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)-cleaving protease. Areas covered: VWF/ADAMTS13 interaction is specific, and ADAMTS13 conformation has been elucidated recently. ADAMTS13 deficiency is congenital or acquired. Several targets are used in TTP treatment. Expert opinion: Differential diagnosis of TTP may be challenging and ADAMTS13 investigations are needed. ADAMTS13 conformation could be a promising parameter for TTP diagnosis and prognosis in the coming years. Plasma therapy remains the first-line treatment of the acute phase of TTP. Additional curative and preemptive rituximab are used in acquired TTP. Clinical trials dedicated to innovative drugs are promising.
journal_name
Expert Rev Hematoljournal_title
Expert review of hematologyauthors
Joly BS,Coppo P,Veyradier Adoi
10.1080/17474086.2019.1611423subject
Has Abstractpub_date
2019-06-01 00:00:00pages
383-395issue
6eissn
1747-4086issn
1747-4094journal_volume
12pub_type
杂志文章,评审abstract::Allogeneic hematopoietic stem cell transplantation is still the only potentially curative treatment for patients with myelodysplastic syndromes. Improvements in donor selection, supportive care and the introduction of reduced-intensity conditioning have led to a decrease in early transplant mortality. However, relapse...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.11.58
更新日期:2011-12-01 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::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::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::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: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: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::Hematopoietic stem cells can be mobilized out of the bone marrow into the blood for the reconstitution of hematopoiesis following high-dose therapy. Methods to improve mobilization efficiency and yields are rapidly emerging. Traditional methods include chemotherapy with or without myeloid growth factors. Plerixafor, a...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.09.54
更新日期:2009-12-01 00:00:00
abstract::Introduction: Myelodysplastic Syndrome (MDS) represents a group of cancers characterized by abnormal blood cell formation and maturation, leading to various degrees of cytopenias and potential transformation to acute myeloid leukemia. Deletion of the long arm of chromosome 5 (del(5q)) is the most common clonal chromos...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2020.1730806
更新日期:2020-03-01 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: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::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: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: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: 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::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::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: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: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::β-thalassemias are caused by nearly 300 mutations of the β-globin gene, leading to a low or absent production of adult hemoglobin (HbA). Two major therapeutic approaches have recently been proposed: gene therapy and induction of fetal hemoglobin (HbF) with the objective of achieving clinically relevant levels of Hbs. ...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1586/ehm.13.24
更新日期:2013-06-01 00:00:00
abstract::Key paper evaluation: Craddock C, et al. Combination Lenalidomide and Azacitidine: A Novel Salvage Therapy in Patients Who Relapse After Allogeneic Stem-Cell Transplantation for Acute Myeloid Leukemia. J Clin Oncol. 2019; 37: 580-8. Allogeneic hematopoietic stem cell transplant (allo-HSCT) is the only potentially cura...
journal_title:Expert review of hematology
pub_type: 杂志文章
doi:10.1080/17474086.2019.1635005
更新日期:2019-08-01 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::Hemopoietic stem-cell transplant patients may require intensive blood component support. Complications of transfusions include transmission of viral and bacterial infections, transfusion-associated graft-versus-host disease and transfusion-related acute lung injury. Alloimmunization to red cell antigens may cause diff...
journal_title:Expert review of hematology
pub_type: 杂志文章,评审
doi:10.1586/ehm.11.14
更新日期:2011-04-01 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: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: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::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: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: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::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