Abstract:
:In order to determine if mutant hemoglobins can be identified by relatively simple methods, a Working Group of the ICSH Expert Panel on Abnormal Hemoglobins and Thalassemia analyzed 17 hemolysates containing 14 different mutant hemoglobins by four electrophoretic methods: (1) cellulose acetate in alkaline buffers, (2) citrate agar pH 6.0, (3) urea 2-mercaptoethanol buffer pH 8.9, and (4) urea 2-mercaptoethanol buffer pH 6.0. The examined mutants included several of great numerical and clinical importance as well as some rare ones, namely, HbS, C, D Los Angeles (Punjab), E, G Philadelphia, N Baltimore, and O Arab; also Hb Ft. Worth, Montgomery, Winnepeg, Rush, Q India, Bethesda, and Lepore. Comparative mobilities of these hemoglobins in all of the methods are presented here. The combined data permit their presumptive identification, often with a high degree of specificity. The system has been applied in Iran, where the four prevalent mutants can be differentiated by these methods, at considerable saving of time and resources previously expended on structural analyses. It is proposed as a basis for an ICSH Tentative Standard. There is little doubt that this presumptive identification of hemoglobin variants by simple electrophoresis will be improved or complemented by the introduction of newer techniques, such as immunologic analysis. However, for the present and for some time to come, the system outlined here should be found valuable. The present report does not concern itself with the numerous auxiliary techniques involved in the identification of abnormal hemoglobins-sickle-cell test, solubility tests, lability test--and no claim is made that the simple system described here eliminates these other techniques from the diagnostic armamentarium of the laboratory.
journal_name
Bloodjournal_title
Bloodauthors
subject
Has Abstractpub_date
1978-11-01 00:00:00pages
1058-64issue
5eissn
0006-4971issn
1528-0020journal_volume
52pub_type
杂志文章相关文献
BLOOD文献大全abstract::The large multimeric glyocoprotein von Willebrand factor (VWF) is a crucial component of both primary and secondary hemostasis. It is stored in secretory granules of vascular endothelial cells, the Weibel-Palade bodies (WPBs), and is released following stimulation by agonists that raise intracellular Ca(2+) or cyclic ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2012-12-475251
更新日期:2013-08-08 00:00:00
abstract::Leukocyte transendothelial migration (TEM) is a critical event during inflammation. CD47 has been implicated in myeloid cell migration across endothelium and epithelium. CD47 binds to signal regulatory protein (SIRP), SIRPalpha and SIRPgamma. So far, little is known about the role of endothelial CD47 in T-cell TEM in ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-01-134429
更新日期:2008-08-15 00:00:00
abstract::Wiskott-Aldrich syndrome (WAS) is a rare X-linked immunodeficiency with microthrombocytopenia, eczema, recurrent infections, autoimmune disorders, and malignancies that are life-threatening in the majority of patients. In this long-term, retrospective, multicenter study, we analyzed events that occurred in 96 WAS pati...
journal_title:Blood
pub_type: 杂志文章,多中心研究
doi:10.1182/blood-2007-03-076679
更新日期:2008-01-01 00:00:00
abstract::The Ca(2+)-activated K+ channels of human red blood cells (RBCs) (Gardos channels, hIK1, hSK4) can mediate rapid cell dehydration, of particular relevance to the pathophysiology of sickle cell disease. Previous investigations gave widely discrepant estimates of the number of Gardos channels per RBC, from as few as 1 t...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2004-01-0125
更新日期:2005-01-01 00:00:00
abstract::Mitochondrial clearance is a well recognized but poorly understood biologic process, and reticulocytes, which undergo programmed mitochondrial clearance, provide a useful model to study this phenomenon. At the ultrastructural level, mitochondrial clearance resembles an autophagy-related process; however, the role of a...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-04-151639
更新日期:2009-07-02 00:00:00
abstract::We investigated the effects of granulocyte colony-stimulating factor (G-CSF) on cytokine and cytokine receptor plasma levels and on the in vivo host response to Salmonella abortus equi endotoxin in healthy males. Twenty volunteers received 0.8 ng/kg endotoxin and saline intravenously 1 week apart in randomized order. ...
