Patterns of iron distribution in liver cells in beta-thalassemia studied by X-ray microanalysis.

Abstract:

BACKGROUND AND OBJECTIVES:beta-thalassemia is an important public health problem in the countries bordering the Mediterranean sea. One of the major consequences of this disorder, primarily (due to an ineffective erythropoiesis) or secondarily to blood transfusions (which are necessary for the patient's survival), is iron storage. Applying X-ray microanalysis we wanted to demonstrate the different sites of iron storage in subcellular compartments (mitochondria, cytosol, nucleus, rough endoplasmic reticulum and lipid droplets) and whether there were any other trace elements stored in liver cell. DESIGN AND METHODS:X-ray microanalysis was performed (at 100 kV in the STEM mode of a Hitachi H7000) on thin sections from specimens of liver biopsies from 6 patients affected by b-thalassemia, during follow-up after bone marrow transplantation. RESULTS:Spectra showed no correlation between iron peaks of lysosomes and hepatic iron concentration (HIC) or serum ferritin levels. Iron peaks were also detected in other subcellular compartments such as cisternae of rough endoplasmic reticulum, mitochondria and cytosol. No iron peaks were detected in lipid droplets and no significant iron peaks were found in the nuclei. Traces of copper were almost constantly found in lysosomes and cytosol. INTERPRETATION AND CONCLUSIONS:These results demonstrated iron storage within subcellular organelles other than lysosomes and highlighted a non-correlation between lysosomal iron peaks and HIC or serum ferritin levels. The presence of traces of copper in the lysosomes and in the cytosol may be correlated with the stronger hypothesis of links in the metabolism of the two elements (iron and copper), as ceruloplasmin is a ferroxidase copper-dependent protein. X-ray microanalysis may become a relevant tool in the localization of iron storage within hepatocytes in the evaluation of the effectiveness of bone marrow transplantation and iron chelation therapy. It also may provide some interesting information about iron metabolism in hepatocytes.

journal_name

Haematologica

journal_title

Haematologica

authors

Faa G,Terlizzo M,Gerosa C,Congiu T,Angelucci E

keywords:

subject

Has Abstract

pub_date

2002-05-01 00:00:00

pages

479-84

issue

5

eissn

0390-6078

issn

1592-8721

journal_volume

87

pub_type

杂志文章
  • Cholesterol embolism of bone marrow clinically masquerading as systemic or metastatic tumor.

    abstract::A case of cholesterol embolism of bone marrow, concerning the pelvis and lumbar region and clinically masquerading as systemic disease or metastatic tumor, is reported in an 82-year-old man hospitalized for acute onset of reddish purple nodules on the legs and toes, intense myalgia and dorsal vertebral bone pain. The ...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:

    authors: Muretto P,Carnevali A,Ansini AL

    更新日期:1991-05-01 00:00:00

  • Impact on survival through consolidation radiotherapy for diffuse large B-cell lymphoma: a comprehensive meta-analysis.

    abstract::Rituximab has improved response rates and overall survival in B-cell lymphoma (DLBCL). Radiotherapy is an effective treatment modality for lymphomas, but there is uncertainty on its use as consolidation after chemo-immunotherapy mainly in advanced stages. We evaluated its efficacy with a comprehensive meta-analysis an...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2020.249680

    authors: Berger MD,Trelle S,Büchi AE,Jegerlehner S,Ionescu C,Lamy de la Chapelle T,Novak U

    更新日期:2020-06-18 00:00:00

  • Chromosomal alterations detected by comparative genomic hybridization in subgroups of gene expression-defined Burkitt's lymphoma.

