Abstract:
:Genetic defects of the blood platelet membrane glycoproteins, GPIIb-IIIa (alpha IIb/beta 3; CD41/CD61) and GPIb-V-IX (CD42) are the origin of several rare bleeding disorders, the best known of which are Glanzmann's thrombasthenia, Bernard-Soulier syndrome, and platelet-type von Willebrand's disease. In Glanzmann's thrombasthenia, GPIIb-IIIa are missing or defective and platelet aggregation is lacking or reduced. Either gene can be affected and mutations leading to lack of expression or to expression of poorly functional forms have been described. In Bernard-Soulier syndrome, GPIb-V-IX are missing or defective, leading to poor platelet adhesion at high-shear stress to damaged vessel wall and reduced platelet response to thrombin. Mutations in both GPIb alpha (CD42b) and GPIX (CD42a) have been described. Mutations in GPIb alpha can also lead to platelet-type von Willebrand's disease in which GPIb-V-IX are expressed normally but bind von Willebrand's factor spontaneously, which leads to platelet aggregation and thrombocytopenia.
journal_name
Curr Opin Hematoljournal_title
Current opinion in hematologyauthors
Clemetson KJ,Clemetson JMsubject
Has Abstractpub_date
1994-09-01 00:00:00pages
388-93issue
5eissn
1065-6251issn
1531-7048journal_volume
1pub_type
杂志文章,评审abstract:PURPOSE OF REVIEW:Hematopoietic stem cells are functionally heterogeneous even when isolated as phenotypically homogenous populations. How this heterogeneity is generated is incompletely understood. Several models have been formulated to explain the generation of diversity. All of these assume the existence of a single...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000231421.00407.65
更新日期:2006-07-01 00:00:00
abstract:PURPOSE OF REVIEW:New discoveries focused on mitochondrial metabolism and gene silencing and their regulation by the sirtuin family of protein deacetylases is stimulating new ideas on how to improve geriatric medicine. Information about sertuins in stem cell biology is scarce. We consider recent information on sirtuin ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283043819
更新日期:2008-07-01 00:00:00
abstract::The advent of safe and practical means to correct, enhance or protect blood cells at the genetic level offers tantalizing therapeutic perspectives. At present, gene delivery using a replication-defective retrovirus is the most efficient method to stably transduce hematopoietic cells. The successful adaptation of retro...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200011000-00008
更新日期:2000-11-01 00:00:00
abstract:PURPOSE OF REVIEW:The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by recurrent thrombosis and/or obstetrical morbidity in the presence of persistently positive antiphospholipid antibodies. Recent insights into the pathogenesis of APS have begun to elucidate pathophysiology and led to...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000067
更新日期:2014-09-01 00:00:00
abstract::Acute chest syndrome (ACS) is the presence of a new pulmonary infiltrate in combination with fever or respiratory symptoms in a patient with sickle cell disease. ACS is the leading cause of death in sickle cell disease, and many patients suffer from multiple, severe episodes. Age has a striking effect on the clinical ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199803000-00002
更新日期:1998-03-01 00:00:00
abstract:PURPOSE OF REVIEW:B-cell tumors originating from the transformation of germinal center B cells frequently harbor genetic mutations, leading to constitutive activation of the nuclear factor-κB (NF-κB) signaling pathway. The present review highlights recent insights into the roles of separate NF-κB transcription factors ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000160
更新日期:2015-07-01 00:00:00
abstract:PURPOSE OF REVIEW:Hematopoietic stem cells (HSCs) predominantly reside either in direct contact or in close proximity to the vascular endothelium throughout their lifespan. From the moment of HSC embryonic specification from hemogenic endothelium, endothelial cells (ECs) act as a critical cellular-hub that regulates a ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000350
更新日期:2017-07-01 00:00:00
abstract::Matching for human leukocyte antigen (HLA) loci is the most crucial factor in the selection of bone marrow donors. The techniques utilized to assign HLA type have evolved significantly since the application of polymerase chain reaction-based methodologies. A significant challenge for DNA typing methodologies is to be ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199811000-00012
更新日期:1998-11-01 00:00:00