journal_title:Blood
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1996-02-01 00:00:00
abstract::Kaposi's sarcoma-associated herpesvirus (KSHV; also known as human herpesvirus 8 [HHV-8]) is a herpesvirus linked to the development of Kaposi's sarcoma (KS), primary effusion lymphoma, and a proportion of Castleman's disease. KSHV encodes viral interleukin-6 (vIL-6), which is structurally homologous to human and muri...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1999-06-15 00:00:00
abstract::In this issue of Blood, Doyle et al provide evidence that knockout of the genes encoding the two most abundant eosinophil secondary granule proteins disrupts the normal differentiation of eosinophils from progenitors in the bone marrow, providing a novel strain of mice with a highly specific deficiency in eosinophilop...
journal_title:Blood
pub_type: 评论,杂志文章
doi:10.1182/blood-2013-06-508507
更新日期:2013-08-01 00:00:00
abstract::This 2-part, double-blind, placebo-controlled study was conducted to determine the safety and efficacy of etoricoxib, a COX-2 selective inhibitor, for the treatment of hemophilic arthropathy. In part 1 (6 weeks), 102 patients (> or = 12 years old) with hemophilic arthropathy were randomized to receive 90 mg etoricoxib...
journal_title:Blood
pub_type: 杂志文章,随机对照试验
doi:10.1182/blood-2004-09-3501
更新日期:2006-03-01 00:00:00
abstract::The timely clearance of apoptotic neutrophils from inflammation sites is an important function of macrophages; however, the role of macrophages in maintaining neutrophil homeostasis under steady-state conditions is less well understood. By conditionally deleting the antiapoptotic gene cellular FLICE-like inhibitory pr...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-01-265959
更新日期:2011-01-13 00:00:00
abstract::Secondary acute myeloid leukemias (AMLs) evolving from an antecedent myeloproliferative neoplasm (MPN) are characterized by a unique set of cytogenetic and molecular features distinct from de novo AML. Given the high frequency of poor-risk cytogenetic and molecular features, malignant clones are frequently insensitive...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2019000943
更新日期:2020-07-02 00:00:00
abstract::Among 1153 new adult cases of peripheral/T-cell lymphoma from 1990-2002 at 22 centers in 13 countries, 136 cases (11.8%) of extranodal natural killer (NK)/T-cell lymphoma were identified (nasal 68%, extranasal 26%, aggressive/unclassifiable 6%). The disease frequency was higher in Asian than in Western countries and i...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-10-185256
更新日期:2009-04-23 00:00:00
abstract::The proapoptotic function of p53 is thought to underlie most anticancer modalities and is also activated in response to oncogenic insults, such as overexpression of the Myc oncoprotein. Here we generated tractable B lymphomas using retroviral transduction of the MYC oncogene into hematopoietic cells with 2 knock-in al...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-10-050294
更新日期:2007-06-01 00:00:00
abstract::Chromosomal translocation involving the BCL6 gene affects not only immunoglobulin (Ig) genes but also a number of non-Ig genes as partners. The molecular anatomy of the BCL6 gene rearrangements in 39 cases with diffuse large B-cell lymphoma (DLBCL) by long-distance polymerase chain reaction-based assays was determined...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:2000-10-15 00:00:00
abstract::Red blood cells deliver O(2) from the lungs to every cell in the human body. Reduced tissue oxygenation triggers increased production of erythropoietin by hypoxia-inducible factor 1 (HIF-1), which is a transcriptional activator composed of an O(2)-regulated alpha subunit and a constitutively expressed beta subunit. Hy...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2009-05-189985
更新日期:2009-09-03 00:00:00
abstract::Additional sex combs-like 1 (ASXL1), an epigenetic modulator, is frequently mutated in myeloid neoplasms. Recent analyses of mutant ASXL1 conditional knockin (ASXL1-MT-KI) mice suggested that ASXL1-MT alone is insufficient for myeloid transformation. In our previous study, we used retrovirus-mediated insertional mutag...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2019004613
更新日期:2020-10-01 00:00:00
abstract::Chemotherapy depletes T cells in AIDS patients on HAART, but preexisting lymphopenia limits the scale compared with non-AIDS patients. Thus T-cell recovery time to baseline may be rapid, but the risk of additional AIDS-related complications persists. ...