    abstract:BACKGROUND:Burkitt's lymphoma is an aggressive B-cell lymphoma characterized by typical morphological, immunophenotypic and molecular features. Gene expression profiling provided a molecular signature of Burkitt's lymphoma, but also demonstrated that a subset of aggressive B-cell lymphomas not fulfilling the current Wo...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.13071

    authors: Salaverria I,Zettl A,Beà S,Hartmann EM,Dave SS,Wright GW,Boerma EJ,Kluin PM,Ott G,Chan WC,Weisenburger DD,Lopez-Guillermo A,Gascoyne RD,Delabie J,Rimsza LM,Braziel RM,Jaffe ES,Staudt LM,Müller-Hermelink HK,Campo E,

    更新日期:2008-09-01 00:00:00

  • Quantitatively different red cell/nucleated cell chimerism in patients with long-term, persistent hematopoietic mixed chimerism after bone marrow transplantation for thalassemia major or sickle cell disease.

    abstract:BACKGROUND:Persistent mixed chimerism represents a state in which recipient and donor cells stably co-exist after hematopoietic stem cell transplantation. However, since in most of the studies reported in literature the engraftment state was observed in the nucleated cells, in this study we determined the donor origin ...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2010.031013

    authors: Andreani M,Testi M,Gaziev J,Condello R,Bontadini A,Tazzari PL,Ricci F,De Felice L,Agostini F,Fraboni D,Ferrari G,Battarra M,Troiano M,Sodani P,Lucarelli G

    更新日期:2011-01-01 00:00:00

  • Natural killer cell lines preferentially kill clonogenic multiple myeloma cells and decrease myeloma engraftment in a bioluminescent xenograft mouse model.

    abstract:BACKGROUND:Novel therapies capable of targeting drug resistant clonogenic MM cells are required for more effective treatment of multiple myeloma. This study investigates the cytotoxicity of natural killer cell lines against bulk and clonogenic multiple myeloma and evaluates the tumor burden after NK cell therapy in a b...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2011.054254

    authors: Swift BE,Williams BA,Kosaka Y,Wang XH,Medin JA,Viswanathan S,Martinez-Lopez J,Keating A

    更新日期:2012-07-01 00:00:00

  • Clinical practice recommendation on hematopoietic stem cell transplantation for acute myeloid leukemia patients with FLT3-internal tandem duplication: a position statement from the Acute Leukemia Working Party of the European Society for Blood and Marrow

    abstract::The FMS-like tyrosine kinase 3 (FLT3) gene is mutated in 25-30% of patients with acute myeloid leukemia (AML). Because of the poor prognosis associated with FLT3-internal tandem duplication mutated AML, allogeneic hematopoietic stem-cell transplantation (SCT) was commonly performed in first complete remission. Remarka...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2019.243410

    authors: Bazarbachi A,Bug G,Baron F,Brissot E,Ciceri F,Dalle IA,Döhner H,Esteve J,Floisand Y,Giebel S,Gilleece M,Gorin NC,Jabbour E,Aljurf M,Kantarjian H,Kharfan-Dabaja M,Labopin M,Lanza F,Malard F,Peric Z,Prebet T,Ravan

    更新日期:2020-06-01 00:00:00

  • Multiplex reverse transcription polymerase chain reaction screening in acute myeloid leukemia detects cytogenetically unrevealed abnormalities of prognostic significance.

    abstract::A commercial multiplex reverse transcription polymerase chain reaction screening assay, covering 28 leukemic fusion transcripts, was applied in 143 samples obtained from patients with acute myeloid leukemia at primary diagnosis. In five patients, a cytogenetically unrevealed fusion gene of prognostic importance was de...

    journal_title:Haematologica

    pub_type: 信件

    doi:

    authors: Hutchings Hoffmann M,Wirenfeldt Klausen T,Hasle H,Schmiegelow K,Brondum-Nielsen K,Johnsen HE

    更新日期:2005-07-01 00:00:00

  • Heme controls ferroportin1 (FPN1) transcription involving Bach1, Nrf2 and a MARE/ARE sequence motif at position -7007 of the FPN1 promoter.

