Abstract:
BACKGROUND AND OBJECTIVES:Myeloid leukemia of Down's syndrome (ML-DS) has characteristic biological features (e.g. expression of the truncated GATA1s), which are different from those of non-DS childhood acute myeloid leukemias (AML). The objective of this study was to investigate factors predisposing to the development of ML-DS. DESIGN AND METHODS:We analyzed 134 bone marrow specimens from 64 children with ML-DS and non-DS AML during chemotherapy and 7 specimens from DS children with- out leukemia,who did not receive any chemotherapy,The specimens were analyzed by multiparameter flow cytometry and quantitative reverse transcriptase polymerase chain reaction for transcription factors involved in hematopoiesis. RESULTS:Samples taken from children with ML-DS in complete remission during chemotherapy aberrantly expressed CD56 (NCAM) at the surface of monocytic and granulocytic cells. Compared to non-DS AML cases,children with ML-DS had a statistically significant higher proportion of CD56+ cells in the CD33+ fraction: 71%+/-6% vs. 4%+/-1% (p<0.00001). A significant decrease of the amount of CD33+/CD56+ cells was observed during and after maintenance therapy. An increased number of CD33+/CD56+ cells was also present (>85%) in children with DS who did not receive chemotherapy, but showed a left-shift (due to infection), compared with DS children without left-shift (<10% CD33+/CD56+ cells). Within the CD33+/CD56+ fraction, RUNX1 was overexpressed more than 5-fold (p<0.02) compared to CD33+/CD56- cells, whereas there were no differences regarding GATA1, SPI1, ERG or ETS-2 levels. INTERPRETATION AND CONCLUSIONS:The combined overexpression of RUNX1 and NCAM during stress hematopoiesis in children with DS might be a key factor in the development of overt leukemia and/or in the growth advantage of the malignant GATA1s clone in ML- DS.
journal_name
Haematologicajournal_title
Haematologicaauthors
Langebrake C,Klusmann JH,Wortmann K,Kolar M,Puhlmann U,Reinhardt Dsubject
Has Abstractpub_date
2006-11-01 00:00:00pages
1473-80issue
11eissn
0390-6078issn
1592-8721pii
03906078_10293journal_volume
91pub_type
杂志文章相关文献
HAEMATOLOGICA文献大全abstract:BACKGROUND:Acute myeloid leukemia is a clonal hematopoietic malignant disease; about 45-50% of cases do not have detectable chromosomal abnormalities. Here, we identified hidden genomic alterations and novel disease-related regions in normal karyotype acute myeloid leukemia/myelodysplastic syndrome samples. DESIGN AND...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.13024
更新日期:2009-02-01 00:00:00
abstract::In this article we summarize the clinical outcome of unrelated placental/umbilical cord blood (CB) transplantation, discuss the biological characteristics of CB hematopoietic progenitor/stem cells (HPC) and balance the relative advantages and disadvantages of this therapy as compared with transplantation of other HPC ...
journal_title:Haematologica
pub_type: 杂志文章,评审
doi:
更新日期:1999-08-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:To assess the efficacy and the toxic profile of gemcitabine, a novel pyrimidine antimetabolite active against several solid tumors, we carried out a study in heavily pretreated Hodgkin's disease (HD) patients. DESIGN AND METHODS:From May 1997 to January 1999, 14 pretreated patients (10 relaps...
journal_title:Haematologica
pub_type: 临床试验,杂志文章
doi:
更新日期:2000-09-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Recombinant erythropoietin (r-EPO) induces erythroid responses in patients affected by myelodysplastic syndromes (MDS). However, the response rate declines to 10-15% in MDS with substantial transfusion needs. Both in vitro and in vivo studies have suggested that the addition of growth factors ...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2001-01-01 00:00:00
abstract::A new method, combining seminested polymerase chain reaction (PCR) with heteroduplex analysis, was utilized to detect follicular lymphoma (FL) cells in peripheral blood. The method, based on the detection of IgH rearrangements in DNA, detected the presence of monoclonal B cells in FL patients with a high frequency. ...
