Abstract：
:Inherited hemoglobin-related disorders, which include the structural variants (hemoglobin S, C, and E) and the alpha (α)- and beta (β)-thalassemias, affect more than 300,000 children annually, particularly in malaria-endemic regions stretching from sub-Saharan Africa and the Mediterranean to Southeast Asia. Screening for carriers of these traits is important to provide prenatal genetic counseling and to accurately estimate the true prevalence and public health burden of these disorders. The clinical course of thalassemias, which affect nearly 70,000 children annually, is highly variable depending on the mixture of inherited alleles. The primary forms of non-transfusion-dependent thalassemia include β-thalassemia intermedia, hemoglobin E β-thalassemia, and hemoglobin H disease. Early clinical recognition of these disorders is essential to prevent affected children from being mistakenly placed on life-long transfusion therapy.
journal_name
Blood Revjournal_title
Blood reviewsauthors
Weatherall DJdoi
10.1016/S0268-960X(12)70003-6subject
Has Abstractpub_date
2012-04-01 00:00:00pages
S3-6eissn
0268-960Xissn
1532-1681pii
S0268-960X(12)70003-6journal_volume
26 Suppl 1pub_type
杂志文章,评审相关文献
BLOOD REVIEWS文献大全abstract：:Drug-induced agranulocytosis is a highly individualized and unexpected reaction to specific drugs. It may be due to immunogenic or cytotoxic factors. Most instances are produced by a poorly understood immune response to immunogenic drugs. Others are associated with direct suppression of marrow committed stem cells by ...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(90)90002-a
更新日期：1990-12-01 00:00:00
abstract：:The process of megakaryocytopoiesis begins with the commitment of a pluripotent hematopoietic stem cell to a differentiation pathway that culminates in the release of mature platelets into the circulation. A variety of megakaryocyte precursor cells have been identified after stem cell commitment has occurred and these...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(95)90034-9
更新日期：1995-03-01 00:00:00
abstract：:Hemophagocytic syndromes (hemophagocytic lymphohistiocytosis, HLH) represent a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. Biochemical markers include elevated ferritin and triglyce...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2007.05.001
更新日期：2007-09-01 00:00:00
abstract：:Upon activation, platelets secrete more than 300 active substances from their intracellular granules. Platelet dense granule components, such as ADP and polyphosphates, contribute to haemostasis and coagulation, but also play a role in cancer metastasis. α-Granules contain multiple cytokines, mitogens, pro- and anti-i...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2014.10.003
更新日期：2015-05-01 00:00:00
abstract：:The myeloproliferative neoplasms (MPNs) encompass a heterogenous set of diseases that have variable survival, but in the setting of treatment refractory and progressive disease, prognosis has been characteristically poor. JAK inhibition with ruxolitinib or fedratinib therapy has become the first line treatment for sym...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2020.100716
更新日期：2020-07-01 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：:Anemia is present in over two-thirds of patients with malignant hematological disorders. The etiology of anemia predominates from ineffective erythropoiesis from marrow infiltration, cytokine related suppression, erythropoietin suppression, and vitamin deficiency; ineffective erythropoiesis is further exacerbated by a...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2015.02.001
更新日期：2015-09-01 00:00:00
abstract：:Reactivation of fetal hemoglobin (HbF) expression is an important therapeutic option in patients with hemoglobin disorders. In sickle cell disease (SCD), an increase in HbF would interfere with the polymerization of sickle hemoglobin while in beta-thalassemia, an increase in gamma-globin chain synthesis would decrease...
