Abstract:
INTRODUCTION:Pulmonary hypertension (PH) develops in a significant number of patients with sickle cell disease (SCD), resulting in increased morbidity and mortality. This review focuses on PH pathophysiology, risk stratification, and new recommendations for screening and treatment for patients with SCD. Areas covered: An extensive PubMed literature search was performed. While the pathophysiology of PH in SCD is yet to be fully deciphered, it is known that the etiology is multifactorial; hemolysis, hypercoagulability, hypoxemia, ischemic-reperfusion injury, oxidative stress, and genetic susceptibility all contribute in varying degrees to endothelial dysfunction. Hemolysis, in particular, seems to play a key role by inciting an imbalance in the regulatory axis of nitric oxide and arginine metabolism. Systematic risk stratification starting in childhood based on clinical features and biomarkers that enable early detection is necessary. Multi-faceted, targeted interventions, before irreversible vasculopathy develops, will allow for improved patient outcomes and life expectancy. Expert commentary: Despite progress in our understanding of PH in SCD, clinically proven therapies remain elusive and additional controlled clinical trials are needed. Prevention of disease starts in childhood, a critical window for intervention. Given the complex and multifactorial nature of SCD, patients will ultimately benefit from combination therapies that simultaneously targets multiple mechanisms.
journal_name
Expert Rev Hematoljournal_title
Expert review of hematologyauthors
Shilo NR,Morris CRdoi
10.1080/17474086.2017.1364989subject
Has Abstractpub_date
2017-10-01 00:00:00pages
875-890issue
10eissn
1747-4086issn
1747-4094journal_volume
10pub_type
杂志文章,meta分析,评审abstract::In 2008, we reviewed the practical interface between transfusion medicine and the surgery and critical care of severely injured patients. Reviewed topics ranged from epidemiology of trauma to patterns of resuscitation to the problems of transfusion reactions. In the interim, trauma specialists have adopted damage cont...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.11.49
更新日期:2011-10-01 00:00:00
abstract::Myeloid sarcoma (MS) is an extramedullary myeloid neoplasm characterized by proliferation of myeloblasts which can occur in any organ or site. Bronchial and pulmonary involvement, however, is uncommon. We describe a case of bronchial MS in an 81-year-old female with a history of high-grade myelodysplastic syndrome; sh...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/17474086.2015.1044747
更新日期:2015-08-01 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::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::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::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
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::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 incidence of non-Hodgkin's lymphoma (NHL) is increasing among all age groups, with a median age at diagnosis of 67 years. With the increase in the geriatric population, there is a need for the development and validation of treatment strategies for NHL for these patients. Therapy in elderly patients is affected by ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.09.7
更新日期:2009-04-01 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::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:Guidelines provide recommendations on the management of multiple myeloma (MM), but there are no standard algorithms for the choice and sequencing of treatments. As a result, there is widespread variation in the interpretation and implementation of these guidelines. Areas covered: This review will cover: th...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1437345
更新日期:2018-03-01 00:00:00
abstract::Although guidelines exist to deal with some aspects of platelet transfusion practice, many important clinical issues have not been addressed in large randomized controlled trials (RCTs). Slichter et al. conducted a RCT of prophylactic platelet transfusions to determine the effects of the dose of platelets on clinical ...
journal_title:Expert review of hematology
pub_type: 评论,杂志文章
doi:10.1586/ehm.10.36
更新日期:2010-08-01 00:00:00
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: ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1410433
更新日期:2018-01-01 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:Most patients with Hodgkin lymphoma (HL) enjoy durable remissions following front-line treatment but 30% of patients are refractory or relapse after first line therapy. Salvage chemotherapy followed by autologous stem cell transplantation (ASCT) can cure an additional 50-55% of relapsing patients but new t...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1313701
更新日期:2017-05-01 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: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::Introduction: The purpose of this article is to review which data about hemophilia are currently provided by the Cochrane database of systematic reviews (CDBSR). Methodological consideration: All statements about hemophilia in the Cochrane Collaboration are based on evidence generated in randomized controlled clinical...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2019.1676718
更新日期:2019-11-01 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::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::Professor John Gribben is Chair of the International Workshop on non-Hodgkin Lymphoma and the Gordon Hamilton Fairley Chair of Medical Oncology at St. Bartholomew's Hospital, Bart's Cancer Institute, London, UK, a Cancer Research UK Centre of Excellence. His doctoral studies were performed at University College London...
journal_title:Expert review of hematology
pub_type: 面试
doi:10.1586/ehm.13.13
更新日期:2013-04-01 00:00:00
abstract:INTRODUCTION:Haploidentical stem cell transplantation (Haplo-SCT) is currently a suitable alternative worldwide for patients with hematological diseases, who lack human leukocyte antigen (HLA)-matched siblings or unrelated donors. Areas covered: This review summarizes the advancements in Haplo-SCT in recent years, prim...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1447379
更新日期:2018-04-01 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: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:Large granular lymphocytic leukemia (LGLL) is a low grade lymphoproliferative disorder characterized by the clonal proliferation of large granular lymphocytes (LGL) and recognised by the WHO. The diagnosis and management of these patients is challenging due to the limited information from prospective studi...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1284585
更新日期:2017-03-01 00:00:00
abstract::Chronic immune thrombocytopenia (ITP) is an autoimmune disorder characterized by a low platelet count that has persisted for more than 12 months. Patients with severe, symptomatic disease may have significant morbidity and require treatment. Historically, the underlying cause of ITP was believed to be accelerated plat...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/ehm.11.25
更新日期:2011-06-01 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: 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