Abstract：
:Erythropoiesis is a dynamic process regulated at multiple levels to balance proliferation, differentiation and survival of erythroid progenitors. Ineffective erythropoiesis is a key feature of various diseases, including β-thalassemia. The pathogenic mechanisms leading to ineffective erythropoiesis are complex and still not fully understood. Altered survival and decreased differentiation of erythroid progenitors are both critical processes contributing to reduced production of mature red blood cells. Recent studies have identified novel important players and provided major advances in the development of targeted therapeutic approaches. In this review, β-thalassemia is used as a paradigmatic example to describe our current knowledge on the mechanisms leading to ineffective erythropoiesis and novel treatments that may have the potential to improve the clinical phenotype of associated diseases in the future.
journal_name
Blood Revjournal_title
Blood reviewsauthors
Oikonomidou PR,Rivella Sdoi
10.1016/j.blre.2017.10.001subject
Has Abstractpub_date
2018-03-01 00:00:00pages
130-143issue
2eissn
0268-960Xissn
1532-1681pii
S0268-960X(17)30131-5journal_volume
32pub_type
杂志文章,评审相关文献
BLOOD REVIEWS文献大全abstract：:Methaemoglobinaemia arises from the production of non-functional haemoglobin containing oxidised Fe(3+) which results in reduced oxygen supply to the tissues and manifests as cyanosis in the patient. It can develop by three distinct mechanisms: genetic mutation resulting in the presence of abnormal haemoglobin, a defi...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2004.02.001
更新日期：2005-03-01 00:00:00
abstract：:Nearly 20 years after murine embryonic stem cells (mESC) were isolated, the first report of the derivation of human embryonic stem cells (hESC) in 1998 spawned the field of hESC research [Evans MJ, Kaufman MH, Establishment in culture of pluripotential cells from mouse embryos. Nature 1981; 292 (5819): 154-6; Thomson ...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2005.01.005
更新日期：2005-11-01 00:00:00
abstract：:Fears about the risks of disease transmission through the transfusion of homologous blood, as well as problems with blood supply and cost have increased the interest in reducing perioperative bleeding. This article discusses the causes of non-surgical perioperative bleeding and attempts to reduce perioperative bleedin...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(91)90034-a
更新日期：1991-09-01 00:00:00
abstract：:Hepatic veno-occlusive disease (VOD) is a major cause of morbidity and mortality following high dose cytotoxic therapy for stem cell transplantation (SCT). Pre-existing liver damage, SCT-related therapy, and genetic polymorphisms all appear to increase the risk of developing VOD. Studies of biological markers during S...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/s0268-960x(03)00002-x
更新日期：2003-06-01 00:00:00
abstract：:The rate of relapse after allogeneic bone marrow transplantation (BMT) varies between 15 and 60%. New therapeutic strategies are required urgently as no significant results have been obtained with standard chemotherapy. The best results of second allogeneic BMT have been obtained when the interval between the first an...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(94)90111-2
更新日期：1994-12-01 00:00:00
abstract：:The majority of aggressive lymphomas is characterized by an up regulated glycolytic activity, which enables the visualization by F-18 FDG-PET/CT. One-stop hybrid FDG-PET/CT combines the functional and morphologic information, outperforming both, CT and FDG-PET as separate imaging modalities. This has resulted in sever...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2016.02.003
更新日期：2016-07-01 00:00:00
abstract：:Major bacterial infections are most commonly associated with agranulocytosis or an abnormality of immunoglobulins or complement. Occasionally, repeated infections cannot be attributed to these relatively common causes. In such cases, a quantitative abnormality in neutrophil function should be sought. Complete evaluati...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(95)90024-1
更新日期：1995-09-01 00:00:00
abstract：:Tissue factor pathway inhibitor (TFPI) is the main inhibitor of tissue factor (TF)-mediated coagulation. In atherosclerotic plaques TFPI co-localizes with TF, where it is believed to play an important role in attenuating TF activity. Findings in animal models such as TFPI knockout models and gene transfer models are c...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2013.03.001
更新日期：2013-05-01 00:00:00
