Abstract:
PURPOSE OF REVIEW:This review focuses on recent advances in the use of immune-based therapy to treat patients with refractory and relapsing acquired thrombotic thrombocytopenic purpura. RECENT FINDINGS:Advances in understanding of the pathophysiology of idiopathic thrombotic thrombocytopenic purpura have provided the rationale for immune-based treatment approaches to refractory and relapsing cases of thrombotic thrombocytopenic purpura. The demonstration that a severe deficiency of von Willebrand factor-cleaving protease activity in acquired thrombotic thrombocytopenic purpura is due to an autoantibody inhibitor of the von Willebrand factor-cleaving protease argues for targeting the antibody inhibitor of the von Willebrand factor-cleaving protease with immunosuppressive therapy as a means of increasing protease activity and inducing a sustained remission of the disease. Recent publications, largely case reports and small series of patients, have suggested a role for rituximab and cyclosporine in cases refractory to plasma exchange or resistant to the tapering of plasma exchange therapy. SUMMARY:In the future, a better understanding of the relation between the von Willebrand factor-cleaving protease activity, the nature and titer of von Willebrand factor-cleaving protease inhibitors, and the risk of relapse of thrombotic thrombocytopenic purpura will be important to further validate the strategy of targeting the von Willebrand factor-cleaving protease inhibitory antibody as an effective means of treating acquired thrombotic thrombocytopenic purpura. Prospective data from carefully designed clinical trials are needed to define the effectiveness of specific immunosuppressive therapies at suppressing antibody inhibitors of von Willebrand factor-cleaving protease, improving von Willebrand factor-cleaving protease activity, and inducing a sustained remission of the disease.
journal_name
Curr Opin Hematoljournal_title
Current opinion in hematologyauthors
Cataland SR,Wu HMdoi
10.1097/01.moh.0000170534.33517.99keywords:
subject
Has Abstractpub_date
2005-09-01 00:00:00pages
359-63issue
5eissn
1065-6251issn
1531-7048pii
00062752-200509000-00002journal_volume
12pub_type
杂志文章,评审abstract::Acute chest syndrome (ACS) is the presence of a new pulmonary infiltrate in combination with fever or respiratory symptoms in a patient with sickle cell disease. ACS is the leading cause of death in sickle cell disease, and many patients suffer from multiple, severe episodes. Age has a striking effect on the clinical ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199803000-00002
更新日期:1998-03-01 00:00:00
abstract::A variety of agents are available to improve hemostasis and reduce blood loss in multiple clinical settings. These agents are most commonly used to reduce bleeding when an underlying hemostatic defect is present. Some new agents offer the potential to decrease blood loss even in the absence of an obvious underlying he...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200211000-00014
更新日期:2002-11-01 00:00:00
abstract::The reconstitution of T-cell populations is a critical component of immune recovery after allogeneic stem cell transplantation. Recent studies have used new techniques to focus on the interplay of thymopoiesis and peripheral expansion that defines T-cell repopulation. Peripheral expansion, driven by host cytokines and...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200211000-00004
更新日期:2002-11-01 00:00:00
abstract:PURPOSE OF REVIEW:The aim of this short study is to review recently published data bearing on how to resuscitate massive uncontrolled hemorrhage. RECENT FINDINGS:New data inform our understanding of the mechanisms of the acute coagulopathy of trauma, the median time to death of trauma patients with uncontrolled hemorr...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000184
更新日期:2015-11-01 00:00:00
abstract:PURPOSE OF REVIEW:To review the data on angiogenesis related to exosomes secreted by tumor cells in hematologic neoplasia and to elucidate the role of exosomes and exosomal miRNA in the bone marrow microenvironment, especially under hypoxic conditions. RECENT FINDINGS:Cross-talk between bone marrow tumor cells and sur...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000235
更新日期:2016-05-01 00:00:00
abstract:PURPOSE OF REVIEW:Neutrophils priming has been long studied in vitro. Recent studies describe it in vivo. In pathophysiological conditions, complex, heterogeneous characteristics of priming are described in the last few years. RECENT FINDINGS:Priming can occur systemically when insults such as sepsis or trauma result ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000471
更新日期:2019-01-01 00:00:00
abstract:PURPOSE OF REVIEW:B-cell tumors originating from the transformation of germinal center B cells frequently harbor genetic mutations, leading to constitutive activation of the nuclear factor-κB (NF-κB) signaling pathway. The present review highlights recent insights into the roles of separate NF-κB transcription factors ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000160
更新日期:2015-07-01 00:00:00
