01077.x

    authors: Zhao Y,Fei M,Wang Y,Lu M,Cheng C,Shen A

    更新日期:2008-08-01 00:00:00

  • A new exon 12 mutation in the EPAS1 gene possibly associated with erythrocytosis.

    abstract::In secondary erythrocytosis, the elevated red cell count is powered by factors outside the erythroid compartment, for instance by raised erythropoietin (EPO) synthesis based on congenital defects of the oxygen-sensing pathway. The principal transcriptional regulator of EPO synthesis is endothelial PAS domain-containin...

    journal_title:European journal of haematology

    pub_type:

    doi:10.1111/ejh.13241

    authors: Schelker RC,Herr W,Grassinger J

    更新日期:2019-07-01 00:00:00

  • Anagrelide influences thrombotic risk, and prolongs progression-free and overall survival in essential thrombocythaemia vs hydroxyurea plus aspirin.

    abstract:OBJECTIVE:We report an extension study of patients with essential thrombocythaemia (ET) in the Hungarian Myeloproliferative Neoplasm (HUMYPRON) Registry, which demonstrated that over 6 years anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombotic events (TEs) vs...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13459

    authors: Kellner A,Dombi P,Illes A,Demeter J,Homor L,Ercsei I,Simon Z,Karadi E,Herczeg J,Gy Korom V,Gasztonyi Z,Szerafin L,Udvardy M,Egyed M

    更新日期:2020-10-01 00:00:00

  • The clinical and biological features of a series of immunophenotypic variant of B-CLL.

    abstract:OBJECTIVES:To describe the clinical and biological features of a series of immunophenotypic variant of B-CLL (v-CLL) characterised by intermediate RMH score, in the absence of t(11;14)(q13;q32) in FISH analysis in comparison with a series of typical CLL. METHODS:We studied the clinical and biological features of 63 ca...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2010.01454.x

    authors: Cro L,Ferrario A,Lionetti M,Bertoni F,Zucal N N,Nobili L,Fabris S,Todoerti K,Cortelezzi A,Guffanti A,Goldaniga M,Marcheselli L,Neri A,Lambertenghi-Deliliers G,Baldini L

    更新日期:2010-08-01 00:00:00

  • Pirarubicin nuclear uptake does not correlate with its induced cell death effect during reversal of multidrug resistance by quinine in human K562 and CEM leukemic cells.

    abstract::A number of small and lipophilic cations are able to reverse in vitro the resistance to anthracyclines and other natural products through their interaction with P-glycoprotein or P-gp. However, some modulators do not interact with P-gp. We have demonstrated in a previous a work, using confocal laser microspectrofluoro...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1998.tb01709.x

    authors: Morjani H,Belhoussine R,Lahlil R,Manfait M

    更新日期:1998-10-01 00:00:00

  • Effect of splenectomy on iron balance in patients with β-thalassemia major: a long-term follow-up.

    abstract:OBJECTIVE:A retrospective study was performed to explore the effect of splenectomy on iron balance in thalassemia major (TM). METHODS:Twenty two TM patients treated with splenectomy were compared with a control group (non-splenectomized patients) matched for sex, age, pretransfusional Hb, chelation therapy, and durati...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.12121

    authors: Casale M,Cinque P,Ricchi P,Costantini S,Spasiano A,Prossomariti L,Minelli S,Frega V,Filosa A

    更新日期:2013-07-01 00:00:00

  • No evidence for myocardial iron overload and free iron species in multitransfused patients with sickle/beta-thalassaemia.

    abstract::Iron overload (IO) in the heart is a life-threatening complication in transfusion-dependent patients with thalassaemia major (TM) and to a lesser extent in sickle cell disease (SCD), while no data are available in patients with sickle/beta(0)-thalassaemia. Iron deposition in the heart, liver and pancreas was assessed ...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2009.01355.x

    authors: Ghoti H,Goitein O,Koren A,Levin C,Kushnir T,Rachmilewitz E,Konen E

    更新日期:2010-01-01 00:00:00

  • Differences in treatment and monitoring of chronic myeloid leukemia with regard to age, but not sex: Results from a population-based study.