abstract：:The finding that T cell immune responses could be divided into those promoting cell mediated immunity (Th1) and humoral responses (Th2) has had a profound effect on the understanding of immune response generation over the last 15 years. With ever increasing knowledge of the immune system, the model has come under crit...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1054/blre.2000.0136
更新日期：2000-12-01 00:00:00
abstract：:Immune checkpoint inhibitors (ICI) have yielded mixed but largely underwhelming results in clinical trials in patients with acute myeloid leukemia and myelodysplastic syndromes to date. However, increasing understanding of the immunologic landscape, potential biomarkers for benefits, and mechanisms of resistance, as w...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2020.100709
更新日期：2020-05-23 00:00:00
abstract：:The characteristics and the effectiveness of programmes designed to prevent beta-thalassemia major present in high frequency in several areas of the world such as Cyprus, Greece and Sardinia are reviewed. All these programmes are based on heterozygote detection, counselling and foetal diagnosis. The target population ...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(87)90032-4
更新日期：1987-09-01 00:00:00
abstract：:A working diagnosis of essential thrombocythemia (ET) is made in the presence of nonreactive thrombocytosis and after the exclusion of another chronic myeloid disorder that may mimic ET in its presentation. Clinically, ET is characterized by vasomotor symptoms, thrombohemorrhagic complications, recurrent fetal loss, a...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1054/blre.2001.0158
更新日期：2001-09-01 00:00:00
abstract：:Factor X circulates as a serine protease which is converted to the active form at the point of convergence of the intrinsic and extrinsic coagulation pathways. Subsequently, the enzymatic species, factor Xa, is involved in macromolecular complex formation with its cofactor factor Va, a phospholipid surface and calcium...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(94)90007-8
更新日期：1994-03-01 00:00:00
abstract：:Chronic lymphocytic leukemia (CLL) is a disease of older patients and median age at diagnosis is 72 years. This older group is under-represented in clinical trials, (median age 58-62 years). Here we review background data on incidence, survival, definitions of older age, fitness criteria, frailty and co-morbidities. I...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2011.09.002
更新日期：2012-01-01 00:00:00
abstract：:Four decades ago, two staging systems were developed to help stratify CLL into different prognostic categories. These systems, the Rai and the Binet staging, depended entirely on abnormal exam findings and evidence of anemia and thrombocytopenia. Better understanding of biologic, genetic, and molecular characteristics...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2017.10.003
更新日期：2018-03-01 00:00:00
abstract：:The improvement in supportive care and the introduction of new therapeutic agents, including lenalidomide and hypomethylating agents, in myelodysplastic syndromes have improved patients' outcomes; however, at the same time, the frequency and epidemiology of infections have changed. Therefore, the great strides in the ...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2018.10.002
更新日期：2019-03-01 00:00:00
abstract：:Advances in therapy can essentially be measured using two parameters; introduction of a new agent which benefits an increased number of patients over prevailing treatments or more selective use of an existing drug by matching it to the biologic characteristics associated with response. In reviewing the therapeutic lan...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2011.11.001
更新日期：2012-03-01 00:00:00
abstract：:It is the conventional opinion that acquired aplastic anaemia is a heterogenous disease including basically different conditions, such as idiopathic or virus induced pancytopenia, toxic-allergic marrow damage or autoimmunity. Here, an alternative concept is proposed, according to which aplastic anaemia is one disease,...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(90)90032-n
更新日期：1990-06-01 00:00:00
abstract：:Lymphomas cause significant morbidity and mortality worldwide. A substantial number of patients ultimately relapse after standard treatment. However, the efficacy of these therapies can be counteracted by the patients' immune system, more specifically by myeloid-derived suppressor cells (MDSC). MDSC are a heterogeneou...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2018.04.006
更新日期：2018-11-01 00:00:00