abstract:PURPOSE OF REVIEW:To review the data on angiogenesis related to exosomes secreted by tumor cells in hematologic neoplasia and to elucidate the role of exosomes and exosomal miRNA in the bone marrow microenvironment, especially under hypoxic conditions. RECENT FINDINGS:Cross-talk between bone marrow tumor cells and sur...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000235
更新日期:2016-05-01 00:00:00
abstract:PURPOSE OF REVIEW:To outline the rationale, limitations, and execution of bloodless medical and surgical programs, highlighting characteristics that contribute to successful outcomes. RECENT FINDINGS:Clinical experiences with patients who refuse blood transfusions for religious reasons have provided valuable lessons a...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000245692.32085.bd
更新日期:2006-11-01 00:00:00
abstract::This article presents new insights into potential roles that three erythrocyte cytoskeletal proteins, protein 4.1, ankyrin, and spectrin, may play in nonerythroid nucleated cells. Each of these proteins is encoded by several closely related genes characterized by complex alternative splicing of their pre-mRNA, thus re...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200003000-00009
更新日期:2000-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Since the first discovery of microRNAs (miRNAs) in 1993, the involvement of miRNAs in different aspects of vascular disease has emerged as an important research field. In this review, we summarize the fundamental roles of miRNAs in controlling endothelial cell functions and their implication with seve...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000424
更新日期:2018-05-01 00:00:00
abstract:PURPOSE OF REVIEW:While the risks of transfusion-transmitted human immunodeficiency virus, hepatitis C virus, and human T-cell leukemia virus I/II continue to decrease, additional threats to transfusion safety are posed by emerging "new" infectious diseases. RECENT FINDINGS:Following the introduction of nucleic acid t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200311000-00003
更新日期:2003-11-01 00:00:00
abstract:PURPOSE OF REVIEW:Hematopoietic stem cells (HSCs) possess two fundamental characteristics, the capacity for self-renewal and the sustained production of all blood cell lineages. The fine balance between HSC expansion and lineage specification is dynamically regulated by the interplay between external and internal stimu...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000260
更新日期:2016-07-01 00:00:00
abstract:PURPOSE OF REVIEW:The presence of the Philadelphia chromosome causing the fusion between BCR to ABL1 in B cell precursor acute lymphoblastic leukemias (ALLs) was associated with a particularly bad prognosis, which has been markedly improved with the addition of imatinib to chemotherapy. Recent genomic studies have lead...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000050
更新日期:2014-07-01 00:00:00
abstract::The ability to move is one of the most basic and critical functions of the neutrophil. The neutrophil changes its shape from round to highly polar and glides along the surface, pausing every 45 to 60 seconds to reestablish its direction. Iterations of this sequence of small-scale motion, or shape change, sum to form t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199603010-00007
更新日期:1996-01-01 00:00:00
abstract::The bulk of antigens that are presented by major histocompatibility complex (MHC) class I molecules are processed in the cytosol. Therefore, the cellular protein degradation machinery is thought to play a major role in antigen processing. For example, there is clear evidence that the ubiquitin-proteasome pathway, the ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200101000-00003
更新日期:2001-01-01 00:00:00
abstract::Gene transfer has allowed a number of biologic issues in the therapy of hematologic malignancy to be addressed. In autologous bone marrow transplantation, gene marking studies have shown that infused marrow contributes to relapse in acute myeloid leukemia, neuroblastoma, and chronic myeloid leukemia. In addition, doub...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199502060-00004
更新日期:1995-11-01 00:00:00
abstract::Over a decade has passed since the first cases of AIDS were reported in hemophiliacs and transfusion recipients. Studies published over the past year offer an increasingly clear understanding of the extent of the early transfusion-associated AIDS epidemic, and of the effectiveness of progressive donor screening measur...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1994-11-01 00:00:00
