Abstract:
:Peripheral blood B cells in patients with paroxysmal nocturnal hemoglobinuria (PNH) comprise variable mixtures of normal B cells produced before the onset of disease and glycosylphosphatidylinositol (GPI)-deficient B cells derived from the PNH hematopoietic stem cell. In a detailed phenotypic analysis of 29 patients with PNH, this study shows consistent phenotypic differences between PNH B cells and residual normal B cells. In the majority of patients with active disease, PNH B cells comprised mainly naive cells with a CD27(-)IgM(+)IgD(strong+)IgG(-) phenotype. The proportion of CD27(+) memory cells within this compartment was related to disease duration (Spearman [r(s)] 0.403; P =.030). In PNH patients with predominantly GPI-deficient hematopoiesis, that is, a large granulocyte PNH clone, the residual normal B cells had a predominantly memory (CD27(+)) phenotype. Furthermore, the majority of these memory B cells were not immunoglobulin (Ig) class switched and had an IgM(+)IgD(+)IgG(-) phenotype. Using PNH as a novel model with which to study B lymphopoiesis, this study provides direct evidence that production of new naive B cells occurs throughout life and that the major population of long-lived memory B cells are IgM(+)IgD(+). Moreover, studies of GPI(-) B cells in 2 patients in remission from PNH suggest that the life span of a B-cell clone can be more than 24 years.
journal_name
Bloodjournal_title
Bloodauthors
Richards SJ,Morgan GJ,Hillmen Psubject
Has Abstractpub_date
2000-11-15 00:00:00pages
3522-8issue
10eissn
0006-4971issn
1528-0020journal_volume
96pub_type
杂志文章相关文献
BLOOD文献大全abstract::Azacitidine treatment of myelodysplastic syndromes (MDSs) generally exacerbates thrombocytopenia during the first treatment cycles. A Study of Eltrombopag in Myelodysplastic Syndromes Receiving Azacitidine (SUPPORT), a phase 3, randomized, double-blind, placebo-controlled study, investigated the platelet supportive ef...
journal_title:Blood
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1182/blood-2018-06-855221
更新日期:2018-12-20 00:00:00
abstract::There is now strong experimental evidence that tissue factor pathway inhibitor (TFPI) is a critical inhibitor to modulate tissue factor-induced coagulation, but the role of TFPI as a risk factor for thrombosis is yet to be to be determined. This study investigated the role of low TFPI levels for the development of dee...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2002-10-3188
更新日期:2003-06-01 00:00:00
abstract::The efficacy and safety of zanolimumab in patients with refractory cutaneous T-cell lymphoma (CTCL) have been assessed in two phase 2, multicenter, prospective, open-label, uncontrolled clinical studies. Patients with treatment refractory CD4(+) CTCL (mycosis fungoides [MF], n = 38; Sézary syndrome [SS], n = 9) receiv...
journal_title:Blood
pub_type: 杂志文章,多中心研究
doi:10.1182/blood-2006-12-062877
更新日期:2007-06-01 00:00:00
abstract::Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare primary immunodeficiency caused by gain-of-function mutations in the CXCR4 gene. We report the safety, tolerability, pharmacokinetics, pharmacodynamics, and preliminary efficacy of mavorixafor from a phase 2 open-label dose-escalatio...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2020007197
更新日期:2020-12-24 00:00:00
abstract::The MLL gene located at 11q23 is frequently disrupted by chromosomal translocation in a wide spectrum of newly diagnosed acute leukemias. Recently, it has become apparent that the MLL gene is very frequently disrupted by chromosomal translocations in patients with secondary leukemias associated with chemotherapeutic r...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1996-04-01 00:00:00
abstract::Hydroxyurea treatment of sickle cell mice improved their survival from pneumococcal pneumonia, counteracting the abnormally elevated inflammatory response and reducing invasion of bacteria into the bloodstream, through down regulation of E-selectin. ...
