Abstract:
:We are reporting here the results of differential gene expression experiments comparing two siblings, a 21-yr-old male and a 19-yr-old female, with the same alpha-thalassemia genotype (-alpha(3.7)/(--SEA)) and quite different levels of Hb H in the peripheral blood (18.7 and 5%, respectively). By using mRNA differential-display reverse-transcription-PCR and suppression subtractive hybridization, two main transcripts were selected in both procedures and validated by qRT-PCR, one corresponding to the phosphatidylinositol phosphate 4-kinase type II-alpha (PIP4KIIA) gene and the other to the beta-globin gene, both over expressed in the patient with the higher percentage of Hb H. Type II PIP kinases produce phosphatidylinositol 4,5 biphosphate, a critical and pleiotropic regulatory molecule involved in diverse cellular activities, including gene expression. Our results suggest that PIP4KIIA may be one of the factors related to the regulation of the beta-globin gene expression and the different levels of Hb H in alpha-thalassemic patients.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Wenning MR,Mello MP,Andrade TG,Lanaro C,Albuquerque DM,Saad ST,Costa FF,Sonati MFdoi
10.1111/j.1600-0609.2009.01325.xsubject
Has Abstractpub_date
2009-11-01 00:00:00pages
490-3issue
5eissn
0902-4441issn
1600-0609pii
EJH1325journal_volume
83pub_type
杂志文章abstract:OBJECTIVES:Some patients with myelodysplastic syndrome (MDS) show a marked increase in the percentage of immature platelet fraction (IPF%) despite the absence of severe thrombocytopenia. To determine the significance of such an unbalanced increase in the IPF%, we investigated the IPF% and other laboratory findings of 5...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01156.x
更新日期:2009-01-01 00:00:00
abstract:OBJECTIVES:Vitamin E has antioxidant and immunomodulatory effects that might influence the development of acute graft-versus-host disease (GvHD). We investigated the association between plasma vitamin E levels and acute GvHD. METHODS:We studied 115 adults who underwent myeloablative allogeneic hematopoietic cell trans...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13567
更新日期:2020-12-12 00:00:00
abstract::To determine if mutations of the Wilms' tumor predisposing gene (WT1) are associated with haematological malignancies, we have investigated 65 cases of acute leukaemia, including 39 patients in blast crisis of chronic myeloid leukaemia (CML), by amplification of WT1 exons 7, 8 and 9 followed by single-strand conformat...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1997.tb01681.x
更新日期:1997-05-01 00:00:00
abstract::It has been recently demonstrated that ecto-5'nucleotidase (5'NT) activity is significantly decreased in the peripheral blood lymphocytes (PBL) of homosexual men. This paper reports a study of PBL 5'NT activity in 38 hemophiliacs at risk for the acquired immunodeficiency syndrome (AIDS). The enzyme activity was correl...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb00003.x
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abstract::Most cytostatic drugs exert their effect on cells in active cell cycle. To improve the effect of cytostatic drugs we have tried, prior to treatment in vitro, to recruit tumor cells from G0 with growth factors. Leukemic cells from the bone marrows of 26 patients with AML and CML in blast crisis were incubated with G-CS...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1996.tb00285.x
更新日期:1996-01-01 00:00:00
abstract::This study shows that the ingestion of ethanol provokes alterations in iron metabolism which may lead to iron overload. Impaired release of reticuloendothelial iron was shown by a decrease of the maximum red blood cell utilization when radioactive iron was supplied as colloidal iron. An impairment in the erythropoieti...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00204.x
更新日期:1988-10-01 00:00:00
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
更新日期:1987-03-01 00:00:00
abstract::We investigated the significance of cytokines (soluble interleukin-2 receptor, granulocyte-macrophage colony-stimulating factor, interleukin-6, and interferon-gamma) and CD68-positive microparticles in immune thrombocytopenic purpura. Cytokines were measured by enzyme-linked immunosorbent assay and microparticles were...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1995.tb00232.x
更新日期:1995-07-01 00:00:00
abstract::Externalization of phosphatidylserine (PS) is thought to contribute to sickle cell disease (SCD) pathophysiology. The red blood cell (RBC) aminophospholipid translocase (APLT) mediates the transport of PS from the outer to the inner RBC membrane leaflet to maintain an asymmetric distribution of PL, while phospholipid ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12506
更新日期:2015-11-01 00:00:00
abstract::Our aim was to assess liver iron content, in thalassaemic patients, by using three different MR protocols and compare their data. Ninety-four thalassaemic patients (44 M and 50 F, mean age 25.82 +/- 8.3 yrs), were enrolled in the study. In each patient, three measurements of the liver iron content were performed, with...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2009.01223.x
更新日期:2009-05-01 00:00:00
abstract::506 unselected, previously untreated patients with Hodgkin's disease were treated at the Finsen Institute between 1969 and 1983. The prognostic significance of age, sex, stage, systemic symptoms, histologic subtype, number of involved nodal regions, total tumour burden (peripheral + intrathoracic nodal tumour burden, ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:
更新日期:1989-08-01 00:00:00
abstract:OBJECTIVES:Granulocyte colony-stimulating factor-mobilized peripheral blood hematopoietic stem cell transplantation (HSCT) provides a valuable and increasingly used alternative to bone marrow transplantation (BMT). This retrospective study aimed at determining whether the stem cell source is predictive for outcome, rel...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2011.01692.x
更新日期:2011-12-01 00:00:00