journal_title:Haematologica
pub_type: 信件
doi:
更新日期:2005-02-01 00:00:00
abstract::Disease bulk is an important prognostic factor in early stage Hodgkin lymphoma, but its definition is unclear in the computed tomography era. This retrospective analysis investigated the prognostic significance of bulky disease measured in transverse and coronal planes on computed tomography imaging. Early stage Hodgk...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2016.141846
更新日期:2016-10-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Occult hepatitis B virus (HBV) infection might allow the release of viremic units into the blood supply network if blood is tested only for hepatitis B surface antigen (HBsAg). The aim of our study was to evaluate the actual prevalence, viral load and genotype of occult HBV infections among fi...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.11224
更新日期:2007-12-01 00:00:00
abstract::The long-term repopulating capacity of mobilized peripheral blood (MPB) CD34+ cells can be abrogated during ex vivo expansion due to cell-cycle related changes. In this study, we traced cell cycle kinetic and quiescent status of CD34+ cells during ex vivo expansion with exogenous cytokines. The addition of interleukin...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2006-01-01 00:00:00
abstract::The European Myeloma Network has organized two workshops on fluorescence in situ hybridization in multiple myeloma. The first aimed to identify specific indications and consensus technical approaches of current practice. A second workshop followed a quality control exercise in which 21 laboratories analyzed diagnostic...
journal_title:Haematologica
pub_type: 共识发展会议,杂志文章
doi:10.3324/haematol.2011.056176
更新日期:2012-08-01 00:00:00
abstract::Our objective was to determine whether the goal of high-dose therapy should be a partial hematologic response or a complete response. We analyzed 282 consecutive stem cell transplant patients. A partial hematologic response was achieved in 108 patients (38%), and 93 (33%) achieved a complete hematologic response. Surv...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.11413
更新日期:2007-10-01 00:00:00
abstract::In this open label pilot safety study 80 children over 3 months old with deep venous thrombosis were treated with enoxaparin with a target 4 h anti-factor Xa activity between 0.5-0.8 IU/mL. The children were stratified to receive once daily or twice daily doses. The study end-points were post-thrombotic syndrome, re-t...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:
更新日期:2006-12-01 00:00:00
abstract:BACKGROUND:Preclinical studies have highlighted the activity of lenalidomide in mantle cell lymphoma and its anti-proliferative synergy with dexamethasone. DESIGN AND METHODS:In this prospective, multicenter, phase II study, patients with relapsed/refractory mantle cell lymphoma who were not eligible for, or had relap...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:10.3324/haematol.2011.051813
更新日期:2012-03-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Storage pool diseases (SPD) are heterogeneous disorders associated with an abnormal presence of intraplatelet granules, which cause mild to moderate bleeding diathesis. We investigated signaling through tyrosine phosphorylation of proteins occurring in platelets with total or partial absence o...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2002-06-01 00:00:00
abstract::A case-control study was conducted among Italian children treated with a stem cell transplant (SCT). Cases (n = 43) were allogeneic recipients with osteonecrosis, and the controls (n = 129) were matched to the corresponding cases on the basis of survival, SCT center and date of transplant. Univariate analysis showed t...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Although acquired aplastic anemia is usually a chronic disorder, a small proportion of patients with aplastic anemia was reported to recover spontaneously without specific therapy such as allogeneic bone marrow transplantation or immunosuppressive treatment. We attempted to determine the rate ...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2001-09-01 00:00:00
abstract::We conducted a phase 1 study of an anti-CD33 immunotoxin, humanized monoclonal antibody M195 conjugated to recombinant gelonin (HUM-195/rGEL), in patients with relapsed, refractory myeloid leukemias. Twenty-eight patients received the construct intravenously at four dose levels (12, 18, 28 and 40 mg/m(2) per course) i...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2012.071092
更新日期:2013-02-01 00:00:00
abstract:BACKGROUND:The suppression of Ph1-positive hemopoiesis is a major goal in the treatment of CML; in this context the CML patients in blast crisis obtaining a complete clinical remission represent a useful model to investigate the behavior of the Ph1-positive and negative clones during bone marrow repopulation after abla...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1991-03-01 00:00:00
abstract::It is currently unknown whether immunosuppressive therapy or hematopoietic stem cell transplantation is the most appropriate treatment strategy for children with refractory cytopenia and normal karyotype or trisomy 8. We report on 31 children with hypoplastic refractory cytopenia treated with immunosuppressive therapy...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.10683