abstract:PURPOSE OF REVIEW:A major goal in repopulating hematopoietic stem cell (HSC) gene therapies is achieving high-efficacy gene transfer, while maintaining robust HSC engraftment and differentiation in vivo. Recent studies have documented that rapamycin treatment of HSC during lentiviral vector transduction enhances gene t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000150
更新日期:2015-07-01 00:00:00
abstract:PURPOSE OF REVIEW:This article summarizes recent insights into the pathophysiology of immune thrombocytopenic purpura, a disorder in which autoantibodies against cell-specific glycoproteins (GPIIb-IIIa, GPIb-IX and others) accelerate platelet destruction. RECENT FINDINGS:Autoantibodies are produced by a limited number...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3282ba5552
更新日期:2007-09-01 00:00:00
abstract::Considerable data now support the hypothesis that platelets actively regulate the propagation of coagulation by (1) expressing specific, high-affinity receptors for coagulation proteases, zymogens, and cofactors; (2) protecting the bound coagulation enzymes from inactivation/inhibition; (3) restricting coagulant activ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200109000-00001
更新日期:2001-09-01 00:00:00
abstract::Matching for human leukocyte antigen (HLA) loci is the most crucial factor in the selection of bone marrow donors. The techniques utilized to assign HLA type have evolved significantly since the application of polymerase chain reaction-based methodologies. A significant challenge for DNA typing methodologies is to be ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199811000-00012
更新日期:1998-11-01 00:00:00
abstract::Cytomegalovirus (CMV) infection remains a major cause of morbidity and mortality in recipients of an allogeneic stem cell transplant (SCT). Due to the broad application of antiviral prophylaxis and preemptive therapy, a decrease in early-onset and a subsequent increase in late-onset CMV disease has been observed. New ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199811000-00022
更新日期:1998-11-01 00:00:00
abstract:PURPOSE OF REVIEW:The role of a patent foramen ovale (PFO) as a risk factor for ischemic stroke has been established in recent years. However, the best therapeutic option to prevent recurrent events is still controversial, with antithrombotic treatment or transcatheter PFO closure being favored by different clinicians....
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32832d47dd
更新日期:2009-09-01 00:00:00
abstract::This article presents new insights into potential roles that three erythrocyte cytoskeletal proteins, protein 4.1, ankyrin, and spectrin, may play in nonerythroid nucleated cells. Each of these proteins is encoded by several closely related genes characterized by complex alternative splicing of their pre-mRNA, thus re...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200003000-00009
更新日期:2000-03-01 00:00:00
abstract::Platelet glycoprotein (GP) IIb/IIIa receptor antagonists are being used with increasing frequency in the settings of percutaneous coronary interventions and acute ischemic syndromes. The development of bleeding complications following GPIIb/IIIa blockade represents a significant limitation to its effectiveness. Baseli...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199909000-00011
更新日期:1999-09-01 00:00:00
abstract::Multiple myeloma is the second most common hematologic malignancy, with approximately 15,000 new cases each year in the United States. Our understanding of the pathophysiology underlying myeloma continues to expand, but the cause of this plasma cell dyscrasia remains unclear. Though controversy remains regarding a pos...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200007000-00007
更新日期:2000-07-01 00:00:00
abstract:PURPOSE OF REVIEW:Microtransplantation (MST) has been successfully used to manage patients with hematological malignancies, especially older patients with acute myeloid leukemia (AML). Recent clinical applications and mechanistic studies of MST are reviewed. RECENT FINDINGS:MST improves complete remission rates which ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000470
更新日期:2018-11-01 00:00:00
abstract::In the past few years, we have learned a great deal about the biologic function of structures bearing blood group antigens. Some blood group antigen-bearing proteins function as major transport channels within the erythrocyte membrane; these include the anion transporter (band 3: Diego and Wright antigens), the water ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199603060-00013
更新日期:1996-11-01 00:00:00
abstract::Human parvovirus B19 shows remarkable tropism for human erythroid progenitor cells. The pathogenesis of disease resulting from infection is a dynamic between the viral suppression of erythropoiesis and the host's ability to mount an effective immune response. Understanding this process has led to strategies for treati...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199603020-00002
更新日期:1996-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Clonal heterogeneity is a significant obstacle to successful treatment of patients with acute myeloid leukemia (AML). Here, we review new advances in the understanding of genetic heterogeneity in AML using single-cell DNA-sequencing technology. RECENT FINDINGS:New genomics and immunologic discovery t...
