Abstract:
INTRODUCTION:The discovery of the factor V Leiden (FVL) missense mutation (Arg506Gln) causing factor V resistance to the anticoagulant action of activated protein C was a landmark that allowed a better understanding of the basis of inherited thrombotic risk. FVL mutation is currently the most common known hereditary defect predisposing to venous thrombosis. Areas covered: Novel data-driven FVL diagnosis and therapeutic approaches in the management of FVL carriers in various clinical settings. Brief conclusions on topics of direct clinical relevance including currently available indications for primary and secondary prophylaxis, the management of female, pediatric carriers and asymptomatic relatives. Latest evidence on the association between FVL and cancer, as well as the possible use of direct oral anticoagulant therapy. Expert commentary: Although FVL diagnosis nowadays is highly accurate, many doubts remain regarding the best management and therapeutic protocols. The main role of clinicians is to tailor therapeutic strategies to carriers and their relatives. High familial penetrance, distinctive aspects of the first thrombotic event (provoked/unprovoked, age, etc.) and laboratory biomarkers can guide the optimal management of secondary antithrombotic prophylaxis, primary prophylaxis in asymptomatic individuals, and whether to screen relatives.
journal_name
Expert Rev Hematoljournal_title
Expert review of hematologyauthors
Campello E,Spiezia L,Simioni Pdoi
10.1080/17474086.2016.1249364subject
Has Abstractpub_date
2016-12-01 00:00:00pages
1139-1149issue
12eissn
1747-4086issn
1747-4094journal_volume
9pub_type
杂志文章,评审abstract:INTRODUCTION:Peripheral T cell lymphomas (PTCL) are a heterogenous group of lymphoproliferative disorders which are generally not curable with conventional chemotherapy and associated with inferior outcomes. Pralatrexate is a novel folate analog, the first FDA approved drug) for the treatment of relapsed/refractory (R/...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1756257
更新日期:2020-06-01 00:00:00
abstract:INTRODUCTION:Ferumoxytol is a superparamagnetic molecule originally developed as a contrast agent for magnetic resonance imaging. Elemental iron is contained within the carbohydrate core and is released slowly after infusion allowing a large dose of iron to be administered in a short period of time. Ferumoxytol, origin...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1518712
更新日期:2018-10-01 00:00:00
abstract::The fms-like tyrosine kinase 3 (FLT3) plays an important role in both normal and malignant hematopoiesis. Activating mutations in the FLT3 receptor can be detected in approximately 30% of acute myeloid leukemias (AMLs) and are associated with a distinctly poor clinical outcome for patients. There are now several class...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.1.2.153
更新日期:2008-12-01 00:00:00
abstract::Asparaginase has been a mainstay of therapy in the treatment of acute lymphoblastic leukemia since the 1970s. There are two major preparations available and FDA approved in the United States today, one derived from Escherichia coli and the other from Erwinia chrysanthemi. Erwinia asparaginase is antigenically distinct...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2016.1142370
更新日期:2016-03-01 00:00:00
abstract::In chronic lymphocytic leukemia (CLL), the most prevalent lymphoid malignancy in western countries, patients have a median age at diagnosis of 72 years. In the last few years, there has been remarkable progress in understanding the biology of CLL, the detection of molecular prognostic factors and the development of mo...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2013.814845
更新日期:2013-08-01 00:00:00
abstract:OBJECTIVES:We analyzed hemoglobin (Hb) levels and degree of anemia in relation to genotype in patients with hemoglobin H (Hb H) disease, thereby providing a scientific basis for the prevention and treatment of Hb H disease in the Guangxi region of China. METHODS:Hb analysis was conducted in 615 patients using high per...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1803736
更新日期:2020-09-01 00:00:00
abstract:BACKGROUND:Hb H disease is an alpha-thalassemia (α-thal) syndrome characterized by chronic hemolytic anemia that occurs when three of total four α-globin genes lost their function due to completely deletions or different kind of mutations. OBJECTIVE:We here described 66 patients who have been diagnosed for Hb H diseas...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/17474086.2016.1107470
更新日期:2016-01-01 00:00:00
