Abstract:
:Paroxysmal nocturnal hemoglobinuria (PNH) is a disease that affects not only red cells, but other blood cells as well. The common defect is supposed to be an acquired deficiency of glycosyl-phosphatidylinositol (GPI)-anchored membrane proteins, which may be present already at the hematopoietic stem cell level. Recently, a panel of monoclonal antibodies (MoAbs) has become available directed against various GPI-linked membrane proteins. This makes it possible to study various cell lineages for the deficiency of such proteins in PNH in more detail. Using cytofluorography, we could show that the granulocytes of 20 different PNH patients miss not only GPI-linked FcRIII (CD16 antigen), but also three other GPI-linked proteins, ie, CD24 antigen, CD67 antigen and a granulocyte-specific 50 to 80 Kd antigen. The affected granulocytes were not only neutrophils but also eosinophils, as was found in a more detailed analysis of three patients. Moreover, in all 10 PNH patients tested, the monocytes were found to be deficient for the GPI-linked CD14 antigen, and we found with CD24 and CD55 (DAF) antibodies that lymphocytes may be involved as well. However, abnormal B and T lymphocytes were detected only in a subset of patients (2 of 10 tested). The uniform deficiency of GPI-linked proteins of granulocytes allows the introduction of a new diagnostic cytofluorometric assay for PNH with MoAbs against GPI-linked granulocytic antigens. This test was positive in all PNH patients studied and not in a group of 40 control patients or 50 normal donors, with the exception of three of 16 aplastic anemia (AA) patients. In the three AA patients, subpopulations (10% to 20%) of PNH granulocytes could be detected, whereas these patients had a negative acidified serum (Ham) test. This indicates that the new test is more sensitive than the Ham test and allows the early diagnosis of PNH in AA. An advantage of the neutrophil assay is that, in contrast to the Ham test, it is not influenced by recent red-cell transfusions. Moreover, it is possible to quantify the number of affected cells by single cell analysis.
journal_name
Bloodjournal_title
Bloodauthors
van der Schoot CE,Huizinga TW,van 't Veer-Korthof ET,Wijmans R,Pinkster J,von dem Borne AEsubject
Has Abstractpub_date
1990-11-01 00:00:00pages
1853-9issue
9eissn
0006-4971issn
1528-0020journal_volume
76pub_type
杂志文章相关文献
BLOOD文献大全abstract::Through the procedure of gene amplification combined with hybridization to synthetic 19 base pair (bp) oligonucleotide probes, it has been possible to identify nine different mutations in the DNA of 47 subjects from Turkey and Northern Cyprus with a beta-thalassemia homozygosity. The IVS-I nucleotide (nt) 110 G----A a...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1988-01-01 00:00:00
abstract::In this single-center, cross-sectional study, we evaluated 44 very long-term survivors with a median follow-up of 17.5 years (range, 11-26 years) after hematopoietic stem cell transplantation. We assessed the telomere length difference in human leukocyte antigen-identical donor and recipient sibling pairs and searched...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2009-03-209833
更新日期:2009-07-02 00:00:00
abstract::The activities of four purified human growth factors: biosynthetic (recombinant) granulocyte-macrophage colony-stimulating factor (GM-CSF); recombinant erythroid-potentiating activity (EPA); natural and recombinant pluripoietin (Ppo); and natural pluripoietin alpha (Ppo alpha), were compared on the growth of hematopoi...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1987-05-01 00:00:00
abstract::Transfusion-associated graft-versus-host disease can be prevented by treating cellular blood products with gamma irradiation. A wide range of gamma irradiation dose levels has been used in routine practice. We used limiting dilution analysis, which measures clonable T cells, to assess the influence of 500 to 3,000 cGy...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1994-03-15 00:00:00
abstract::In this issue of Blood, Kochenderfer et al show that engineered CD19–chimeric antigen receptor (CAR) and donor-derived allogeneic T cells can safely treat CD19-positive B-cell malignancies, which have relapsed after allogeneic stem cell transplantation. ...