journal_title:Current opinion in hematology
pub_type: 杂志文章
doi:10.1097/MOH.0000000000000626
更新日期:2021-01-01 00:00:00
abstract:PURPOSE OF REVIEW:The review describes recent advances in our understanding of mechanisms leading to development of neutralizing antibodies following factor VIII (FVIII) replacement therapy for hemophilia A. Novel interventions with translational potential to lessen the incidence of these deleterious immune responses a...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e328356ed37
更新日期:2012-09-01 00:00:00
abstract::Allogeneic barriers to transfusion are caused by differences between those portions of the donor and recipient genomes that define the antigenicity and immune response to the transfused cells. Historically, a blood group antigen was identified when an immune response (alloantibody) was detected by hemagglutination in ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200203000-00012
更新日期:2002-03-01 00:00:00
abstract::The Epstein-Barr virus is a ubiquitous human herpesvirus that is associated with an increasing number of human malignancies. Among these are Epstein-Barr virus-associated lymphoproliferative diseases in immunocompromised patients, a spectrum of mainly B-cell diseases that range from polyclonal lymphoproliferative dise...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199901000-00005
更新日期:1999-01-01 00:00:00
abstract:PURPOSE OF REVIEW:To highlight mechanisms that regulate the balance between latent and active von Willebrand factor (VWF), and describe pathological conditions leading to increased levels of active VWF. RECENT FINDINGS:Levels of circulating active VWF are increased in von Willebrand disease type 2B, HELLP syndrome, ma...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3280dce531
更新日期:2007-05-01 00:00:00
abstract::The ability to move is one of the most basic and critical functions of the neutrophil. The neutrophil changes its shape from round to highly polar and glides along the surface, pausing every 45 to 60 seconds to reestablish its direction. Iterations of this sequence of small-scale motion, or shape change, sum to form t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199603010-00007
更新日期:1996-01-01 00:00:00
abstract:PURPOSE OF REVIEW:Core binding factor acute myeloid leukemia (CBF-AML) corresponds to two distinct subtypes of AML characterized by recurrent favorable chromosome translocations, namely t(8;21) and inv(16)/t(16;16). Given the relatively good outcome of patients with CBF-AML, when treated with intensive chemotherapy inc...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283257b18
更新日期:2009-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Bone marrow transplantation is the only curative treatment for severe thalassemia. Since its successful first report in 1981, more than 4000 patients with this disease worldwide underwent bone marrow transplantation. The purpose of this review is to update the most recent reports of matched sibling do...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000286
更新日期:2016-11-01 00:00:00
abstract:PURPOSE OF REVIEW:This review provides an update on advances in the area of iron chelation therapy, including new indications and uses of currently available agents, and preliminary data on potential new agents in development. RECENT FINDINGS:Two new oral agents, deferasirox and deferiprone, have become available in t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000031
更新日期:2014-05-01 00:00:00
abstract::Chronic myelogenous leukemia is a clonal hematopoietic malignancy characterized by a balanced translocation between chromosomes 9 and 22 that results in the generation of an abnormal bcr/abl fusion protein with increased tyrosine kinase activity. This abnormal fusion protein has transforming activity for hematopoietic...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199502040-00009
更新日期:1995-07-01 00:00:00
abstract:PURPOSE OF REVIEW:Technical possibilities to screen for inborn errors of immune function at the neonatal stage have been rapidly progressing, whereas the guidelines that apply for the evaluation of benefits and concerns on expanding screening panels have not been broadly discussed for primary immunodeficiency diseases ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32835a9130
更新日期:2013-01-01 00:00:00