Abstract:
INTRODUCTION:Myeloproliferative neoplasms (MPNs) are associated with a variety of symptoms and signs which cause major morbidity for the patients. The disorders are associated with increased incidence of thromboembolic and hemorrhagic events which can lead to complications and shortened life expectancy. Areas covered: Using systematic literature review and expert clinical and research experience the authors discuss strategies for the management of symptoms and signs including pruritus, fatigue, splenomegaly, and cytopenia. Cytoreduction including treatments to inhibit the JAK/STAT pathway are considered. Pathogenesis and prevention and treatment of thrombotic and hemorrhagic events and their management is addressed and the suggested management of the special situations such as surgery and pregnancy are discussed. Expert commentary: Management of disease has traditionally focused on symptom treatment and complication prevention but the discovery of driver mutations has led to treatments aiming to eliminate the clone, which should be the ultimate goal of therapy. A future challenge is to develop safe and effective MPN therapy and to personalize therapy.
journal_name
Expert Rev Hematoljournal_title
Expert review of hematologyauthors
Greenfield G,McMullin MFdoi
10.1080/17474086.2018.1410433subject
Has Abstractpub_date
2018-01-01 00:00:00pages
25-35issue
1eissn
1747-4086issn
1747-4094journal_volume
11pub_type
杂志文章,评审abstract::Here we review the recent literature on Hemophilia gene transfer/therapy. Gene therapy is one of several new technologies being developed as a treatment for bleeding disorders. We will discuss current and pending clinical efforts and attempt to relate how the field is trending. In doing so, we will focus on the use of...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2016.1182859
更新日期:2016-07-01 00:00:00
abstract::Venous thromboembolism is a common cause of morbidity and mortality among patients undergoing elective orthopedic surgery. Due to the high incidence of venous thromboembolism in this setting, perioperative anticoagulation is the recommended approach for thromboprophylaxis. Low molecular weight heparin (LMWH), fondapar...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2013.853430
更新日期:2013-12-01 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: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::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::It is well known that the risk of venous thromboembolism is increased after surgery; however, specific data on how long this risk lasts or how the risk varies by type of surgery is limited. The Million Women Study is a population-based prospective study that recruited 1.3 million women through the National Health Serv...
journal_title:Expert review of hematology
pub_type: 评论,杂志文章
doi:10.1586/ehm.10.6
更新日期:2010-04-01 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: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: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::Recent studies have identified recurrent mutations in genes that encode proteins crucial in the epigenetic regulation of gene transcription in hematologic malignancies. Somatic mutations in epigenetic modifiers, including IDH1, IDH2, TET2, DNAMT3A, ASXL1, MLL and EZH2 are enriched in patients with acute myeloid leukem...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2016.1144469
更新日期:2016-05-01 00:00:00
abstract::Introduction: The genomic landscape of Waldenström macroglobulinemia (WM) is characterized by recurrent MYD88 (MYD88L265P) and CXCR4 mutations (CXCR4MUT), detected in 90% and 30% of cases, respectively. The role of CXCR4MUT in clinical features and outcomes to therapy in WM patients is evolving. Areas covered: We perf...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2019.1649132
更新日期:2019-10-01 00:00:00
abstract::Introduction: There is emerging evidence linking the commensal gut microbiota with the development of cardiovascular disease and arterial thrombosis. In immunothrombosis, the host clotting system protects against the dissemination of invading microbes, not considering the huge number of microbes that interact with hos...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2019.1627191
更新日期:2019-07-01 00:00:00
abstract:INTRODUCTION:Iliopsoas muscle hemorrhage in patients with hemophilia is a serious complication with relevant related morbidity. AREAS COVERED:A Cochrane Library and PubMed (MEDLINE) search of studies related to iliopsoas hematomas in hemophilia was analyzed. Pain in the hip joint area and femoral nerve involvement in ...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1787146
更新日期:2020-08-01 00:00:00
abstract::Ofatumumab is a humanized second-generation monoclonal antibody with the affinity to a transmembrane protein CD20. In in vitro studies, it exhibits higher efficacy towards chronic lymphocytic leukemia (CLL) cells compared to rituximab, and it can be explained by the fact that its epitope on the target CD20 protein is ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2015.1037736
更新日期:2015-06-01 00:00:00
abstract:INTRODUCTION:Waldenström's macroglobulinemia (WM) is a rare, incurable B-cell lymphoma, with a median survival of 5-10 years in symptomatic patients. There is no consensus on the standard of care and several agents are currently used in these patients. Areas covered: In this article, we will review the use of standard ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1339596
更新日期:2017-07-01 00:00:00
abstract::Introduction: Severe sickle cell disease is associated with progressive end-organ damage and early mortality in adults. While allogeneic hematopoietic cell transplant from a matched related donor is curative, the vast majority of patients do not have a compatible sibling. Accordingly, platforms using haploidentical do...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2019.1642103
更新日期:2019-09-01 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::The introduction of bortezomib, a first-generation proteasome inhibitor, changed the standard-of-care for newly diagnosed and relapsed multiple myeloma patients. The next generation of proteasome inhibitors, such as carfilzomib, provides a novel pharmacokinetic and pharmacodynamic profile. In vitro data suggest a more...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/ehm.12.26
更新日期:2012-08-01 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: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::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: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:BACKGROUND:Polycythemia occurs in 1 to 5% of neonates and is associated with complications, including an increased risk of thrombocytopenia. OBJECTIVE:To evaluate incidence, risk factors, management and outcome of thrombocytopenia in neonates with polycythemia. STUDY DESIGN:All neonates with polycythemia admitted to ...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/17474086.2015.997705
更新日期:2015-02-01 00:00:00
abstract::Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumption thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cl...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2019.1611423
更新日期:2019-06-01 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::Anti-CD20 monoclonal antibodies (mAbs), rituximab, ofatumumab and obinutuzumab, have a significant impact in the treatment of chronic lymphocytic leukemia (CLL), particularly in combination with chemotherapy. Over the last few years, several new mAbs have been developed and investigated in CLL. The most promising newe...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2014.963048
更新日期:2014-12-01 00:00:00
abstract:INTRODUCTION:Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1300522
更新日期:2017-04-01 00:00:00
abstract::Intravenous iron is standard for dialysis-associated anemia and its use is rising dramatically in other settings. Except for the dextrans, full iron replacement requires multiple visits. Nonetheless, safety concerns abound. Ferumoxytol, a recently approved modified dextran with a carbohydrate core that tightly binds t...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.11.31
更新日期:2011-08-01 00:00:00
abstract:INTRODUCTION:Primary plasma cell leukemia (PPCL) is one of the most aggressive hematological malignancies. The prognosis of PPCL patients remains poor, although some improvements have been made in recent years. Areas covered: In this review recent clinical and biological advances in PPCL are reported. Some recommendati...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2019.1598258
更新日期:2019-04-01 00:00:00
abstract::Thromboembolic diseases require anticoagulation for their prevention and treatment. New oral anticoagulants, specifically direct factor Xa and thrombin inhibitors, were developed to overcome the limitations of conventional anticoagulants. Their benefit has been demonstrated using fixed doses without laboratory-guided ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.11.79
更新日期:2012-02-01 00:00:00