journal_title:Blood
pub_type: 评论,杂志文章
doi:10.1182/blood-2011-12-397794
更新日期:2012-02-23 00:00:00
abstract::The effect of cyclosporine A (CyA) on the ability of the human immunodeficiency virus type 1 (HIV-1) to infect the H-9 T-cell leukemic line, as well as interleukin-2 (IL-2)-grown human peripheral blood-derived lymphocytes, has been studied. Pretreatment of H-9 cells and human lymphocytes with CyA over 24 hours complet...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1988-12-01 00:00:00
abstract::Interleukin (IL)-23 is a proinflammatory cytokine belonging to the IL-12 superfamily. The antitumor activity of IL-23 is controversial, and it is unknown whether or not the cytokine can act directly on tumor cells. The aim of this study was to investigate the potential direct antitumor activity of IL-23 in pediatric B...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2009-10-248245
更新日期:2010-11-11 00:00:00
abstract::CC chemokine receptor (CCR) 9, the receptor for the CC-chemokine CCL25/thymus-expressed chemokine (TECK), is mainly expressed by thymocytes and by intraepithelial (IEL) and lamina propria lymphocytes of the small intestine. To study the biologic role of CCR9, a mouse strain was generated in which the CCR9 gene was del...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v98.9.2626
更新日期:2001-11-01 00:00:00
abstract::Escherichia coli were labeled with 59Fe and then either treated with myeloperoxidase, H2O2, and chloride or opsonized and mixed with human neutrophils. The myeloperoxidase system at pH 7.4 caused release of most of the bacterial 59Fe. A similar result has been obtained by Rosen and Klebanoff (J Biol Chem 257:13731, 19...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1990-02-15 00:00:00
abstract::Neutrophils play a crucial early role during the innate response, but little is known about their possible contribution when an adaptive immune response is installed. A robust neutrophilia and a T helper 1 (Th1) immune response are present after immunization with Complete Freund Adjuvant (CFA). We show that when FITC-...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-04-016659
更新日期:2006-11-01 00:00:00
abstract::In an attempt to improve outcomes for patients with acute myeloid leukemia (AML) after allogeneic hematopoietic cell transplantation (HCT), we conducted a phase 1/2 study in which targeted irradiation delivered by 131I-anti-CD45 antibody was combined with targeted busulfan (BU; area-under-curve, 600-900 ng/mL) and cyc...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2005-06-2317
更新日期:2006-03-01 00:00:00
abstract::Liver X receptors (LXRs) are transcription factors involved in the regulation of cholesterol homeostasis. LXR ligands have athero-protective properties independent of their effects on cholesterol metabolism. Platelets are involved in the initiation of atherosclerosis and despite being anucleate express nuclear recepto...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-09-306142
更新日期:2011-05-26 00:00:00
abstract::Lymphomas were studied in kindreds with autoimmune lymphoproliferative syndrome (ALPS; Canale-Smith syndrome), a disorder of lymphocyte homeostasis usually associated with germline Fas mutations. Fas (CD95/APO-1) is a cell surface receptor that initiates programmed cell death, or apoptosis, of activated lymphocytes. L...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v98.1.194
更新日期:2001-07-01 00:00:00
abstract::The belief is widely held, on the basis of indirect evidence, that a substantial, even brief elevation of red cell Ca content must result in a marked shortening of circulatory survival. To test this notion directly, we exposed rabbit red cells in vitro to the ionophore A23187 and Ca so as to produce sustained uniform ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1985-07-01 00:00:00
abstract::During pregnancy the plasma concentration of two different inhibitors of plasminogen activators (PAIs) increases. The only one found in the plasma of nonpregnant women (PAI1) is immunologically related to a PAI of endothelial cells; its plasma activity, as deduced from the inhibition of single-chain tissue-type plasmi...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1987-02-01 00:00:00
abstract::Graft-versus-host disease (GVHD) remains one of the major complications after allogeneic bone marrow transplantation (allo-BMT). Sirtuin-1 (Sirt-1) plays a crucial role in various biological processes including cellular senescence, metabolism, and inflammatory responses. Sirt-1 deacetylation regulates different transc...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2018-07-863233
更新日期:2019-01-17 00:00:00
abstract::Delivery of biologically active peptides into human polymorphonuclear neutrophils (PMNs) has implications for studying cellular functions and may be therapeutically relevant. The transcription factor nuclear factor-kappaB (NF-kappaB) regulates the expression of multiple genes controlling inflammation, proliferation, a...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2002-09-2960
