Abstract：
BACKGROUND:For most cases of idiopathic acquired sideroblastic anemia (IASA), the molecular pathogenesis is unknown, despite the consistent morphological signature of abundant pathological ringed sideroblasts with their characteristic iron-engorged mitochondria. Moderately elevated free erythrocyte protoporphyrin (FEP) levels have been described in IASA, suggesting that the activity of ferrochelatase, the enzyme that catalyzes the final step in heme biosynthesis (incorporation of ferrous iron into protoporphyrin), might be diminished in erythroid progenitor cells from IASA patients. METHODS:We confirmed FEP elevation in IASA, then pursued a candidate gene approach that included screening the gene encoding ferrochelatase, FECH, for promoter and coding region mutations and mRNA expression changes in bone marrow from 37 patients with IASA. RESULTS:The analytical techniques employed detected mutations in a test cohort of previously undiagnosed patients with biochemical evidence for erythropoietic protoporphyria, a condition resulting from germline mutations in FECH, but somatic missense mutations of FECH and its promoter were not observed in IASA patients. FECH was modestly overexpressed in progenitor cells from patients with IASA, compared with MDS patients without sideroblasts and healthy controls. In addition, we analyzed ABCB7 and PUS1, genes implicated in congenital sideroblastic anemia syndromes, but again found no coding mutations in acquired cases. CONCLUSION:We conclude that acquired mutations in the factors currently known to cause inherited sideroblastic anemias are uncommon in IASA.
journal_name
Leuk Resjournal_title
Leukemia researchauthors
Steensma DP,Hecksel KA,Porcher JC,Lasho TLdoi
10.1016/j.leukres.2006.06.005subject
Has Abstractpub_date
2007-05-01 00:00:00pages
623-8issue
5eissn
0145-2126issn
1873-5835pii
S0145-2126(06)00219-0journal_volume
31pub_type
杂志文章abstract：:Regulation of pluripotent stem cell (CFU-S) proliferation kinetics by humoral factors is now well documented. However, the mechanism of choice of CFU-S differentiation pathways is still a controversial issue. We suggest that long-range humoral factors (pluripoietins) are capable of preferentially channelling CFU-S tow...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(84)90041-9
更新日期：1984-01-01 00:00:00
abstract：:Mononuclear cells from a bone marrow infiltrated by plasmacytoma cells were examined by electron microscopy. An analysis was performed according to different cytoarchitectural forms of the endoplasmic reticulum (ER). Six types of plasma cells could be distinguished. The bone marrow cells were treated with an anti-idio...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(84)90090-0
更新日期：1984-01-01 00:00:00
abstract：:Human promyelocytic leukemia cells (HL-60) mature into functional granulocytes in response to in vitro treatment with several classes of chemical agents. Compounds that increase intracellular adenosine 3':5' -cyclic monophosphate induce a modified program of maturation in which the cells demonstrate functional propert...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(85)90311-x
更新日期：1985-01-01 00:00:00
abstract：:Bone marrow cells of five patients with acute myeloid leukemia were fractionated by means of counterflow centrifugation (elutriation). The different fractions were enriched with cells belonging to subsequent stages of the cell cycle. Cytokinetic evaluation of these cell fractions was performed by [3H]thymidine autorad...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(82)90031-5
更新日期：1982-01-01 00:00:00
abstract：:The aim of this study was to describe the presenting features, the frequency and outcome of myeloid sarcoma (MS) diagnosed in our Institution from January 1995 to December 2000. Twelve MS were seen and the frequency account for only 2% of all acute myeloid leukemia (AML) patients observed in our department in the same...
journal_title：Leukemia research
pub_type： 杂志文章,评审
doi：10.1016/j.leukres.2004.01.022
更新日期：2004-11-01 00:00:00
abstract：:The revised IPSS (IPSS-R) was developed aiming at a better prognostication, taking into account patients treated with best supportive care. We herein validated this model on the basis of data from 1314 patients who received BSC only as well as patients who underwent induction chemotherapy (n=214) or allogeneic transpl...
journal_title：Leukemia research
pub_type： 杂志文章,多中心研究
doi：10.1016/j.leukres.2013.10.013
更新日期：2014-01-01 00:00:00
abstract：:Rearrangements in MLL (MLL-r) are common within very young children with leukemia and affect the prognosis and treatment. Previous studies have suggested the use of the NG2 molecule as a marker for MLL-r but these studies were performed using a small number of infants. We analyzed 148 patients (all less than 24 months...
