Abstract:
PURPOSE OF REVIEW:The leukocyte adhesion deficiency (LAD) syndromes are rare genetically determined conditions with challenging clinical features. These immunodeficiencies also provide insights that are broadly relevant to the biology of leukocytes, platelets, intercellular interactions, and intracellular signaling. Recent discoveries merit their review in the context of existing knowledge. RECENT FINDINGS:New activities of β(2) integrins, which are deficient or absent in LAD-I, and new β(2) integrin-dependent functions of neutrophils and other leukocytes have recently been identified. Genetic defects and mechanisms accounting for impaired fucosylation of selectin ligands and defective selectin binding and signaling in LAD-II are now apparent. LAD-III, which presents with bleeding similar to that in Glanzmann thrombasthenia and platelet dysfunction in addition to impaired leukocyte adhesion, is now known to be due to absence of KINDLIN-3, a cytoplasmic protein that acts cooperatively with TALIN-1 in activating β(1), β(2), and β(3) integrins. Understanding of the leukocyte adhesion cascade and interactions of leukocytes with inflamed endothelium, which are impaired in each of the LAD syndromes, continues to be refined. SUMMARY:Although LAD syndromes are rare maladies, their investigation is generating new knowledge directly applicable to the diagnosis and care of patients and to fundamental paradigms in immunobiology and hemostasis.
journal_name
Curr Opin Hematoljournal_title
Current opinion in hematologyauthors
Harris ES,Weyrich AS,Zimmerman GAdoi
10.1097/MOH.0b013e32835a0091subject
Has Abstractpub_date
2013-01-01 00:00:00pages
16-25issue
1eissn
1065-6251issn
1531-7048pii
00062752-201301000-00005journal_volume
20pub_type
杂志文章,评审abstract:PURPOSE OF REVIEW:In addition to its essential role in baseline erythropoiesis, the hormone erythropoietin drives the erythropoietic response to hypoxic stress. A mechanistic understanding of stress erythropoiesis would benefit multiple clinical settings, and may aid in understanding leukemogenesis. RECENT FINDINGS:Th...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3280de2bf1
更新日期:2007-05-01 00:00:00
abstract:PURPOSE OF REVIEW:In addition to the crucial role of vascular endothelial growth factor (VEGF)A in vessel development, it has become apparent that the VEGF signaling pathway (VSP) plays an important role during adulthood in the maintenance of tissue homeostasis in normal physiological processes, as well as in pathologi...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32833865e6
更新日期:2010-05-01 00:00:00
abstract:PURPOSE OF REVIEW:This review is designed to help readers understand seasonal blood shortages and provide solutions through the use of technology that can increase the number of red blood cell units collected and the use of recruitment and marketing initiatives that appeal to the increasingly diverse donor base. RECEN...
journal_title:Current opinion in hematology
pub_type: 杂志文章
doi:10.1097/01.moh.0000180436.98990.ce
更新日期:2005-11-01 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:The discovery of JAK2V617F and other JAK-STAT-activating mutations in BCR-ABL1-negative myeloproliferative neoplasms (MPN) has led to the development of small-molecule ATP-mimetics that inhibit wild-type and mutant JAK. Here, we review the current experience with JAK inhibitors used for the treatment ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283439964
更新日期:2011-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Intravenous immunoglobulin (IVIg) is an effective treatment for an increasing number of autoimmune and inflammatory conditions. However, IVIg continues to be limited by problems of potential shortages and cost. A number of mechanisms have been described for IVIg, which have been captured in newly emer...
journal_title:Current opinion in hematology
pub_type: 杂志文章
doi:10.1097/MOH.0000000000000609
更新日期:2020-11-01 00:00:00
abstract:PURPOSE OF REVIEW:The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by recurrent thrombosis and/or obstetrical morbidity in the presence of persistently positive antiphospholipid antibodies. Recent insights into the pathogenesis of APS have begun to elucidate pathophysiology and led to...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000067
更新日期:2014-09-01 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:Hematopoietic stem cells (HSCs) possess two fundamental characteristics, the capacity for self-renewal and the sustained production of all blood cell lineages. The fine balance between HSC expansion and lineage specification is dynamically regulated by the interplay between external and internal stimu...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000260
更新日期:2016-07-01 00:00:00
abstract:PURPOSE OF REVIEW:To examine the current literature regarding retrievable inferior vena cava filters and to discuss the appropriate indications for their clinical use. RECENT FINDINGS:Permanent filters have been shown to be effective, but have a number of long-term complications such as filter thrombosis or migration....
