Abstract:
:Each year, approximately one in 1000 individuals suffers from venous thromboembolism. The pathogenesis of the disease is multifactorial and a thrombotic event is the result of a combination of genetic and circumstantial risk factors. Until recently, genetic defects could only be identified in a minority of thrombosis patients. The discovery of inherited resistance to activated protein C as a risk factor for thrombosis changed the situation for the better. In Western countries, activated protein C resistance is found in 20% to 60% of patients with thrombosis. Activated protein C resistance is caused by a single point mutation in the Factor V gene, leading to replacement of Arg(R)506 in the activated protein C cleavage site of Factor V with a Gln(Q). As a result, the activated protein C-mediated cleavage and inhibition of mutated Factor V (FV:Q506) is impaired, which leads to increased thrombin generation, a hypercoagulable state, and a life-long increased risk of thrombosis.
journal_name
Curr Opin Hematoljournal_title
Current opinion in hematologyauthors
Zöller B,Dahlbäck Bdoi
10.1097/00062752-199502050-00006subject
Has Abstractpub_date
1995-09-01 00:00:00pages
358-64issue
5eissn
1065-6251issn
1531-7048journal_volume
2pub_type
杂志文章,评审abstract:PURPOSE OF REVIEW:In addition to its essential role in baseline erythropoiesis, the hormone erythropoietin drives the erythropoietic response to hypoxic stress. A mechanistic understanding of stress erythropoiesis would benefit multiple clinical settings, and may aid in understanding leukemogenesis. RECENT FINDINGS:Th...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3280de2bf1
更新日期:2007-05-01 00:00:00
abstract:PURPOSE OF REVIEW:There are three kindlin family members in vertebrates, which have high-sequence homology and a common organization signature with a C-terminal 4.1, ezrin, radixin, moesin (FERM) domain bisected by a pleckstrin-homology domain. Although the cell and tissue distributions of the three kindlins differ, th...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32832ea389
更新日期:2009-09-01 00:00:00
abstract:PURPOSE OF REVIEW:Hematopoietic stem cell (HSC) transplantation is the most powerful treatment modality for a variety of hematological disorders. Successful hematopoietic recovery after transplantation depends on optimal homing of HSCs to the bone marrow and subsequent lodging in the HSC niche. The molecular mechanisms...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32834760e5
更新日期:2011-07-01 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:The advent of tyrosine kinase inhibitors (TKI) has remarkably changed the treatment of chronic myeloid leukemia (CML). Patients treated in chronic phase can enjoy an expected survival similar to the general population. In the last few years we have found that some patients with an excellent therapeuti...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000224
更新日期:2016-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Angioimmunoblastic T cell lymphoma is a complex lymphoproliferative disorder. While recent evidence suggests that the Epstein-Barr virus and B cell disregulation are implicated in the disease's pathogenesis, their mechanistic roles remain largely unknown. The prognosis with traditional chemotherapy ha...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e328186ffbf
更新日期:2007-07-01 00:00:00
abstract::Hematopoietic stem cells are the cells primarily responsible for short term and long term hematological reconstitution when a graft is infused into a myeloablated host. The number and quality of hematopoietic stem cells within a graft are the major determinants of the time to and durability of hematological reconstitu...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199905000-00010
更新日期:1999-05-01 00:00:00
abstract::The risk of transmission of viruses and other pathogenic organisms by blood can be reduced but not eliminated through donor selection and screening methodologies. The safety of blood derivatives prepared from plasma pools from thousands of donors requires virus inactivation, without which transmission rates approach c...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199502060-00014
更新日期:1995-11-01 00:00:00
abstract::Several methods to model human Ph+ leukemia in laboratory mice are available, including propagation of BCR/ABL-expressing cells in mice, xenotransplantation of primary Ph+ leukemia cells into immunodeficient mice, BCR/ABL transgenic mice, and BCR/ABL retroviral bone marrow transduction and transplantation. Recent stud...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200107000-00008
更新日期:2001-07-01 00:00:00
abstract::Myeloablative therapy followed by autologous bone marrow or peripheral blood stem cell rescue is an alternative therapeutic option for patients who are not candidates for allogeneic transplantation due to the lack of an appropriate HLA-matched donor or to advanced age. It allows therapy to be administered that is of c...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199704060-00011
更新日期:1997-11-01 00:00:00
abstract::Over the past year, substantial progress was made toward understanding transcriptional control of red cell differentiation. Complementary DNAs encoding two novel erythroid-restricted transcription factors--globin locus control region regulatory factor NF-E2 and CACC-binding protein EKLF--were cloned and characterized....