    abstract:BACKGROUND:Macrophages of the reticuloendothelial system play a key role in recycling iron from hemoglobin of senescent or damaged erythrocytes. Heme oxygenase 1 degrades the heme moiety and releases inorganic iron that is stored in ferritin or exported to the plasma via the iron export protein ferroportin. In the plas...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2009.020123

    authors: Marro S,Chiabrando D,Messana E,Stolte J,Turco E,Tolosano E,Muckenthaler MU

    更新日期:2010-08-01 00:00:00

  • Mitoxantrone, etoposide, cisplatin and dexamethasone (MEPD) as salvage chemotherapy in resistant non-Hodgkin's lymphoma.

    abstract:BACKGROUND:An effective second-line treatment for intermediate and high grade non-Hodgkin's lymphoma is greatly needed since 30% of patients do not achieved complete remission (CR) and another 20% to 30% of the CRs will eventually relapse. METHODS:A four-drug combination with Mitoxantrone, Etoposide, Cisplatin and Dex...

    journal_title:Haematologica

    pub_type: 临床试验,杂志文章

    doi:

    authors: Vitolo U,Orsucci L,Bertini M,Cavallero G,Gallamini A,Ghio R,Levis A,Rota-Scalabrini D,Resegotti L

    更新日期:1991-01-01 00:00:00

  • Verification and comparison of two different predictive equations in Hodgkin's disease.

    abstract:BACKGROUND AND OBJECTIVE:In recent years, two predictive equations to estimate median expected survival at diagnosis for patients affected with Hodgkin's disease have been developed at the University of Pavia Medical School. The present retrospective work was aimed at testing correlation between mean survival estimated...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:

    authors: Bettini R,Tonolini M,Maccianti E

    更新日期:1997-05-01 00:00:00

  • Non-myeloablative conditioning with allogeneic hematopoietic cell transplantation for the treatment of high-risk acute lymphoblastic leukemia.

    abstract:BACKGROUND:Allogeneic hematopoietic cell transplantation is a potentially curative treatment for patients with acute lymphoblastic leukemia. However, the majority of older adults with acute lymphoblastic leukemia are not candidates for myeloablative conditioning regimens. A non-myeloablative preparative regimen is a re...

    journal_title:Haematologica

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.3324/haematol.2011.040261

    authors: Ram R,Storb R,Sandmaier BM,Maloney DG,Woolfrey A,Flowers ME,Maris MB,Laport GG,Chauncey TR,Lange T,Langston AA,Storer B,Georges GE

    更新日期:2011-08-01 00:00:00

  • Protein arginine methyltransferase 6 controls erythroid gene expression and differentiation of human CD34+ progenitor cells.

    abstract::Hematopoietic differentiation is driven by transcription factors, which orchestrate a finely tuned transcriptional network. At bipotential branching points lineage decisions are made, where key transcription factors initiate cell type-specific gene expression programs. These programs are stabilized by the epigenetic a...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2017.174516

    authors: Herkt SC,Kuvardina ON,Herglotz J,Schneider L,Meyer A,Pommerenke C,Salinas-Riester G,Seifried E,Bonig H,Lausen J

    更新日期:2018-01-01 00:00:00

  • Interferon-alpha 2b is not effective in the treatment of refractory immune thrombocytopenic purpura.

    abstract::About 25-30% of patients with immune thrombocytopenic purpura (ITP) are refractory to corticosteroids, splenectomy and other treatments. It has been suggested that interferon-alpha 2b (IFN-alpha 2b) may be useful in the treatment of chronic refractory ITP patients. We treated 9 chronic refractory ITP patients with IFN...

    journal_title:Haematologica

    pub_type: 信件

    doi:

    authors: Vianelli N,Tazzari PL,Baravelli S,Ricci F,Valdrè L,Tura S

    更新日期:1998-08-01 00:00:00

  • A cooperative study of epirubicin with cyclophosphamide, vincristine and prednisone (CEOP) in non-Hodgkin's lymphoma.

