Abstract:
INTRODUCTION:In sickle cell disease (SCD), hemoglobin S (HbS) red blood cells (RBCs) are characteristically deformed and inflexible. Often breaking down in the circulation, they exhibit increased adhesive properties with the endothelium and activated neutrophils and platelets, increasing the risk of occlusion of the microcirculation. SCD is categorized into two sub-phenotypes: hyperhemolytic, associated with priapism, leg ulcers, pulmonary hypertension, and stroke, and high hemoglobin/viscosity, which may promote vaso-occlusion-associated pain, acute chest syndrome, and osteonecrosis. AREAS COVERED:The sub-phenotypes are not completely distinct. Hemolysis may trigger vaso-occlusion, contributing to vascular complications. Targeting P-selectin, a key mediator of cross-talk between hyperhemolysis and vaso-occlusion, may be beneficial for vascular and vaso-occlusion-associated complications. English-language articles from PubMed on the topic of SCD and vaso-occlusive crises (VOCs) were reviewed from 1 January 2000 to 1 January 2019 using the search terms 'sickle cell disease,' 'vaso-occlusive crises,' and 'selectin.' EXPERT OPINION:Besides targeting P-selectin, other strategies to counter VOCs and RBC sickling are being pursued. These include platelet inhibition to counter aggregation, intercellular adhesion, and thrombosis during VOCs; gene therapy to correct the homozygous missense mutation in the β-globin gene, causing polymerization of HbS; L-glutamine, possibly reducing oxidative stress in sickled RBCs; and fetal hemoglobin inducers.
journal_name
Expert Rev Hematoljournal_title
Expert review of hematologyauthors
Ofori-Acquah SFdoi
10.1080/17474086.2020.1758555subject
Has Abstractpub_date
2020-06-01 00:00:00pages
645-653issue
6eissn
1747-4086issn
1747-4094journal_volume
13pub_type
杂志文章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: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:INTRODUCTION:Treatment of elderly patients with acute leukemia is challenging. Older age is associated with increased risk of treatment-related toxicity. Currently, no consensus exists regarding optimal therapy in this patient population. Areas covered: The following review is a comprehensive summary of various therape...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1382345
更新日期:2017-11-01 00:00:00
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: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: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: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: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::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
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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::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::Introduction: Venous thromboembolism (VTE) is a frequent and serious complication in cancer patients. Nonetheless, patients with hematological cancers receive less attention as compared with their solid tumor counterparts regarding this potentially fatal complication.Areas covered: Risk factors that are associated wit...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1751608
更新日期:2020-05-01 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::Despite significant improvements in treatment modalities over the 10 years, the clinical course of patients with follicular lymphoma (FL) remains heterogeneous. Thus, prognostic indexes are still required to direct treatment choices and for the design of clinical trials. Investigators have conducted a variety of studi...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.09.34
更新日期:2009-10-01 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::Iron overload is frequently observed in patients with hematologic diseases before and after allogeneic stem-cell transplantation because they usually receive multiple red blood cell transfusions. Elevated pretransplant serum ferritin levels, which are widely used as indicators of body iron status, are significantly as...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.10.81
更新日期:2011-02-01 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::Introduction: Secondary central nervous system lymphoma (SCNSL) is a potentially fatal event in the setting of aggressive Non-Hodgkin Lymphomas. Nowadays, despite of the very poor outcome of SCNSL, several studies are going to identify the high-risk patients' subgroup that could early develop this detrimental event an...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2019.1643232
更新日期:2019-09-01 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: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: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
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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
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journal_title:Expert review of hematology
pub_type: 杂志文章,meta分析,评审
doi:10.1080/17474086.2017.1364989
更新日期:2017-10-01 00:00:00
abstract::Relapsed or refractory classical Hodgkin lymphoma (HL) remains a therapeutic challenge. Patients with relapsed HL should be identified according to their prognostic factors at relapse (duration of remission and extranodal disease or stage). Patients with refractory disease, defined as progression during induction trea...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.09.12
更新日期:2009-06-01 00:00:00
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journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2017.1398080
更新日期:2017-12-01 00:00:00
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journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2021.1856653
更新日期:2021-01-01 00:00:00
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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::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::Major advances have significantly improved the outcome of mantle cell lymphoma (MCL). Incorporation of rituximab to CHOP regimen, the adoption of high dose cytarabine with frontline autologous stem cell transplantation in young patients, maintenance rituximab or bortezomib based chemotherapy in elderly patients, impro...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/17474086.2016.1134309
更新日期:2016-03-01 00:00:00