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1994-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Vasculogenesis and hematopoiesis are essential for development. Recently, the ETS domain transcription factor Etv2 has been identified as an essential regulator of vasculogenesis and hematopoiesis. Here, we review the recent studies that have established the critical role of Etv2 in the specification ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283523e07
更新日期:2012-05-01 00:00:00
abstract::Although the mature enucleated erythrocyte is no longer active in nuclear processes such as pre-mRNA splicing, the function of many of its major structural proteins is dependent on alternative splicing choices made during the earlier stages of erythropoiesis. These splicing decisions fundamentally regulate many aspect...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200103000-00003
更新日期:2001-03-01 00:00:00
abstract::Chronic myelogenous leukemia is a clonal hematopoietic malignancy characterized by a balanced translocation between chromosomes 9 and 22 that results in the generation of an abnormal bcr/abl fusion protein with increased tyrosine kinase activity. This abnormal fusion protein has transforming activity for hematopoietic...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199502040-00009
更新日期:1995-07-01 00:00:00
abstract::Platelet glycoprotein (GP) IIb/IIIa receptor antagonists are being used with increasing frequency in the settings of percutaneous coronary interventions and acute ischemic syndromes. The development of bleeding complications following GPIIb/IIIa blockade represents a significant limitation to its effectiveness. Baseli...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199909000-00011
更新日期:1999-09-01 00:00:00
abstract:PURPOSE OF REVIEW:Graft-versus-host disease is one of the commonest complications of allogeneic bone marrow or peripheral blood stem cell transplantation. This review will cover advances in the pathophysiology of graft-versus-host disease and new agents under investigation for the treatment of this disorder. Patients d...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000148763.12733.bb
更新日期:2005-01-01 00:00:00
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 thr...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1994-09-01 00:00:00
abstract:PURPOSE OF REVIEW:The peptide hormone hepcidin regulates iron metabolism in response to erythropoietic demand, iron stores and inflammation. Major advances have been made in understanding the regulation of hepcidin production, and consequently the availability of iron for erythropoiesis. RECENT FINDINGS:It is becoming...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3282f73335
更新日期:2008-05-01 00:00:00
abstract:PURPOSE OF REVIEW:Fibroblast growth factor (FGF) signaling activates many bone marrow cell types, including various stem cells, osteoblasts, and osteoclasts. However, the role of FGF signaling in regulation of normal and leukemic stem cells is poorly understood. This review highlights the physiological roles of FGF sig...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283606162
更新日期:2013-05-01 00:00:00
abstract::The reconstitution of T-cell populations is a critical component of immune recovery after allogeneic stem cell transplantation. Recent studies have used new techniques to focus on the interplay of thymopoiesis and peripheral expansion that defines T-cell repopulation. Peripheral expansion, driven by host cytokines and...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200211000-00004
更新日期:2002-11-01 00:00:00
abstract:PURPOSE OF REVIEW:Inconsistent results regarding the clinical efficacy of granulocyte transfusions for the treatment or prophylaxis of life-threatening infections in neutropenic patients have been attributed to insufficient number of transfused neutrophils. Since the introduction of granulocyte colony-stimulating facto...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000203
更新日期:2016-01-01 00:00:00
abstract:PURPOSE OF REVIEW:The leukocyte adhesion deficiency (LAD) syndromes are rare genetically determined conditions with challenging clinical features. These immunodeficiencies also provide insights that are broadly relevant to the biology of leukocytes, platelets, intercellular interactions, and intracellular signaling. Re...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32835a0091
更新日期:2013-01-01 00:00:00
abstract:PURPOSE OF REVIEW:Early T-cell precursor (ETP) leukaemias have been recently recognized as a form of T-cell acute lymphoblastic leukaemia (T-ALL) with a poor prognosis. The purpose of this review is to outline the most recent advances in the biology, genetics and prognostic significance of this aggressive disease. REC...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283623c61
更新日期:2013-07-01 00:00:00
abstract:PURPOSE OF REVIEW:Therapy-related myelodysplastic syndromes and acute myelogenous leukemia (t-MDS/AML) comprise an increasingly common, poor-risk disease cohort without standard treatment options. This review describes available treatments and recent advances that may influence the clinical management of t-MDS/AML. RE...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32835d82e6
更新日期:2013-03-01 00:00:00
abstract:PURPOSE OF REVIEW:The treatment of patients with refractory or relapsed acute myeloid leukemia remains challenging. Management of these patients must take into account patient and leukemia-related factors in order to organize a comprehensive approach to treatment. Many new therapies are under study. RECENT FINDINGS:Ne...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32801684ef
更新日期:2007-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Risk assessments for transmission of variant Creutzfeldt-Jakob disease predicted that leukocyte reduction would be inefficient at preventing transmission of the disease by blood transfusion. Nevertheless, approximately 14 years ago, a significant proportion of European countries decided to implement l...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000101
更新日期:2015-01-01 00:00:00
abstract::Fibrinogen abnormalities can be classified as congenital or acquired. Each class manifests quantitative or qualitative alterations; the latter are known as dysfibrinogenemias. In dysfibrinogenemias, structural defects cause alterations in the conversion of fibrinogen to fibrin. Approximately 300 abnormal fibrinogens h...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199704050-00010
更新日期:1997-09-01 00:00:00
abstract:PURPOSE OF REVIEW:The proteome is the pool of proteins expressed at a given time and circumstance. The word 'proteomics' summarizes several technologies for visualization, quantitation and identification of these proteins. Recent advances in these techniques are helping to elucidate platelet processes which are relevan...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32832e9dc6
更新日期:2009-09-01 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:PURPOSE OF REVIEW:A major goal in repopulating hematopoietic stem cell (HSC) gene therapies is achieving high-efficacy gene transfer, while maintaining robust HSC engraftment and differentiation in vivo. Recent studies have documented that rapamycin treatment of HSC during lentiviral vector transduction enhances gene t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000150
更新日期:2015-07-01 00:00:00