abstract::Akt is a Ser-Thr kinase with pleiotropic effects on cell survival, growth and metabolism. Recent evidence from gene-deletion studies in mice, and analysis of human platelets treated with Akt inhibitors, suggest that Akt regulates platelet activation, with potential consequences for thrombosis. Akt activation is regula...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/ehm.09.75
更新日期:2010-02-01 00:00:00
abstract::Indolent non-Hodgkin's lymphoma (iNHL) describes a group of B-cell lymphomas with a long median survival and a relapsing-remitting clinical course. Although existing treatments are initially effective, patients often relapse, demonstrating decreasing efficacy with successive treatment courses. Alternative treatments a...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2015.1071663
更新日期:2015-10-01 00:00:00
abstract::Adult T-cell leukemia-lymphoma (ATL) is a peripheral T-cell malignancy, closely associated with human T-cell lymphotropic virus type I infection. Clinically, ATL is classified into four subtypes: acute, lymphoma, chronic and smoldering type. Although the prognosis of chronic and smoldering-type ATL is relatively good,...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.10.73
更新日期:2010-12-01 00:00:00
abstract:INTRODUCTION:Recent advances and drug approvals in the last decade have substantially changed the landscape of relapsed and refractory multiple myeloma (RRMM), which not only improved outcomes for patients but also increased complexity of treatment decisions. The approvals are based on randomized studies of novel agent...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1804356
更新日期:2020-09-01 00:00:00
abstract:INTRODUCTION:Hodgkin's lymphoma (HL) is largely a curable disease with excellent prognosis. The standard of care in patients with relapsed disease has been to try salvage chemotherapy followed by an autologous stem cell transplantation (ASCT). Managing the patients who relapse after ASCT, is challenging. With the appro...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1449637
更新日期:2018-04-01 00:00:00
abstract:INTRODUCTION:Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) are considered indolent lymphocytic malignancies, more often requiring active surveillance rather than intervention. Despite the indolent nature of CLL/SLL, treatment is likely indicated in a patients' lifetime. Recent changes in the t...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1270203
更新日期:2017-02-01 00:00:00
abstract:INTRODUCTION:Human term placenta is comprised of various tissues from which different cells can be obtained, including hematopoietic stem cells and mesenchymal stem/stromal cells (MSCs). Areas covered: This review will discuss the possibility to incorporate placental tissue cells in cord blood banking. It will discuss ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1483717
更新日期:2018-08-01 00:00:00
abstract::Many patients with acute myeloid leukemia will eventually develop refractory or relapsed disease. In the absence of standard therapy for this population, there is currently an urgent unmet need for novel therapeutic agents. Targeted therapy with small molecule inhibitors represents a new therapeutic intervention that ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2014.932687
更新日期:2014-08-01 00:00:00
abstract:INTRODUCTION:Mitochondria are cellular organelles that perform numerous bioenergetic, biosynthetic, and regulatory functions and play a central role in iron metabolism. Extracellular iron is taken up by cells and transported to the mitochondria, where it is utilized for synthesis of cofactors essential to the function ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2016.1268047
更新日期:2017-01-01 00:00:00
abstract:INTRODUCTION:Immune surveillance is the dynamic process whereby the immune system identifies and kills tumor cells based on their aberrant expression of stress-related surface molecules or presentation of tumor neoantigens. It plays a crucial role in controlling the initiation and progression of hematologic cancers suc...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1755958
更新日期:2020-06-01 00:00:00
abstract::Over two-thirds of newly diagnosed multiple myeloma are over 65 years. The treatment goals for the non-transplant-eligible patients should be to prolong survival by achieving the best response, while ensuring quality of life. New upfront treatment combinations based on first generation of novel proteasome inhibitors a...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2014.864230
更新日期:2014-02-01 00:00:00
abstract::Tumor lysis syndrome (TLS) is a rare but potentially life-threatening complication of neoplasms, preferentially hematological malignancies. Well known since at least ninety years ago, this condition can be misdiagnosed and incorrectly managed due to rapid onset of symptoms, sometimes overlapping with cancer-derived cl...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2016.1127156