journal_title:Blood
pub_type: 评论,杂志文章
doi:10.1182/blood-2008-01-134528
更新日期:2008-04-15 00:00:00
abstract::The possibility of primitive hematopoietic cell ex vivo expansion is of interest for both gene therapy and transplantation applications. The engraftment of autologous rhesus peripheral blood (PB) progenitors expanded 10 to 14 days were tracked in vivo using genetic marking. Stem cell factor (SCF)/granulocyte colony-st...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1998-08-15 00:00:00
abstract::Chronic graft-v-host disease (chronic GVHD) is a frequent cause of late morbidity and death after bone marrow transplantation (BMT). The actuarial survival after onset of chronic GVHD in 85 patients was 42% (95%Cl = 29%, 54%) at 10 years. Baseline characteristics present at the onset of chronic GVHD (before therapy) i...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1989-09-01 00:00:00
abstract::Myeloperoxidase (MPO) is a lysosomal enzyme present in the azurophilic granules of human neutrophils and monocytes and is important for optimal oxygen-dependent killing of microorganisms. The native molecule is a heterodimer composed of a pair of heavy-light protomers, each containing a 59-kDa and 13.5-kDa subunit. Th...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1987-10-01 00:00:00
abstract::The aim of this study was to understand the impact of chronic graft-versus-host disease (cGVHD) on the overall health status of hematopoietic cell transplantation (HCT) survivors. Subjects included 584 individuals who had undergone allogeneic HCT between 1976 and 1999, survived 2 or more years, and completed a 255-ite...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-02-003954
更新日期:2006-10-15 00:00:00
abstract::Toll-like receptors (TLRs) and complement are 2 components of innate immunity that are critical for first-line host defense and elicitation of adaptive immune responses. Many pathogen-associated molecular patterns activate both TLR and complement, but whether and how these 2 systems, when coactivated in vivo, interact...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-12-063636
更新日期:2007-07-01 00:00:00
abstract::A pathogenic role for high-mobility group box 1 (HMGB1) protein has been postulated in severe sepsis. Activated protein C (APC) is the only drug approved by the Food and Drug Administration for severe sepsis; however, its effect on HMGB1 signaling has never been investigated. Here, we monitored the effect of APC on th...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-06-360701
更新日期:2011-10-06 00:00:00
abstract::Identification of genes involved in in vitro differentiation induction of embryonic stem cells (ESCs) into hematopoietic stem cells (HSCs) has been challenged during last decade. To date, a homeobox transcription factor Hoxb4 has been only demonstrated to possess such an effect in mice. Here, we show that HSC-like cel...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-07-298596
更新日期:2011-04-07 00:00:00
abstract::RUNX1 is crucial for the regulation of megakaryocyte specification, maturation, and thrombopoiesis. Runx1 possesses 2 promoters: the distal P1 and proximal P2 promoters. The major protein isoforms generated by P1 and P2 are RUNX1C and RUNX1B, respectively, which differ solely in their N-terminal amino acid sequences. ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2016-06-723635
更新日期:2017-07-20 00:00:00
abstract::In contrast to other diverse therapies for the X-linked bleeding disorder hemophilia that are currently in clinical development, gene therapy holds the promise of a lasting cure with a single drug administration. Near-to-complete correction of hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficienc...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2018-07-820720
更新日期:2019-01-31 00:00:00
abstract::The three-dimensional tertiary structure of human von Willebrand Factor (vWF) on a hydrophobic surface under aqueous conditions and different shear stress regimes was studied by atomic force microscopy (AFM). vWF was imaged by AFM at molecular level resolution under negligible shear stress, under a local applied shear...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1996-10-15 00:00:00
abstract::We studied the impact of driver mutations of JAK2, CALR, (calreticulin gene) or MPL on clinical course, leukemic transformation, and survival of patients with primary myelofibrosis (PMF). Of the 617 subjects studied, 399 (64.7%) carried JAK2 (V617F), 140 (22.7%) had a CALR exon 9 indel, 25 (4.0%) carried an MPL (W515)...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2014-05-578435
更新日期:2014-08-14 00:00:00
abstract::Polymorphonuclear neutrophil (PMN) extravasation requires selectin-mediated tethering, intercellular adhesion molecule-1 (ICAM-1)-dependent firm adhesion, and platelet/endothelial cell adhesion molecule 1 (PECAM-1)-mediated transendothelial migration. An important unanswered question is whether ICAM-1-activated signal...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-12-397430
更新日期:2012-08-30 00:00:00
abstract::Basic fibroblast growth factor or fibroblast growth factor-2 (FGF) has been shown to affect myeloid cell proliferation and hypothesized to stimulate primitive hematopoietic cells. We sought to evaluate the effect of FGF on hematopoietic stem cells and to determine if FGF mediated its effects on progenitor cells direct...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1995-09-15 00:00:00