abstract:OBJECTIVES:Myeloma bone disease is a result of excessive osteoclast activation and impaired osteoblast function. Recent in vitro studies suggested that proteasome inhibitors might increase osteoblast function. METHODS:We analyzed serum markers of osteoblast activity in 25 patients with multiple myeloma receiving borte...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2006.00692.x
更新日期:2006-09-01 00:00:00
abstract::A 1-yr-old Japanese male infant developed hepatitis-associated aplastic anemia (AA), and anti-thymocyte globulin (ATG) plus cyclosporine A (CsA) was administered without any appreciable effects. Laboratory examination of the patient's serum obtained before therapy revealed various autoantibodies, such as PA-IgG, anti-...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2011.01612.x
更新日期:2011-06-01 00:00:00
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
更新日期:1998-09-01 00:00:00
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
更新日期:2010-01-01 00:00:00
abstract::Oxidative damage to erythrocytes in thalassaemia has been related to generation of free radicals by an excess of denaturated alpha- or beta-globin chains, intracellular iron overload and low concentration of normal haemoglobin (HGB). Two good indicators of such oxidative damage are the high red blood cell (RBC) malony...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1995.tb00705.x
更新日期:1995-11-01 00:00:00
abstract:OBJECTIVES:The benefits of combined chelation therapy with daily deferiprone (DFP) and subcutaneous desferrioxamine (DFO) have been widely reported in literature. We retrospectively evaluated the efficacy of different schedules of combined chelation therapy and the incidence of adverse events. METHODS:We evaluated 36 ...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.2010.01447.x
更新日期:2010-07-01 00:00:00
abstract:OBJECTIVES:Recent studies have indicated that patients who receive stem cell transplantation (SCT) and rituximab demonstrate an increased risk of developing hypogammaglobulinemia. Such hypogammaglobulinemia has been found to be due to delayed recovery of memory B cells with an abnormal cell marker expression and impair...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01174.x
更新日期:2009-02-01 00:00:00
abstract:OBJECTIVES:An excessive intramedullar progenitor cell apoptosis, to which elevated expression of tumor necrosis factor-alpha (TNF-alpha) might contribute, is considered the main cause of inefficient hematopoiesis in myelodysplastic syndromes (MDS). Enhanced bone marrow (BM) angiogenesis is regarded as an essential cofa...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2005.00551.x
更新日期:2005-12-01 00:00:00
abstract::Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder of the hematopoietic stem cell that makes blood cells more sensitive to the action of complement. Patients experience intravascular hemolysis, smooth muscle dystonia, renal failure, arterial and pulmonary hypertension, recurrent infectious diseases and ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12543
更新日期:2015-09-01 00:00:00
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journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/ejh.13314
更新日期:2019-11-01 00:00:00
abstract::Host cells are protected from the lytic effect of the complement system by complement regulatory proteins. This study was designed to investigate the expression of complement regulatory proteins on leukemic blasts which may be susceptible to the lytic effects of the complement system in the circulation. The surface ex...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2000.80097.x
更新日期:2000-01-01 00:00:00
abstract:OBJECTIVES:Imatinib (Glivec, STI571) has been successfully used in patients with chronic myelogenous leukemia and acute lymphoblastic leukemia with the Philadelphia chromosome (Ph). We used imatinib interim therapy for four consecutive patients with newly diagnosed Ph+ acute myeloid leukemia (AML). We monitored the pat...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00890.x
更新日期:2007-08-01 00:00:00
abstract::We analyzed the expression of the inhibitor of apoptosis survivin by immunocytochemistry in bone marrow cells from patients with chronic myelomonocytic leukemia (CMML) to evaluate possible abnormalities in comparison with other myelodysplastic (MDS) and myeloproliferative syndromes, and to investigate a possible corre...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.0902-4441.2006.t01-1-EJH2588.x
更新日期:2006-06-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Since a study published in 2002 showed a survival advantage of melphalan-only conditioning for stem cell transplantation (HSCT) over melphalan-total body irradiation (mel-TBI) in patients with multiple myeloma (MM), most centers abandoned mel-TBI. Mel-TBI causes more early toxicity and is more...
journal_title:European journal of haematology
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doi:10.1111/ejh.12890
更新日期:2017-07-01 00:00:00
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
更新日期:2016-08-01 00:00:00
abstract::The hematopoietic capacity of bone marrow treated with 4-hydroperoxycyclophosphamide (4-HC) was studied using long-term suspension cultures. It was shown that, in the presence of a previously established marrow adherent cell layer (ACL), the 4-HC treated bone marrow, deprived of GM-CFC, generates GM-CFC in long-term s...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb00740.x
更新日期:1987-08-01 00:00:00
abstract::Haemophilia remains a complex disorder to diagnose and manage, requiring close cooperation between multidisciplinary healthcare professionals. There are still many unmet challenges in haemophilia care. The first Team Haemophilia Education (THE) meeting, held on 7-8 May 2015 in Amsterdam, The Netherlands, aimed to prom...
journal_title:European journal of haematology
pub_type:
doi:10.1111/ejh.12760
更新日期:2016-07-01 00:00:00
abstract::Osteolytic lesions with activated osteoclast (OC) and suppressed osteoblast (OB) activity are characteristics of myeloma bone lesion. Recently, it has been shown that bortezomib treatment enhances OB function. To evaluate the effect of bortezomib on myeloma bone lesions, we performed bone scans, where increased uptake...
journal_title:European journal of haematology
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doi:10.1111/j.1600-0609.2010.01523.x
更新日期:2011-01-01 00:00:00