更新日期:2003-09-15 00:00:00
abstract::The effects of the deoxygenation rate on the formation of irreversibly sickled cells (ISCs) were investigated by using metabolically replete sickle cells (SS cells). We found that the formation of ISCs required Ca2+ and that the amount formed depended on the rate of deoxygenation. When less dense SS discocytes were de...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1988-01-01 00:00:00
abstract::Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against the A subunit of FXIII, which interfere with different functions of FXIII, have been described. Here, for the first time, we report an autoantibody against the B subunit of...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-09-179333
更新日期:2009-01-15 00:00:00
abstract::The granulocyte-macrophage colony-stimulating factor (GM-CSF) gene promoter contains a consensus sequence for the polyomavirus enhancer binding-protein 2 (PEBP2) transcription factor, which consists of alpha and beta subunits. There are at least two genes, alpha A and alpha B, encoding the alpha subunit. alpha B is th...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1995-07-15 00:00:00
abstract::To investigate the tropism of the T-lymphotropic human herpesvirus 7 (HHV-7) for hematopoietic progenitors, cord blood CD34(+) cells were inoculated in vitro with HHV-7 and then induced to differentiate along the granulocytic and erythroid lineages by the addition of appropriate cytokine cocktails. In semisolid assays...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:2000-07-01 00:00:00
abstract::Mammalian TOR (mTOR) regulates cell growth, proliferation, and migration. Because mTOR knock-outs are embryonic lethal, we generated a viable hypomorphic mouse by neo-insertion that partially disrupts mTOR transcription and creates a potential physiologic model of mTORC1/TORC2 inhibition. Homozygous knock-in mice exhi...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-05-287821
更新日期:2011-01-27 00:00:00
abstract::The t(8;21)(q22;q22) is common in adult acute myeloid leukemia (AML). The RUNX1-ETO fusion protein that is expressed by this translocation is poorly leukemogenic and requires additional mutations for transformation. Loss of sex chromosome (LOS) is frequently observed in t(8;21) AML. In the present study, to evaluate w...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-04-350694
更新日期:2012-03-29 00:00:00
abstract::The cell-surface expression and the functional status of the CD95/Fas antigen on primitive hematopoietic progenitors (PHPs) freshly isolated from human fetal liver (FL) were studied. PHPs were phenotypically defined as CD34++ CD38 -/+ cells. The most immature subfractions of PHPs, CD34++CD38- and CD34+2CD38+ FL cells,...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1996-09-15 00:00:00
abstract::Unstained human eosinophils exhibit unusually bright autofluorescence, which allows them to be distinguished from other leukocytes using fluorescence microscopy. Eosinophil fluorescence is associated with the cytoplasmic granules of the cells. Eosinophil granule extracts, containing an as-yet-undefined eosinophil fluo...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1981-06-01 00:00:00
abstract::Naive and memory CD8(+) T cells can undergo programmed activation and expansion in response to a short T-cell receptor stimulus, but the extent to which in vitro programming can qualitatively substitute for an in vivo antigen stimulation remains unknown. We show that self-/tumor-reactive effector memory CD8(+) T cells...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-12-192419
更新日期:2009-08-27 00:00:00
abstract::Bone marrow CD34(+) cell apoptosis (annexin V), proliferation (Ki-67), and Bcl-2-related protein expression was evaluated by flow cytometry in 102 patients with myelodysplastic syndrome (MDS) and acute myeloid leukemia secondary to MDS (MDS-AML) and in 30 normal donors (NBM). Apoptosis was significantly increased in r...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:2000-12-01 00:00:00
abstract::We here report the initiation of hematopoietic recovery in congenitally hypoplastic S1/S1d mice by the cytotoxic ablation of cells bearing the natural killer (NK) phenotype (NK 1.1+). The most striking finding was the early several-fold increase in the cycling fraction of stem and progenitor cells (with the exception ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1991-09-15 00:00:00
abstract::Blockade of the CD40/CD154 signal is a potential immunomodulatory strategy for T-cell-mediated diseases. As a part of a phase 1, multicenter, dose-escalating trial of humanized monoclonal antibody to CD154 (IDEC-131/E6040) in patients with refractory immune thrombocytopenic purpura (ITP), the autoimmune response to gl...
journal_title:Blood
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1182/blood-2003-06-2167
更新日期:2004-02-15 00:00:00