更新日期:2007-03-01 00:00:00
abstract::Chronic lymphocytic leukemia is a disease with up-regulated expression of the transmembrane tyrosine-protein kinase ROR1, a member of the Wnt/planar cell polarity pathway. In this study, we identified COBLL1 as a novel interaction partner of ROR1. COBLL1 shows clear bimodal expression with high levels in chronic lymph...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2017.178699
更新日期:2018-02-01 00:00:00
abstract::Proteolysis targeting chimeric molecule ARV 825 causes ubiquitination of bromodomains resulting in their efficient degradation by proteasome activity. Bromodomain degradation down-regulates MYC transcription contributing to growth inhibition of various human cancers. We examined the therapeutic potential of ARV 825 ag...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2018.201483
更新日期:2019-06-01 00:00:00
abstract::Reduction in minimal residual disease, measured by real-time quantitative PCR or flow cytometry, predicts prognosis in childhood B-cell precursor acute lymphoblastic leukemia. We explored whether cells reported as minimal residual disease by flow cytometry represent the malignant clone harboring clone-specific genomic...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2011.051383
更新日期:2012-01-01 00:00:00
abstract::Since HA-1-specific T cells have been shown to make a significant contribution to the clinical responses in patients with relapsed leukemia, we investigated the feasibility of adoptive transfer of in vitro induced HA-1-specific CD8 positive T cells to patients with relapsed leukemia after allogeneic stem cell transpla...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2011.053371
更新日期:2012-08-01 00:00:00
abstract:BACKGROUND:During the final stages of differentiation of mammalian erythroid cells, the chromatin is condensed and enucleated. We previously reported that Rac GTPases and their downstream target, mammalian homolog of Drosophila diaphanous 2 (mDia2), are required for enucleation of in vitro cultured mouse fetal liver er...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2010.029827
更新日期:2010-12-01 00:00:00
abstract::Sixteen autologous stem cell transplant recipients received three vaccinations with conjugated haemophilus influenzae type b vaccine. Quantitative and qualitative aspects of the antibody response were studied. The vaccination schedule resulted in high antibody response rates and functional maturation of antibodies, as...
journal_title:Haematologica
pub_type: 信件
doi:
更新日期:2005-11-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:The use of intensive therapy supported by autologous stem cell transplantation (ASCT) is being investigated as treatment for poor-prognosis follicular lymphomas (FL). A single-center experience is herein reported. DESIGN AND METHODS:From September 1990 to October 1997, 30 consecutive patients ...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1999-04-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:The available data on the immunoglobulin gene (IG) repertoire in multiple myeloma (MM) derive mainly from heavy chains; considerably less is known about light chains. We assessed in parallel IGH and IGK/IGL rearrangements in 101 MM patients so as to gain insight into: (i) IG repertoires; (ii) ...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2006-06-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:The rate of post-operative asymptomatic deep-vein thrombosis in Asian patients remains uncertain. The aim of this study was to estimate the rate of venous thromboembolism, including asymptomatic deep-vein thrombosis, undergoing hip or knee surgery and not receiving pharmacological thromboproph...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:10.3324/haematol.10819
更新日期:2007-09-01 00:00:00
abstract::With the deaths of Janet Rowley and John Goldman in December 2013, the world lost two pioneers in the field of chronic myeloid leukemia. In 1973, Janet Rowley, unraveled the cytogenetic anatomy of the Philadelphia chromosome, which subsequently led to the identification of the BCR-ABL1 fusion gene and its principal pa...
journal_title:Haematologica
pub_type: 传,历史文章,杂志文章,评审
doi:10.3324/haematol.2015.139337
更新日期:2016-05-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:The modulation of disease severity in hemophilia A (HA) patients may be related to the co-inheritance of mutations in genes with a known thrombotic effect such as factor V Leiden (FVL) and prothrombin. In the Spanish population, the prothrombin 20210A (PT20210A) allele is the most prevalent ge...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2002-03-01 00:00:00
abstract::The role of reduced intensity allogeneic stem cell transplantation for the treatment of relapsed/refractory Hodgkin's lymphoma remains controversial. We retrospectively analyzed 191 patients who underwent reduced intensity allogeneic stem cell transplantation between 1998 and 2008 for relapsed or refractory Hodgkin's ...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2012.080895
更新日期:2013-09-01 00:00:00