    abstract::There are established guidelines for treatment and monitoring of chronic myeloid leukemia (CML) but little is known about routine care. Data on ICD-10 codes as well as prescribed medications were available for 10.5 million patients in the statutory health insurance system in Bavaria for the years 2010 to 2016. Also, d...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13293

    authors: Lauseker M,Gerlach R,Worseg W,Haferlach T,Tauscher M,Hasford J,Hoffmann VS

    更新日期:2019-10-01 00:00:00

  • High rate of hematological responses to sorafenib in FLT3-ITD acute myeloid leukemia relapsed after allogeneic hematopoietic stem cell transplantation.

    abstract::Relapse represents the most significant cause of failure of allogeneic hematopoietic stem cell transplantation (HSCT) for FLT3-ITD-positive acute myeloid leukemia (AML), and available therapies are largely unsatisfactory. In this study, we retrospectively collected data on the off-label use of the tyrosine kinase inhi...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.12647

    authors: De Freitas T,Marktel S,Piemontese S,Carrabba MG,Tresoldi C,Messina C,Lupo Stanghellini MT,Assanelli A,Corti C,Bernardi M,Peccatori J,Vago L,Ciceri F

    更新日期:2016-06-01 00:00:00

  • Biochemical characterization of size-separated human red blood cells.

    abstract::Human red blood cells (RBC) are heterogeneous with respect to their size; the physiological significance of this heterogeneity has not yet been fully elucidated. To further investigate this problem, some characteristics of human RBC fractionated according to their mean corpuscular volume (MCV) by counterflow centrifug...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1992.tb00570.x

    authors: Vaysse J,Gattegno L,Pilardeau P

    更新日期:1992-02-01 00:00:00

  • Can t(8;21) oligoblastic leukemia be called a myelodysplastic syndrome?

    abstract::The new World Health Organization (WHO) classification of hematologic malignancies has incorporated t(8;21) myelodysplastic syndromes (MDS) according to the French-American-British classification into the category of acute myeloid leukemia (AML) with t(8;21)(q22;q22), while our knowledge about clinicopathological feat...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1034/j.1600-0609.2002.02770.x

    authors: Kaneda K,Kojima K,Takeuchi M,Takimoto H,Takaba S,Shinagawa K,Kobayashi H,Miyata A,Hara M,Tanimoto M

    更新日期:2002-09-01 00:00:00

  • Serum leptin levels in patients with idiopathic thrombocytopenic purpura.

    abstract:OBJECTIVES:The purpose of this study was to evaluate serum leptin levels in idiopathic thrombocytopenic purpura (ITP), in order to determine the influence of leptin on the pathogenesis of ITP. SUBJECTS AND METHODS:Forty-six untreated patients with chronic ITP were compared with 40 healthy people of similar age, sex an...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2004.00231.x

    authors: Zhan M,Zhao H,Yang R,Han ZC

    更新日期:2004-05-01 00:00:00

  • Drug-induced skin reactions in patients with acute non-lymphocytic leukaemia.

    abstract::The incidence of drug-induced skin rashes and related factors were analysed in a retrospective study of 151 patients with acute non-lymphocytic leukaemia (ANLL). 91 (60%) developed a drug-related toxicodermia to one or more drugs during remission, induction and maintenance therapy. The incidence of rashes was mainly c...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1987.tb01168.x

    authors: Verhagen C,Stalpers LJ,de Pauw BE,Haanen C

    更新日期:1987-03-01 00:00:00

  • Feasibility of fludarabine added to VAD during induction therapy in multiple myeloma: a randomised phase II-study.