    abstract:BACKGROUND:The purpose of our cooperative trial was to investigate whether epirubicin (EPI) at 90 mg/m2 in a CHOP-like combination (called CEOP) could increase complete response (CR) and survival rates in non-Hodgkin lymphoma (NHL) patients while maintaining a tolerable degree of toxicity. METHODS:Between September 19...

    journal_title:Haematologica

    pub_type: 临床试验,杂志文章,多中心研究

    doi:

    authors: Lambertenghi Deliliers G,Butti C,Baldini L,Ceriani A,Lombardi F,Luoni M,Montalbetti L,Pavia G,Pinotti G,Pogliani E

    更新日期:1995-07-01 00:00:00

  • Recovery of cord blood hematopoietic progenitors after successive freezing and thawing procedures.

    abstract:BACKGROUND AND OBJECTIVES:Cord blood (CB) is a valuable source of stem cells. Most CB units are still cryopreserved in single bags in the world's CB banks. Thawing a single CB unit, dividing it into two parts, expanding the smaller one and refreezing the other would optimize ex vivo expansion of CB progenitors prior to...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:

    authors: Timeus F,Crescenzio N,Saracco P,Doria A,Fazio L,Albiani R,Cordero Di Montezemolo L,Perugini L,Incarbone E

    更新日期:2003-01-01 00:00:00

  • MicroRNA-125b transforms myeloid cell lines by repressing multiple mRNA.

    abstract:BACKGROUND:We previously described a t(2;11)(p21;q23) chromosomal translocation found in patients with myelodysplasia or acute myeloid leukemia that leads to over-expression of the microRNA miR-125b, and we showed that transplantation of mice with murine stem/progenitor cells overexpressing miR-125b is able to induce l...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2011.061515

    authors: Bousquet M,Nguyen D,Chen C,Shields L,Lodish HF

    更新日期:2012-11-01 00:00:00

  • Phase I study of the aurora A kinase inhibitor alisertib with induction chemotherapy in patients with acute myeloid leukemia.

    abstract::Aberrant expression of aurora kinase A is implicated in the genesis of various neoplasms, including acute myeloid leukemia. Alisertib, an aurora A kinase inhibitor, has demonstrated efficacy as monotherapy in trials of myeloid malignancy, and this efficacy appears enhanced in combination with conventional chemotherapi...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2016.158394

    authors: Fathi AT,Wander SA,Blonquist TM,Brunner AM,Amrein PC,Supko J,Hermance NM,Manning AL,Sadrzadeh H,Ballen KK,Attar EC,Graubert TA,Hobbs G,Joseph C,Perry AM,Burke M,Silver R,Foster J,Bergeron M,Ramos AY,Som TT,Fishm

    更新日期:2017-04-01 00:00:00

  • AML1 gene amplification: a novel finding in childhood acute lymphoblastic leukemia.

    abstract:BACKGROUND AND OBJECTIVE:We previously found a high-level amplification in chromosomal region 21q22 in two children with acute lymphoblastic leukemia (ALL) using comparative genomic hybridization. The same region harbors the AML1 gene. The aim of the present study was to investigate whether AML1 is a target gene in the...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:

    authors: Niini T,Kanerva J,Vettenranta K,Saarinen-Pihkala UM,Knuutila S

    更新日期:2000-04-01 00:00:00

  • Frequency and distribution of megakaryoblasts in FAB subtypes of ANLL: an immunocytochemical ultrastructural study.

    abstract:BACKGROUND:Identification of early megakaryocyte (MK) precursors is difficult and conflicting. The prevalence of leukemias with a megakaryocytic component may be underestimated when conventional techniques are utilized. The recent development of immuno-ultrastructural procedures for simultaneous detection of platelet p...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:

    authors: Tomasi P,Tallarico A,Vasi V,Scapoli G,Castoldi G

    更新日期:1993-03-01 00:00:00

  • Genome-wide profiling identifies a DNA methylation signature that associates with TET2 mutations in diffuse large B-cell lymphoma.