abstract：:In the vicinity of an acute inflammatory response both cellular and non-cellular elements may interact to modify the overall response. Evidence suggests that leukocytes may play an active role in the modulation of platelet function and vice-versa. This interaction may be abnormal in certain pathological states. Neutro...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(92)90010-n
更新日期：1992-06-01 00:00:00
abstract：:For the majority of patients presenting with early Hodgkin's disease, the chance of death due to their disease is related to prognostic factors, e.g. age, systemic symptoms, ESR bulk, number of sites of disease, histology, haemoglobin, lymphocyte count etc. More than 50% of those with Stage I and IIA disease fall into...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(91)90011-z
更新日期：1991-12-01 00:00:00
abstract：:Platelet transfusions are widely used. Prophylactic transfusions are employed in severely thrombocytopenic patients without evidence of bleeding, but no randomized trial data prove the safety or efficacy of this approach. Randomized trials have demonstrated the equivalence of transfusion triggers of 10,000 and 20,000/...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/S0268-960X(03)00057-2
更新日期：2004-09-01 00:00:00
abstract：:Childhood immune thrombocytopenic purpura (ITP) is acute and generally seasonal in nature, suggesting that infectious or environmental agents may trigger the immune response to produce platelet-reactive autoantibodies 4 to 8 weeks following an infection. In general, the patient is well apart from the diffuse bruising ...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1054/blre.2001.0177
更新日期：2002-03-01 00:00:00
abstract：:Recurrent fetal loss and other placental vascular pathologies of pregnancy have long been associated with antiphospholipid syndrome-an acquired autoimmune thrombophilic state. The number of known heritable thrombophilic disorders has grown rapidly in recent years with the identification of activated protein C resistan...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/s0268-960x(97)90013-8
更新日期：1997-06-01 00:00:00
abstract：:Venous thromboembolism (VTE) is a common condition with high associated morbidity and mortality. Athletes have unique VTE risk factors compared with the general population, and may have a higher than anticipated risk of thrombosis. Anticoagulant treatment poses additional challenges in athletes, as these individuals u...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2020.100780
更新日期：2020-11-15 00:00:00
abstract：:Thrombopoietin (TPO) is the key cytokine involved in thrombopoiesis, and is the endogenous ligand for the thrombopoietin receptor that is expressed on the surface of platelets, megakaryocytes, and megakaryocytic precursors. First-generation thrombopoietic agents were recombinant forms of human TPO, and their developme...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2010.04.002
更新日期：2010-07-01 00:00:00
abstract：:Notch signaling can regulate both hematopoietic progenitors and alloimmune T cells in the setting of allogeneic bone marrow or hematopoietic cell transplantation (allo-HCT). Ex vivo culture of multipotent blood progenitors with immobilized Delta-like ligands induces supraphysiological Notch signals and can markedly en...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2013.08.001
更新日期：2013-11-01 00:00:00
abstract：:The clinical relevance of flow cytometry (FC)-based bone marrow involvement (BMI) in B cell non-Hodgkin lymphoma (B-NHL) is not well established. We conducted a systematic review of MEDLINE regarding the use of FC to establish BMI in B-NHL to determine the prevalence of BMI by FC, to understand the interrelation betwe...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2020.100778
更新日期：2020-11-08 00:00:00
abstract：:Fetal/neonatal alloimmune thrombocytopenia results from a maternal alloimmunization against fetal platelet antigens. Care must be taken in making a correct diagnosis that eliminates other causes of thrombocytopenia that may occur during pregnancy. Biological diagnosis is normally made by platelet genotyping and search...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1054/blre.2001.0187
更新日期：2002-03-01 00:00:00
abstract：:Long-term survival rates in childhood acute lymphoblastic leukemia (ALL) are currently above 85% due to huge improvements in treatment. However, 15-20% of children still experience relapses. Relapses can either occur in the bone marrow or at extramedullary sites, such as gonads or the central nervous system (CNS), for...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2019.04.003
更新日期：2019-07-01 00:00:00