journal_title:Blood
pub_type: 评论,杂志文章
doi:10.1182/blood-2013-10-528984
更新日期:2013-12-12 00:00:00
abstract::A distinct pathologic entity (ALK+ lymphoma) that is characterized by expression of the anaplastic lymphoma kinase (ALK) protein has recently emerged within the heterogeneous group of CD30(+) anaplastic large-cell lymphomas. Information on clinical findings and treatment outcome of ALK+ lymphoma is still limited, and ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1999-04-15 00:00:00
abstract::Incubation of human platelets, human neutrophils, or highly metastatic mouse lymphoma cells with sulfate-labeled extracellular matrix (ECM) results in heparanase-mediated release of labeled heparan sulfate cleavage fragments (0.5 less than Kav less than 0.85 on Sepharose 6B). This degradation was inhibited by native h...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1987-08-01 00:00:00
abstract::Primary myelofibrosis (PMF) is the most serious myeloproliferative disorder, characterized by clonal myeloproliferation associated with cytokine-mediated bone marrow stromal reaction including fibrosis and osteosclerosis. Current drug therapy remains mainly palliative. Because the NF-kappaB pathway is implicated in th...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-10-054502
更新日期:2007-07-01 00:00:00
abstract::Macrophage mannose receptor (MR) participates in pathogen recognition, clearance of endogenous serum glycoproteins, and antigen presentation. MR is also present on lymphatic vessels, where its function is unknown. Here we show that migration of lymphocytes from the skin into the draining lymph nodes through the affere...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2007-10-118984
更新日期:2008-07-01 00:00:00
abstract::Type 1 T regulatory (Tr1) cells suppress immune responses in vivo and in vitro and play a key role in maintaining tolerance to self- and non-self-antigens. Interleukin-10 (IL-10) is the crucial driving factor for Tr1 cell differentiation, but the molecular mechanisms underlying this induction remain unknown. We identi...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2009-07-234872
更新日期:2010-08-12 00:00:00
abstract::An inverse relationship between BCL-2 expression and cell cycle transition has been suggested by recent studies in murine models. To investigate the clinical relevance of these laboratory studies, a group of 116 paraffin-embedded non-Hodgkin's lymphoma (NHL) biopsy specimens (Working Formulation Groups D-H, and J) fro...
journal_title:Blood
pub_type: 临床试验,杂志文章
doi:
更新日期:1998-02-15 00:00:00
abstract::To address the mechanism by which the human globin genes are activated during erythropoiesis, we have used a tiled microarray to analyze the pattern of transcription factor binding and associated histone modifications across the telomeric region of human chromosome 16 in primary erythroid and nonerythroid cells. This ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2007-06-097964
更新日期:2007-12-15 00:00:00
abstract::Genome and transcriptome sequencing have revealed a rich assortment of noncoding RNAs in eukaryote cells, including long noncoding RNAs (lncRNAs), which regulate gene expression independent of protein coding potential. LncRNAs modulate protein coding gene expression in many cell types by regulating multiple processes,...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2013-03-456111
更新日期:2013-06-13 00:00:00
abstract::Primary effusion lymphoma (PEL) is a rare B-cell malignancy that most often occurs in immunocompromised patients, such as HIV-infected individuals and patients receiving organ transplantation. The main characteristic of PEL is neoplastic effusions in body cavities without detectable tumor masses. The onset of the dise...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2018-03-791426
更新日期:2018-11-01 00:00:00
abstract::As tyrosine kinase inhibitors (TKIs) fail to induce long-term response in blast crisis chronic myelogenous leukemia (CML-BC) and Philadelphia chromosome-positive (Ph(+)) acute lymphoblastic leukemia (ALL), novel therapies targeting leukemia-dysregulated pathways are necessary. Exportin-1 (XPO1), also known as chromoso...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2013-04-495374
更新日期:2013-10-24 00:00:00
abstract::Monocytes regulate host defenses, inflammation, and tissue homeostasis. The transcription factor interferon regulatory factor-8 (IRF8) stimulates monocyte/macrophage differentiation, yet genome-wide understanding of the differentiation program initiated by IRF8 is lacking. By combining chromatin immunoprecipitation se...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2012-06-437863
更新日期:2013-03-07 00:00:00
abstract::Reduced lymphopoiesis during aging contributes to declines in immunity, but little consideration has been given to its effect on the development of hematologic disease. This report demonstrates that age-related defects in lymphopoiesis underlie the myeloid dominance of adult leukemia. Using a murine model of chronic m...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2007-01-069401
更新日期:2007-09-15 00:00:00