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000239707.63168.ee
更新日期:2006-09-01 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::Recent studies have improved our understanding of the cytogenesis, biology, and therapy of chronic lymphocytic leukemia (CLL). This review highlights this recent progress reported over the past year. We have improved our understanding of the cytogenetic abnormalities in CLL and soon may see identification of new tumor...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199807000-00003
更新日期:1998-07-01 00:00:00
abstract:PURPOSE OF REVIEW:Endothelial cells dysfunctions are crucial determinants of several human diseases. We review here the most recent reports on endothelial cell defects in cerebral cavernous malformations (CCMs), particularly focusing on adherens junctions. CCM is a vascular disease that affects specifically the venous ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000338
更新日期:2017-05-01 00:00:00
abstract::Contrast venography remains the gold standard for the diagnosis of deep-vein thrombosis (DVT). However, an ongoing interest exists in developing safe diagnostic strategies that are less invasive, less expensive, widely and readily available, and more acceptable to patients than venography. For patients with symptoms s...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199909000-00001
更新日期:1999-09-01 00:00:00
abstract::Human immunodeficiency virus infection causes multilineage hematopoietic defects. Defects in the production and function of CD4+ helper cells have been the focus of the majority of HIV research, but anemia, neutropenia, and thrombocytopenia are significant clinical problems as well. Bone marrow suppression is the dose...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199502030-00012
更新日期:1995-05-01 00:00:00
abstract:PURPOSE OF REVIEW:New discoveries focused on mitochondrial metabolism and gene silencing and their regulation by the sirtuin family of protein deacetylases is stimulating new ideas on how to improve geriatric medicine. Information about sertuins in stem cell biology is scarce. We consider recent information on sirtuin ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283043819
更新日期:2008-07-01 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:Deficiency of antithrombin, protein C, and protein S increases the risk of a first venous thromboembolism (VTE) by at least 10-fold and is rare (i.e., <0.5% of population), whereas factor V Leiden and the prothrombin G20210A gene increase this risk by 2-5-fold and are common (2-5% of whites). Antiphos...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e328356745b
更新日期:2012-09-01 00:00:00
abstract:PURPOSE OF REVIEW:The presence of the Philadelphia chromosome causing the fusion between BCR to ABL1 in B cell precursor acute lymphoblastic leukemias (ALLs) was associated with a particularly bad prognosis, which has been markedly improved with the addition of imatinib to chemotherapy. Recent genomic studies have lead...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000050
更新日期:2014-07-01 00:00:00
abstract::Leukocytes use an array of antimicrobial peptides and proteins to help them destroy invading microorganisms. These endogenous antibiotic molecules are remarkable for their structural variety, rapid evolutionary divergence, intraspecies variation, and complex yet subtle interactions with their targets. This arsenal has...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199704010-00009
更新日期:1997-01-01 00:00:00
abstract::Much progress has been made in delineating the pathogenesis of multiple myeloma and chronic lymphocytic leukemia. The cell of origin in both diseases has been better defined, which has led to important clinical treatments. For myeloma, reduction of tumor burden in autografts has been accomplished and been associated w...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199603040-00007
更新日期:1996-07-01 00:00:00
abstract:PURPOSE OF REVIEW:To discuss how mechanical cues and Rho signaling contribute to control of vascular development and hematopoiesis. RECENT FINDINGS:Rho guanine trinucleotide phosphatases are ubiquitious regulators of cytoskeletal structure and tension generation. Recent work shows that Rho-dependent mechanical interac...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3282fa7445
更新日期:2008-05-01 00:00:00
abstract::Platelet production is primarily regulated by the thrombopoietic cytokine thrombopoietin (TPO). In most cases thrombopoietin serum levels are determined by the rate of c-mpl receptor-mediated degradation after TPO uptake into platelets and megakaryocytes. The contribution of increased TPO protein synthesis by a transl...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200005000-00003
更新日期:2000-05-01 00:00:00
abstract:PURPOSE OF REVIEW:Despite improvements in the achievement of complete remission and progress in the supportive care of adults with acute lymphoblastic leukemia during the last decade, the majority of patients have eventually relapsed with overall survival in adult acute lymphoblastic leukemia of only 30-40%. However, t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283034697
更新日期:2008-07-01 00:00:00
abstract::Neutropenia is often attributed to immunologically mediated injury to mature neutrophils or their precursors. Clinically it is useful to classify immune mediated neutropenias as isoimmune, autoimmune (including some drug-associated neutropenias), and idiopathic (cases possibly with an immune mechanism). Isoimmune neut...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199801000-00006
更新日期:1998-01-01 00:00:00
abstract:PURPOSE OF REVIEW:Type 1 von Willebrand disease (VWD) is the most common form of VWD, but has remained [corrected] the least well understood. Recent work is changing this situation. This review summarizes recent analysis of the genetic basis of the disease. RECENT FINDINGS:Linkage analysis demonstrates that dominantly...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32826f4b41
更新日期:2007-09-01 00:00:00
abstract:PURPOSE OF REVIEW:The purpose of this review is to summarize the recent experimental and clinical literature on the pathogenesis of transfusion-related acute lung injury (TRALI). RECENT FINDINGS:In both experimental and clinical TRALI, an immune priming step is generally necessary to produce lung injury. Experimental ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32833c07d3
更新日期:2010-09-01 00:00:00
abstract::Fibrinogen abnormalities can be classified as congenital or acquired. Each class manifests quantitative or qualitative alterations; the latter are known as dysfibrinogenemias. In dysfibrinogenemias, structural defects cause alterations in the conversion of fibrinogen to fibrin. Approximately 300 abnormal fibrinogens h...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199704050-00010
更新日期:1997-09-01 00:00:00
abstract:PURPOSE OF REVIEW:Lymphocyte trafficking is regulated by adhesion molecules mediating the initial shear-resistant binding of circulating cells to target tissue endothelium (Step 1). This review focuses on the current and emerging perspectives of the biology of these 'homing receptors.' RECENT FINDINGS:The conventional...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000177827.78280.79
更新日期:2005-11-01 00:00:00
abstract:PURPOSE OF REVIEW:Hematopoietic cell transplantation offers potentially curative therapy for patients with myelodysplastic syndrome. The median patient age at diagnosis, however, is about 70 years, which has limited the application of conventional hematopoietic cell transplantation. In addition, the optimum timing of t...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000208466.63861.9f
更新日期:2006-03-01 00:00:00