    abstract::Multiple myeloma (MM) is considered to be an essentially incurable haematological malignant disease, probably because of the existence of resistant clonal precursor cell with self-renewal capacity. Recent data have indicated that the myeloma cell hierarchy includes circulating clonal memory B cells, which differ consi...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1034/j.1600-0609.2003.00068.x

    authors: Björkstrand B,Rasmussen T,Remes K,Gruber A,Pelliniemi TT,Johnsen HE

    更新日期:2003-06-01 00:00:00

  • The antiplatelet activity of Escherichia coli lipopolysaccharide is mediated through a nitric oxide/cyclic GMP pathway.

    abstract::In this study, Escherichia coli LPS dose-dependently (100-500 microg/ml) and time-dependently (10-60 min) inhibited platelet aggregation in human and rabbit platelets stimulated by agonists. LPS also dose-dependently inhibited the intracellular Ca2+ mobilization in human platelets stimulated by collagen. In addition, ...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1999.tb01909.x

    authors: Sheu JR,Hung WC,Su CH,Lin CH,Lee LW,Lee YM,Yen MH

    更新日期:1999-05-01 00:00:00

  • Prognostic significance of apoptotic index in multiple myeloma patients treated by conventional therapy and novel agents, thalidomide and bortezomib.

    abstract:OBJECTIVE:To assess the outcome of the measurement of apoptotic index in myeloma patients treated by conventional chemotherapy and novel drugs with biological mechanism of action, thalidomide and bortezomib. PATIENTS AND METHODS:In a cohort of 189 patients with newly diagnosed multiple myeloma from November 1997 throu...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2009.01321.x

    authors: Minarik J,Scudla V,Ordeltova M,Bacovsky J,Pika T,Langova K

    更新日期:2009-12-01 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

  • Persistent altered fusion transcript splicing identifies RUNX1-RUNX1T1+ AML patients likely to relapse.

    abstract::In acute myeloid leukemia (AML) mouse models, the RUNX1-RUNX1T1 fusion protein has failed to produce leukemia by itself, but alternative splicing of exon 9a of the RUNX1-RUNX1T1 fusion transcript (FT) has recently been shown to enhance the leukemogenic potential. We have analyzed 138 diagnosis and follow-up samples fr...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2009.01371.x

    authors: Ommen HB,Ostergaard M,Yan M,Braendstrup K,Zhang DE,Hokland P

    更新日期:2010-02-01 00:00:00

  • Evaluation of the prognostic relevance of L-selectin and ICAM1 expression in myelodysplastic syndromes.

    abstract:OBJECTIVES:An aberrant pattern of expression of L-selectin and intercellular adhesion molecule 1 (ICAM1) may characterise CD34+ blast cells in myelodysplastic syndromes (MDS) and secondary acute myeloid leukaemia (sAML). METHODS:In a three-colour flow cytometric assay, we evaluated the expression of L-selectin and ICA...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2007.00986.x

    authors: Buccisano F,Maurillo L,Tamburini A,Del Poeta G,Del Principe MI,Ammatuna E,Consalvo MI,Campagna S,Ottaviani L,Sarlo C,Renzi D,Faccia S,Fraboni D,Lo Coco F,Amadori S,Venditti A

    更新日期:2008-02-01 00:00:00

  • Evaluating prophylaxis of invasive fungal infections in patients with haematologic malignancies.

    abstract:OBJECTIVE:Patients with hematologic malignancies are at substantial risk of developing invasive fungal infections (IFI) that are associated with substantial morbidity and mortality. This article reviews the epidemiology, risk factors, and efficacy of antifungal prophylaxis in patients with hematologic malignancies. ME...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1600-0609.2006.00805.x

    authors: Maertens J

    更新日期:2007-04-01 00:00:00

  • Allogeneic hematopoietic stem cell transplantation following reduced-intensity conditioning regimen in children: a single-center experience.

    abstract::This single-center retrospective study reported the outcome of 19 children treated with a reduced-intensity conditioning (RIC) regimen prior to allogeneic stem cell transplantation (allo-SCT), for hematologic malignancies (n = 17), bone marrow failure (n = 1), and neuroblastoma (n = 1). Children were ineligible for st...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.2012.01776.x

    authors: Strullu M,Rialland F,Cahu X,Brissot E,Corradini N,Thomas C,Blin N,Rialland X,Méchinaud F,Mohty M