    abstract::The discovery that the Ten-Eleven Translocation (TET) hydroxylases cause DNA demethylation has fundamentally changed the notion of how DNA methylation is regulated. Clonal analysis of the hematopoetic stem cell compartment suggests that TET2 mutations can be early events in hematologic cancers and recent investigation...

    journal_title:Haematologica

    pub_type: 杂志文章,随机对照试验

    doi:10.3324/haematol.2013.088740

    authors: Asmar F,Punj V,Christensen J,Pedersen MT,Pedersen A,Nielsen AB,Hother C,Ralfkiaer U,Brown P,Ralfkiaer E,Helin K,Grønbæk K

    更新日期:2013-12-01 00:00:00

  • Red blood cell metabolism in Rhesus macaques and humans: comparative biology of blood storage.

    abstract::Macaques are emerging as a critical animal model in transfusion medicine, because of their evolutionary similarity to humans and perceived utility in discovery and translational science. However, little is known about the metabolism of Rhesus macaque red blood cells (RBC) and how this compares to human RBC metabolism ...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2019.229930

    authors: Stefanoni D,Shin HKH,Baek JH,Champagne DP,Nemkov T,Thomas T,Francis RO,Zimring JC,Yoshida T,Reisz JA,Spitalnik SL,Buehler PW,D'Alessandro A

    更新日期:2020-08-01 00:00:00

  • Fatal Epstein-Barr virus-associated lymphoproliferative disorder following treatment with a novel mTOR inhibitor for relapsed chronic lymphocytic leukemia leukemia cells.

    abstract::We report on a patient with relapsed chronic lymphocytic leukemia (CLL) treated with the novel mTOR inhibitor RAD001 within a phase II clinical trial. Although the patient initially responded to therapy, RAD001 was discontinued after 32 weeks due to progression and fludarabine-based chemotherapy was started. The patie...

    journal_title:Haematologica

    pub_type: 信件

    doi:10.3324/haematol.11155

    authors: Götze KS,Hoffmann D,Schätzl HM,Peschel C,Fend F,Decker T

    更新日期:2007-09-01 00:00:00

  • Combined modality therapy in advanced Hodgkin's disease: a report on 218 patients with a median follow-up of eight years.

    abstract:BACKGROUND AND OBJECTIVE:This study was designed to evaluate the efficacy and toxicity of monthly alternating ABVD/MOPP compared to ABVD/OPP regimens in patients with advanced stage Hodgkin's disease (HD), as well as in early stage patients with systemic symptoms and/or bulky disease. DESIGN AND METHODS:218 patients w...

    journal_title:Haematologica

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:

    authors: Anselmo AP,Cavalieri E,Enrici RM,Donato V,Pescarmona E,Biagini C,Mandelli F

    更新日期:1998-07-01 00:00:00

  • Pulmonary hypertension in POEMS syndrome.

    abstract::POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. We reviewed records of 154 POEMS syndrome patients with complete duplex echocardiography data for estimation of pulmonary ...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2012.073031

    authors: Li J,Tian Z,Zheng HY,Zhang W,Duan MH,Liu YT,Cao XX,Zhou DB

    更新日期:2013-03-01 00:00:00

  • Trypsin-encoding PRSS1-PRSS2 variations influence the risk of asparaginase-associated pancreatitis in children with acute lymphoblastic leukemia: a Ponte di Legno toxicity working group report.

    abstract::Asparaginase-associated pancreatitis is a life-threatening toxicity to childhood acute lymphoblastic leukemia treatment. To elucidate genetic predisposition and asparaginase-associated pancreatitis pathogenesis, ten trial groups contributed remission samples from patients aged 1.0-17.9 years treated for acute lymphobl...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2018.199356

    authors: Wolthers BO,Frandsen TL,Patel CJ,Abaji R,Attarbaschi A,Barzilai S,Colombini A,Escherich G,Grosjean M,Krajinovic M,Larsen E,Liang DC,Möricke A,Rasmussen KK,Samarasinghe S,Silverman LB,van der Sluis IM,Stanulla M,Tulstr