更新日期:2016-01-01 00:00:00
abstract::Human acute myeloid leukemia is a heterogeneous disease and the effect of therapeutic targeting of specific molecular mechanisms will probably vary between patient subsets. Cell cycle regulators are among the emerging targets (e.g., aurora and polo-like kinases, cyclin-dependent kinases). Inhibition of communication b...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2015.1032935
更新日期:2015-06-01 00:00:00
abstract:INTRODUCTION:Chronic Myeloid Leukemia (CML) is a myeloproliferative disorder that has become the neoplastic poster child for understanding the disease biology of a malignant disease and targeting effective therapy. The targeted therapy of BCR-ABL inhibition by tyrosine kinase inhibitors (TKI) has provided the epitome f...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2016.1232163
更新日期:2016-10-01 00:00:00
abstract:INTRODUCTION:Ruxolitinib is a potent and selective JAK1/JAK2 inhibitor that has shown superiority as compared to available conventional chemotherapies, in terms of reduction in splenomegaly and improvement of symptoms and quality of life. Areas covered: Data published about overall survival in the major randomized spon...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1273766
更新日期:2017-02-01 00:00:00
abstract::Introduction: Oral anticoagulants (OACs) are established treatments to reduce thromboembolic risk in patients with atrial fibrillation (AF). Due to their mechanisms of action, they are associated with increased bleeding risk. Evaluation with bleeding risk scores may be useful to identify patients at high risk. However...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2019.1647779
更新日期:2019-10-01 00:00:00
abstract:INTRODUCTION:Purine analogs made dramatic improvements for patients with hairy cell leukemia (HCL), but patients often relapse, require multiple treatments, and may become refractory. Major developments in treatment of relapsed/refractory HCL occurred with discovery of disease biology. New agents increase the complexit...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1819231
更新日期:2020-10-01 00:00:00
abstract::Recombinant IFN-α (rIFN-α) induces complete hematologic remissions in patients with myeloproliferative neoplasms (MPNs), but its use has been limited by side effects owing to the relatively high doses used. Now, low-dose rIFN-α is stressed, starting relatively early in the course of the MPNs. In polycythemia vera, thi...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/ehm.12.69
更新日期:2013-02-01 00:00:00
abstract:INTRODUCTION:Activation of the hemostatic system can occur in many clinical conditions. However, a systemic and strong activation of coagulation complicating clinical settings such as sepsis, trauma or malignant disease may result in the occurrence disseminated intravascular coagulation (DIC). Areas covered: This artic...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1500173
更新日期:2018-08-01 00:00:00
abstract::Hemophilia A is a congenital, recessive, X-linked bleeding disorder that is managed with infusions of plasma-derived or recombinant factor (F) VIII. The primary considerations in FVIII replacement therapy today are the: 1) immunogenicity of FVIII concentrates, 2) role of longer-acting FVIII products, 3) prophylactic u...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2014.899896
更新日期:2014-06-01 00:00:00
abstract::Adult immune thrombocytopenia was previously considered a benign disease affecting young people and with a low risk of severe bleeding. This view was challenged by studies published during the past decade, as the median age of adult immune thrombocytopenia patients has been found to be 55-60 years and the incidence in...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.12.7
更新日期:2012-04-01 00:00:00
abstract:INTRODUCTION:Inhibitors against factor VIII (FVIII), which develop in around 20-30% of patients with severe hemophilia A, represent a significant complication of on-demand or prophylactic FVIII therapy. Currently, the main treatment option for inhibitor patients is eradication using immune tolerance induction (ITI) the...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1521718
更新日期:2018-11-01 00:00:00
abstract::Internal tandem duplication mutations of FMS-like tyrosine kinase-3 (FLT3) have been associated with poor outcomes in acute myelogenous leukemia. Over the course of the last several years, multiple agents have been developed and studied as potential inhibitors of FLT3 with the hope of providing clinical benefit for th...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2.1.17
更新日期:2009-02-01 00:00:00