journal_title：Blood reviews
pub_type： 杂志文章
doi：10.1016/j.blre.2006.01.002
更新日期：2006-07-01 00:00:00
abstract：:Myelodysplastic syndromes (MDS) are a heterogeneous group of myeloid disorders characterized by cytopenias and increased risk of acute leukemia transformation. Prognosis of MDS patients can be assessed by various scoring systems, the most common being the International Prognostic Scoring System (IPSS) now refined by t...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2020.100689
更新日期：2020-03-31 00:00:00
abstract：:Hemophilia A is an X-linked hereditary bleeding disorder due to the deficiency of coagulation factor VIII (FVIII). According to the degree of FVIII deficiency, mild, moderate or severe forms are recognized. Although patients with mild hemophilia A usually bleed excessively only after trauma or surgery, those with seve...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2013.06.002
更新日期：2013-07-01 00:00:00
abstract：:Congenital platelet disorders include thrombocytopathies and thrombocytopenias, which often occur in association. Thrombocytopathies constitute a model for exploring platelet physiology at the molecular level: adhesion, activation, release phenomena, aggregation. Further advances in understanding thrombocytopenias now...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(88)90004-5
更新日期：1988-03-01 00:00:00
abstract：:The anthracyclines are widely used in the treatment of haematological and non-haematological malignancy and there is now more than 30 years' clinical experience with these agents but despite this, their mechanism of action is incompletely understood. The anthracyclines have been shown to intercalate with DNA and indir...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/s0268-960x(97)90020-5
更新日期：1997-12-01 00:00:00
abstract：:Current genomic technologies have immensely improved disease classification and prognostication of major subtypes of B-cell lymphomas. This novel genetic information has not only aided in diagnosis, but has also revealed a landscape of critical molecular events that determine the biological and clinical behavior of a ...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2015.08.002
更新日期：2016-03-01 00:00:00
abstract：:Current trends in the treatment of patients with Stages I and II Hodgkin's disease are discussed in this review. Recommendations for staging procedures and the updated staging classification are described. Long-term results with extended field radiation therapy overall and in subgroups of patients are detailed. As fol...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(93)90022-v
更新日期：1993-03-01 00:00:00
abstract：:In refractory thrombocytopenia one should first evaluate whether the therapeutic approach has more risks to the patient than no treatment at all. The patient may remain relatively asymptomatic and the only incommoding circumstances be cosmetic. Coincidental medical problems such as hypertension and peptic ulceration a...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(88)90020-3
更新日期：1988-09-01 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：:Allogeneic stem cell transplantation (alloSCT) is the treatment of choice in the majority of young patients with advanced stages MDS if they have a suitable donor. Since outcome of transplantation is superior for patients with a low blast percentage, this supports the use of chemotherapy prior to transplantation in pa...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2006.03.002
更新日期：2007-01-01 00:00:00
abstract：:This relatively rare group of disorders may cause quite marked morbidity and occasionally be life-threatening. As their inheritance is largely known accurate information in one family member has obvious benefits to other family members as well as the patient. The identification of the defect is dependent on an accurat...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(90)90042-q
更新日期：1990-09-01 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：:This review sets out to synthesize and critically evaluate the current reported data regarding therapeutic options for the neutropenia associated with Felty syndrome (Felty neutropenia). A MEDLINE search and bibliographies from recent reviews were used to identify trials and case reports that provided sufficient data ...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/s0268-960x(96)90024-7
更新日期：1996-09-01 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：: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：:Platelets play a major role in primary hemostasis and thrombus formation. After vascular injury, platelets adhere to injured site and rapidly change their shape that switches the resting platelets to active state. Activated platelets aggregate and secrete biologically active intermediate substances that further potent...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2019.05.007
更新日期：2019-09-01 00:00:00
abstract：:After many unsuccessful years of searching, the first pathogenic human retrovirus, the human T-cell leukaemia lymphoma virus (HTLV-I), was reported as recently as 1980 and since that time has been causally linked to the adult T-cell leukaemia lymphoma syndrome. A second HTLV (HTLV-II) isolated shortly afterwards is le...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/0268-960x(88)90010-0
更新日期：1988-12-01 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：:Red blood cell (RBC) alloimmunization is a serious complication of blood transfusions, challenging selection of compatible units for future transfusions. Genetic characteristics may be associated with the risk of RBC alloimmunization and may therefore serve to identify high-risk patients. The aim of this systematic re...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2020.100794
更新日期：2021-01-05 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：:The innate immune system orchestrated by leukocytes primarily neutrophils, serves to remove dead and dying host cells and to provide protection against invasion by pathogens. Failure of this system results in the onset of sepsis leading to grave consequences for the host. Together with mechanical methods to physically...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2012.09.001
更新日期：2012-11-01 00:00:00
abstract：:Myeloid neoplasms like acute myeloid leukemia (AML) originate from genomic disruption, usually in a multi-step fashion. Hematopoietic stem/progenitor cell acquisition of abnormalities in vital cellular processes, when coupled with intrinsic factors such as germline predisposition or extrinsic factors such as the marro...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2020.100760
更新日期：2020-09-22 00:00:00
abstract：:Uncontrolled bleeding is the most common preventable cause of death for patients with severe injury. Coagulopathy inevitably accompanies severe bleeding, exacerbated by the ongoing blood loss and the treatments administered. There is debate about the underlying pathophysiological mechanisms of early traumatic coagulop...
journal_title：Blood reviews
pub_type： 杂志文章,评审
doi：10.1016/j.blre.2012.06.004
更新日期：2012-09-01 00:00:00