    更新日期:2019-03-01 00:00:00

  • Senescence-accelerated mice (SAMP1/TA-1) treated repeatedly with lipopolysaccharide develop a condition that resembles hemophagocytic lymphohistiocytosis.

    abstract::Hemophagocytic lymphohistiocytosis is a life-threatening systemic hyperinflammatory disorder with primary and secondary forms. Primary hemophagocytic lymphohistiocytosis is associated with inherited defects in various genes that affect the immunological cytolytic pathway. Secondary hemophagocytic lymphohistiocytosis i...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2018.209551

    authors: Tsuboi I,Harada T,Hirabayashi Y,Aizawa S

    更新日期:2019-10-01 00:00:00

  • Recovery of polyclonal immunoglobulins one year after autologous stem cell transplantation as a long-term predictor marker of progression and survival in multiple myeloma.

    abstract::Immunoparesis or suppression of polyclonal immunoglobulins is a very common condition in newly diagnosed myeloma patients. However, the recovery of polyclonal immunoglobulins in the setting of immune reconstitution after autologous stem cell transplantation and its effect on outcome has not yet been explored. We condu...

    journal_title:Haematologica

    pub_type: 杂志文章,多中心研究

    doi:10.3324/haematol.2016.158345

    authors: González-Calle V,Cerdá S,Labrador J,Sobejano E,González-Mena B,Aguilera C,Ocio EM,Vidriales MB,Puig N,Gutiérrez NC,García-Sanz R,Alonso JM,López R,Aguilar C,de Coca AG,Hernández R,Hernández JM,Escalante F,Mateos MV

    更新日期:2017-05-01 00:00:00

  • Erythroid marrow activity and functional anemia in patients with the rare interaction of a single functional a-globin and beta-globin gene.

    abstract:BACKGROUND AND OBJECTIVES:The degree of globin chain imbalance and tissue hypoxia are important determinants of clinical severity in thalassemia syndromes. Thus phenotypic expression may be modified by interaction of alpha- and beta-thalassemia defects, level and type of hemoglobin synthesized and oxygen release to the...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:

    authors: Traeger-Synodinos J,Papassotiriou I,Vrettou C,Skarmoutsou C,Stamoulakatou A,Kanavakis E

    更新日期:2001-04-01 00:00:00

  • Megakaryocyte volume modulates bone marrow niche properties and cell migration dynamics.

    abstract::All hematopoietic cells that develop in the bone marrow must cross the endothelial barrier to enter the blood circulation. Blood platelets, however, are released by bigger protrusions of huge progenitor cells, named megakaryocytes, and enter the blood stream as so-called proplatelets before fragmenting into mature pla...

    journal_title:Haematologica

    pub_type: 杂志文章

    doi:10.3324/haematol.2018.202010

    authors: Gorelashvili MG,Angay O,Hemmen K,Klaus V,Stegner D,Heinze KG

    更新日期:2020-04-01 00:00:00

  • Detection of recombinant human erythropoietin abuse in athletes utilizing markers of altered erythropoiesis.

    abstract:BACKGROUND AND OBJECTIVES:The detection of recombinant human erythropoietin (r-HuEPO) abuse by athletes remains problematic. The main aim of this study was to demonstrate that the five indirect markers of altered erythropoiesis identified in our earlier work were reliable evidence of current or recently discontinued r-...

    journal_title:Haematologica

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验

    doi:

    authors: Parisotto R,Wu M,Ashenden MJ,Emslie KR,Gore CJ,Howe C,Kazlauskas R,Sharpe K,Trout GJ,Xie M

    更新日期:2001-02-01 00:00:00