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) therapy. Areas covered: This article reviews inhibitor eradication in clinically complicated hemophilia A patients. The benefits and disadvantages of ITI therapy are discussed, with reference to the new nonfactor replacement agents such as emicizumab, which are becoming available. Expert commentary: At present, ITI therapy is the method of choice for all inhibitor patients, but as alternative treatment options become increasingly available, this may change in the future. Despite the development of nonfactor replacement agents, ITI therapy is likely to maintain a key role in the management of inhibitor patients, although changes in ITI therapy regimens including the timing of ITI initiation, the dosing regimen, the management of thrombotic risk or safety, laboratory assessment of ITI outcomes, and maintenance treatment after ITI success, may be necessary.
journal_name
Expert Rev Hematoljournal_title
Expert review of hematologyauthors
Oldenburg J,Young G,Santagostino E,Escuriola Ettingshausen Cdoi
10.1080/17474086.2018.1521718subject
Has Abstractpub_date
2018-11-01 00:00:00pages
857-862issue
11eissn
1747-4086issn
1747-4094journal_volume
11pub_type
杂志文章,评审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:INTRODUCTION:Disorders of iron metabolism are commonly seen in onco-hematological clinical practice. Iron-deficiency anemia and cancer-associated anemia are usually treated with supportive therapies. Optimal management of these conditions are discussed in this perspective paper. Areas covered: A position paper discussi...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2017.1343140
更新日期:2017-08-01 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::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: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::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: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 mi...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1758555
更新日期:2020-06-01 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::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::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: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: ...
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: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: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::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::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:While associated life-threatening and fatal bleeding events are less frequent with the direct factor Xa inhibitors compared to vitamin K antagonists, significant concern surrounding management of major bleeds and urgent periprocedural interruption of these agents exists among clinicians. Andexanet alfa is ...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2018.1532287
更新日期:2018-11-01 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::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: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::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: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 de...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1080/17474086.2016.1249364
更新日期:2016-12-01 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::Objectives: CD43 can be useful in routine flow cytometry. We conducted a systematic review aiming to describe when CD43 is used by flow cytometry in malignant hematology and to determine its value in these settings. Methods: Systematic review of MEDLINE (search 'CD43' AND 'flow cytometry,' starting in 2010). Results: ...
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
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
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:Multiple myeloma (MM) is characterized by notable inter-patient and intra-clonal heterogeneity that is gradually decoded over the last decade. Despite the deeper and better understanding of its biology and the development of novel therapeutic strategies that have prolonged overall survival, MM still retain...
journal_title:Expert review of hematology
pub_type: 杂志文章,meta分析,评审
doi:10.1080/17474086.2018.1537776
更新日期:2018-11-01 00:00:00
abstract:INTRODUCTION:In the last decade, the availability of new drugs for the treatment of Multiple Myeloma (MM) significantly improved patients' outcomes, but also raised attention towards a new spectrum of adverse events. Recently, four novel agents with different mechanisms of action (carfilzomib, elotuzumab, daratumumab a...
journal_title:Expert review of hematology
pub_type: 杂志文章,meta分析,评审
doi:10.1080/17474086.2017.1284584
更新日期:2017-03-01 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: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::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