    更新日期:2012-06-01 00:00:00

  • Prevalence and risk factors for Pulmonary Hypertension associated with chronic Myeloproliferative Neoplasms.

    abstract:OBJECTIVES:Pulmonary hypertension (PH) is commonly reported in Philadelphia-chromosome negative myeloproliferative neoplasms (MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). PH may be diagnosed directly by right heart catheterization (RHC) or estimated by transthoracic ec...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13543

    authors: Ferrari A,Scandura J,Masciulli A,Krichevsky S,Gavazzi A,Barbui T

    更新日期:2021-02-01 00:00:00

  • An increase in basophils in a case of acute myelomonocytic leukaemia associated with marrow eosinophilia and inversion of chromosome 16.

    abstract::A patient with acute myelomonocytic leukaemia (M4 subtype) with pericentric inversion of chromosome 16, inv(16)(p13q22), and a marked basophilia is described. Results from transmission electron microscopy suggested that the basophils were immature. Clinically, although leukaemic cells had a tendency to infiltrate a nu...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1600-0609.1987.tb01456.x

    authors: Matsuura Y,Sato N,Kimura F,Shimomura S,Yamamoto K,Enomoto Y,Takatani O

    更新日期:1987-11-01 00:00:00

  • Immunological effects in patients with steroid-refractory graft-versus-host disease following treatment with basiliximab, a CD25 monoclonal antibody.

    abstract::Steroid-refractory graft-versus-host disease (GvHD) is a complication following an allogeneic stem cell transplantation with limited therapeutic options. Studies have shown a response in up to 80% of patients with this condition after treatment with the CD25 monoclonal antibody, basiliximab. Despite the good responses...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.12691

    authors: Chakupurakal G,García-Márquez MA,Shimabukuro-Vornhagen A,Theurich S,Holtick U,Hallek M,Scheid C,von Bergwelt-Baildon M

    更新日期:2016-08-01 00:00:00

  • Lenalidomide as maintenance treatment for patients with multiple myeloma after autologous stem cell transplantation: A pharmaco-economic assessment.

    abstract:OBJECTIVE:Autologous stem cell transplantation (ASCT) has improved progression-free survival (PFS) and overall survival in eligible patients with newly diagnosed multiple myeloma (NDMM); however, relapse occurs. Maintenance therapy with lenalidomide (Len-Mt) extends survival and delays relapse and the subsequent initia...

    journal_title:European journal of haematology

    pub_type: 杂志文章

    doi:10.1111/ejh.13497

    authors: Uyl-de Groot CA,Ramsden R,Lee D,Boersma J,Zweegman S,Dhanasiri S

    更新日期:2020-11-01 00:00:00

  • Socioeconomic burden of participation in clinical trials in patients with myeloproliferative neoplasms.

    abstract:OBJECTIVE:To determine the financial and psycho-social impact of participation in clinical trials of patients with BCR/ABL-negative myeloproliferative neoplasms (MPN). METHODS:An international, observational cross-sectional study was performed in 143 consecutive MPN patients (51% myelofibrosis, 36% polycythemia vera, ...

    journal_title:European journal of haematology

    pub_type: 杂志文章,多中心研究

    doi:10.1111/ejh.12887

    authors: Goel S,Paoli C,Iurlo A,Pereira A,Efficace F,Barbui T,Tefferi A,Vannucchi AM,Cervantes F

    更新日期:2017-07-01 00:00:00

  • Genetic variations in multiple myeloma I: effect on risk of multiple myeloma.

    abstract::Few risk factors have been established for the plasma cell disorder multiple myeloma, but some of these like African American ethnicity and a family history of B-cell lymphoproliferative diseases suggest a genetic component for the disease. Genetic variation represents the genetic basis of variability in a population....

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1600-0609.2011.01700.x

    authors: Vangsted A,Klausen TW,Vogel U

    更新日期:2012-01-01 00:00:00