abstract:PURPOSE OF REVIEW:This review focuses on recent advances in the use of immune-based therapy to treat patients with refractory and relapsing acquired thrombotic thrombocytopenic purpura. RECENT FINDINGS:Advances in understanding of the pathophysiology of idiopathic thrombotic thrombocytopenic purpura have provided the ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000170534.33517.99
更新日期:2005-09-01 00:00:00
abstract:PURPOSE OF REVIEW:Obesity has become a worldwide epidemic that is driving increased morbidity and mortality from thrombotic disorders such as myocardial infarction, stroke, and venous thromboembolism. Effective prevention and treatment of thrombosis in obese patients is limited by an incomplete understanding of the und...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283634443
更新日期:2013-09-01 00:00:00
abstract::Stem cells continue to attract considerable attention and provide hope for the development of new cell-based therapies for degenerative diseases. Unlocking the full therapeutic potential of stem cells requires an understanding of the mechanisms by which they are generated, self-renew, and differentiate. The applicatio...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200305000-00005
更新日期:2003-05-01 00:00:00
abstract:PURPOSE OF REVIEW:The review describes recent advances in our understanding of mechanisms leading to development of neutralizing antibodies following factor VIII (FVIII) replacement therapy for hemophilia A. Novel interventions with translational potential to lessen the incidence of these deleterious immune responses a...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e328356ed37
更新日期:2012-09-01 00:00:00
abstract::Within the past year substantial progress has been made in understanding the molecular changes underlying the development of acute leukemia. Development of molecular probes allowed substantial improvements in the detection of minimal residual disease. Pilot studies underlined the correlation between the expression of ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1994-07-01 00:00:00
abstract:PURPOSE OF REVIEW:Iron homeostasis and erythropoiesis regulate each other to ensure optimal delivery of oxygen and iron to cells and tissues. Defining the mechanisms of this crosstalk is important for understanding the pathogenesis of common conditions associated with disordered iron metabolism and erythropoiesis. REC...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000132
更新日期:2015-05-01 00:00:00
abstract:PURPOSE OF REVIEW:The discovery of JAK2V617F and other JAK-STAT-activating mutations in BCR-ABL1-negative myeloproliferative neoplasms (MPN) has led to the development of small-molecule ATP-mimetics that inhibit wild-type and mutant JAK. Here, we review the current experience with JAK inhibitors used for the treatment ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283439964
更新日期:2011-03-01 00:00:00
abstract::Therapy with unconjugated monoclonal antibodies (mAbs) and radiolabeled mAbs has shown activity in patients with B-cell non-Hodgkin's lymphoma and leukemia. Drug-conjugated mAbs are active in relapsed leukemia. Using these new agents with and after chemotherapy induces a high rate of remission, but this needs to be co...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199907000-00005
更新日期:1999-07-01 00:00:00
abstract::In the past few years, we have learned a great deal about the biologic function of structures bearing blood group antigens. Some blood group antigen-bearing proteins function as major transport channels within the erythrocyte membrane; these include the anion transporter (band 3: Diego and Wright antigens), the water ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199603060-00013
更新日期:1996-11-01 00:00:00
abstract:PURPOSE OF REVIEW:This review provides an update on advances in the area of iron chelation therapy, including new indications and uses of currently available agents, and preliminary data on potential new agents in development. RECENT FINDINGS:Two new oral agents, deferasirox and deferiprone, have become available in t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000031
更新日期:2014-05-01 00:00:00
abstract::Stem cell factor is an early-acting hematopoietic cytokine that probably functions constitutively to support the proliferation and survival of pluripotent progenitor cells and to increase their receptivity to lineage commitment and differentiation in response to lineage-specific cytokines. Stem cell factor also can in...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199603030-00010
更新日期:1996-05-01 00:00:00