abstract::We have applied a proteomics approach to analyze signaling cascades in human platelets stimulated by thrombin receptor activating peptide (TRAP). By analyzing basal and TRAP-activated platelets using 2-dimensional gel electrophoresis (2-DE), we detected 62 differentially regulated protein features. From these, 41 coul...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-07-2392
更新日期:2004-03-15 00:00:00
abstract::Chimeric antigen receptor (CAR) T cells have radically improved the treatment of B cell-derived malignancies by targeting CD19. The success has not yet expanded to treat acute myeloid leukemia (AML). We developed a Sequentially Tumor-Selected Antibody and Antigen Retrieval (STAR) system to rapidly isolate multiple nan...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2019002779
更新日期:2020-03-05 00:00:00
abstract::Dendritic cells (DCs) represent a small and heterogeneous fraction of the hematopoietic system, specialized in antigen capture, processing, and presentation. The different DC subsets act as sentinels throughout the body and perform a key role in the induction of immunogenic as well as tolerogenic immune responses. Bec...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2011-11-370130
更新日期:2012-04-12 00:00:00
abstract::For many in vivo gene therapy clinical applications, it is desirable to control the expression of the transferred transgene using pharmacologic agents. To evaluate the feasibility of accomplishing this using corticosteroids, pharmacologic agents widely used in clinical medicine, we constructed replication deficient ad...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1998-08-01 00:00:00
abstract::Functionally inhibitory antibody to the plasma membrane complement inhibitor CD59 has been used to investigate control of the terminal complement proteins at the endothelial cell surface. Antibodies against purified human erythrocyte CD59 (polyclonal anti-CD59 and monoclonal antibodies [MoAbs] 1F1 and 1F5) were found ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1990-12-15 00:00:00
abstract::Angiotensin II (Ang-II) exerts inflammatory activity and is involved in different cardiovascular disorders. This study has evaluated the involvement of tumor necrosis factor alpha (TNFalpha) in the leukocyte accumulation elicited by Ang-II. Ang-II (1 nM intraperitoneally in rats) induced TNFalpha release at 1 hour fol...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2007-01-070607
更新日期:2007-09-15 00:00:00
abstract::The tyrosine kinase receptor fetal liver kinase 1 (Flk-1) plays a crucial role in vasculogenesis and angiogenesis, but its target genes remain elusive. Comparing Flk-1(+/+) with Flk-1(-/-) embryonic stem (ES) cells, we identified transcripts regulated by the vascular endothelial growth factor A (VEGF-A)/Flk-1 pathway ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-11-3827
更新日期:2004-06-15 00:00:00
abstract::Growing evidence indicates antibody-dependent cellular cytotoxicity (ADCC) contributes to the clinical response to monoclonal antibody (mAb) therapy of lymphoma. Recent in vitro analysis suggests C3b can inhibit mAb-induced natural killer (NK)-cell activation and ADCC. Further studies were conducted to assess the effe...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2009-01-200469
更新日期:2009-12-17 00:00:00
abstract::Granzyme H (GzmH) belongs to a family of 5 human serine proteases that are expressed by cytotoxic immune effector cells. Although GzmH is most closely related to the caspase-activating granzyme B (GzmB), neither a natural substrate nor a role in immune defense reactions has been demonstrated for this orphan granzyme. ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-10-051649
更新日期:2007-07-15 00:00:00
abstract::The short-term beneficial effect of hydroxyurea (HU) in sickle cell disease (SCD) has been proven by randomized studies in children and adults. The Belgian registry of HU-treated SCD patients was created to evaluate its long-term efficacy and toxicity. The median follow-up of the 93 patients registered is 3.5 years; c...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v97.11.3628
更新日期:2001-06-01 00:00:00
abstract::Using a specific enzyme-linked immunosorbent assay, plasminogen activator inhibitor-2 (PAI-2) was quantitated in cultures of human monocytes. Lipopolysaccharide (LPS) increased both extracellular and cell-associated PAI-2 levels, as well as PAI-2 mRNA measured by Northern analysis. Both the lymphokine, interleukin-4 (...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1992-07-01 00:00:00
abstract::Hematopoietic stem cell (HSC)-based gene therapy holds promise for the cure of many diseases. The field is now moving toward the use of lentiviral vectors (LVs) as evidenced by 4 successful clinical trials. These trials used vesicular-stomatitis-virus-G protein (VSV-G)-LVs at high doses combined with strong cytokine-c...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2014-02-558163
更新日期:2014-08-21 00:00:00
abstract::We recently demonstrated that immunoglobulin E (IgE), in the absence of cross-linking agents, activates signaling pathways in healthy murine bone marrow-derived mast cells (BMMCs) and that this activation enhances BMMC survival, at least in part, via secretion of autocrine-acting cytokines. We report herein that IgE a...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2002-10-3176
更新日期:2003-08-15 00:00:00