journal_title：Leukemia research
pub_type： 杂志文章,多中心研究
doi：10.1016/j.leukres.2011.03.006
更新日期：2011-08-01 00:00:00
abstract：:This study investigated radioresistance mechanisms in the doxorubicin-resistant acute myelogenous leukemia (AML)-2/DX100. AML-2/DX100 also showed resistance to radiation. AML-2/DX100 characterized by down-regulated catalase expression was supersensitive to exogenous hydrogen peroxide whereas they increased defense mec...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2006.11.006
更新日期：2007-09-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2008.07.002
更新日期：2009-03-01 00:00:00
abstract：:Induction of granulocyte maturation in HL-60 leukemic cells by DMSO (1.2%) or RA (1 microM) is accompanied by a 50-60% decrease in cellular thymidine kinase activity. Similarly, the differentiation of HL-60 cells into monocyte-macrophage phenotype by the addition of PMA is paralleled by a 60-80% suppression of thymidi...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(87)90079-8
更新日期：1987-01-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(96)00036-7
更新日期：1996-09-01 00:00:00
abstract：:Our aim was to examine in 17 patients with MDS the effects of PMA activated and non-activated autologous lymphocytes on selected bone marrow CD34+ progenitors, in dose response studies. We used a double layer culture technique. Compared with controls, there was no difference in the colony growth promoting capacity of ...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2011.03.017
更新日期：2011-09-01 00:00:00
abstract：:Chemotherapy resistance has been considered as a major problem for multiple myeloma (MM) treatment and bone marrow microenvironment plays a crucial role in the MM progression and chemoresistance. Recent studies reported that bone marrow mesenchymal stem cells derived from MM patients (MM-MSCs) revealed various charact...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2017.07.002
更新日期：2017-09-01 00:00:00
abstract：:Acute monocytic leukemia (M5) is a subtype of acute myeloid leukemia (AML) with two distinct morphologic subcategories, M5a and M5b. We compared the immunophenotype, cytogenetics and clinical outcome of AML M5 with non-M5 AML and also compared M5a with M5b. One hundred and twelve M5 (76 M5a, 36 M5b) and 726 non-M5 cas...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2007.06.019
更新日期：2008-02-01 00:00:00
abstract：OBJECTIVE:Bortezomib-dexamethasone-thalidomide has been reported to be effective in newly-diagnosed multiple myeloma (MM) with an overall response rate of 92% and a CR rate of 18% (Alexanian et al, Hematology 2007;12(3):235-9), but this regimen has not been tested in the Chinese patients. We report here our results tes...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2009.04.006
更新日期：2009-12-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2009.08.022
更新日期：2010-06-01 00:00:00
abstract：:We compared the efficacy and toxicity of BEAM (BCNU, etoposide, cytarabine and melphalan) and BuCyE (busulfan, cyclophosphamide and etoposide), given prior to autologous stem cell transplantation (ASCT), in 65 patients with non-Hodgkin's lymphoma. Of these 65 patients, 43 received BEAM and 22 received BuCyE. Their age...
journal_title：Leukemia research
pub_type： 临床试验,杂志文章
doi：10.1016/j.leukres.2010.07.016
更新日期：2011-02-01 00:00:00
abstract：:A primary mitochondrial defect may be pivotal in the pathogenesis of acquired idiopathic sideroblastic anemia (AISA). The mitochondrial respiratory chain is involved in mitochondrial iron uptake and supply of ferrous iron (Fe2+) for heme synthesis. Mitochondrial DNA (mtDNA) comes into play because several subunits of ...
journal_title：Leukemia research
pub_type： 杂志文章,评审
doi：10.1016/s0145-2126(99)00160-5
更新日期：2000-02-01 00:00:00
abstract：:Outcome of adult acute lymphoblastic leukemia (ALL) with central nervous system (CNS) involvement is not clearly defined. We studied 104 patients presenting with CNS involvement at diagnosis among 1493 patients (7%) included into the LALA trials, and 109 patients presenting CNS disease at the time of first relapse amo...
journal_title：Leukemia research
pub_type： 杂志文章,多中心研究
doi：10.1016/j.leukres.2008.04.011
更新日期：2008-11-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2013.02.010
更新日期：2013-07-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(84)90001-8
更新日期：1984-01-01 00:00:00
abstract：:c-Myc has been implicated in a variety of hematologic malignancies including Burkitt's lymphoma. Targeting c-Myc driven growth pathways could be therapeutically useful but might require the identification of critical downstream proteins. Here we show that the serine-threonine kinase PBK/TOPK is frequently overexpresse...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2012.11.010
更新日期：2013-04-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2003.10.016
更新日期：2004-06-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2007.03.034
更新日期：2008-01-01 00:00:00
abstract：:Checkpoint genes code for a family of proteins which sense DNA damage in eukaryotic cells. They play an important role in the control of the cell cycle. The human CHK2 is a homolog of the yeast G(2) checkpoint kinases known as CDS1 and RAD53. The CHK2 may be a tumor suppressor gene because it was found to be mutated i...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/s0145-2126(00)00130-2
更新日期：2001-04-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章,评审
doi：10.1016/j.leukres.2015.06.017
更新日期：2015-10-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2012.03.018
更新日期：2012-08-01 00:00:00
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journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/0145-2126(94)90004-3
更新日期：1994-01-01 00:00:00
abstract：:Gain-of-function mutations in the proto-oncogene c-kit that induce constitutive kinase activity of its product, KIT protein, are characteristic of human mast cell disease and are believed to play a central role in mast cell leukemia oncogenesis, proliferation and survival. Nuclear overexpression of the Wnt effector be...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/j.leukres.2007.08.023
更新日期：2008-05-01 00:00:00
abstract：:We report single institution outcome of brief, intensive ara-C-based chemotherapy using bone marrow transplantation as primary intensification for untreated adult patients with acute lymphoblastic leukemia (ALL). Overall disease-free and overall survival were inferior to those reported with prolonged chemotherapy mode...
journal_title：Leukemia research
pub_type： 杂志文章
doi：10.1016/s0145-2126(01)00175-8
更新日期：2002-05-01 00:00:00
