Abstract:
:A 2-yr-old black girl presented with a thalassemic clinical picture and was found to have nearly 100% fetal hemoglobin in her red cells. Pedigree analysis indicated that she was a heterozygote for the hereditary persistence of fetal hemoglobin gene and for a beta O-thalassemia gene. A brother, who also had nearly 100% fetal hemoglobin in his red cells, manifested, in contrast to his sister, no anemia and only minimal splenomegaly. Examination of the family's alpha-globin loci using the restriction endonuclease Eco Rl demonstrated that the brother had a single alpha-locus deletion that he had inherited from his mother. The mild clinical manifestations of this boy are consistent with the often expressed view that excess alpha chains may contribute significantly to the hematologic manifestation of beta-thalassemia.
journal_name
Bloodjournal_title
Bloodauthors
Beutler E,Turner E,Kuhl Wsubject
Has Abstractpub_date
1981-06-01 00:00:00pages
1132-4issue
6eissn
0006-4971issn
1528-0020journal_volume
57pub_type
杂志文章相关文献
BLOOD文献大全abstract::In this issue of Blood, Neufeld and colleagues present promising results on the safety and efficacy of a novel iron chelator for the treatment of transfusional iron overload. ...
journal_title:Blood
pub_type: 评论,杂志文章
doi:10.1182/blood-2012-02-406744
更新日期:2012-04-05 00:00:00
abstract::The human beta-globin locus control region (LCR) confers high-level, tissue-specific expression to the beta-globin genes. Tandem Maf recognition elements (MAREs) within the hypersensitive site 2 (HS2) subregion of the LCR are important for the strong enhancer activity of the LCR. Multiple proteins are capable of inter...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:2000-07-01 00:00:00
abstract::Prediagnostic serum concentration of soluble CD30 (sCD30), a marker for chronic B-cell stimulation, has been associated with increased risk of developing AIDS-related non-Hodgkin lymphoma (NHL) in a recent study of HIV(+) patients. To investigate among healthy persons whether serum sCD30 is associated with NHL risk, w...
journal_title:Blood
pub_type: 杂志文章,多中心研究
doi:10.1182/blood-2009-04-217521
更新日期:2009-09-24 00:00:00
abstract::The efficacy of the addition of intensive therapy with daunorubicin (45 mg/m2 IV on days 1, 2, 3) to an otherwise identical induction program consisting of vincristine, prednisone, and L-asparaginase was assessed in 177 previously untreated adults (greater than or equal to 20 years of age) with acute lymphocytic leuke...
journal_title:Blood
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1984-07-01 00:00:00
abstract::Heavy chain ferritin (H-ferritin) is a component of the iron-binding protein, ferritin. We have previously shown that H-ferritin inhibits anti-CD3-stimulated lymphocyte proliferation and that this was due to increased production of interleukin-10 (IL-10). In the present study we have shown that induction of IL-10 prod...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v99.9.3326
更新日期:2002-05-01 00:00:00
abstract::Under evolutionary pressure to counter the toxicity of iron and to maintain adequate iron supply for hemoglobin synthesis and essential metabolic functions, humans and other vertebrates have effective mechanisms to conserve iron and to regulate its concentration, storage, and distribution in tissues. The iron-regulato...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2011-01-258467
更新日期:2011-04-28 00:00:00
abstract::Mesenchymal stem cells (MSCs) are mostly found around the vasculature system of the adult bone marrow (BM). They function as immune suppressors, express MHC-II, are phagocytic, and support T-cell cytotoxicity. We hypothesize that these contradictory properties of MSCs are important for BM homeostasis and occur partly ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-01-0057
更新日期:2006-06-15 00:00:00
abstract::The microbiota is known to influence the generation of hematopoietic progenitors, although the pathways underlying this process are still poorly understood. NOD1 and NOD2 are intracellular sensors for both Gram-positive and Gram-negative bacteria, but their role in steady-state hematopoiesis has never been characteriz...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2016-06-723742
更新日期:2017-01-12 00:00:00
abstract::We report in this paper that a novel small molecule, JTZ-132, induced growth and differentiation of megakaryocytic progenitor cells and improved thrombocytopenia in myelosuppressed mice. JTZ-132 stimulated proliferation of UT-7/TPO cells, a cell line highly sensitive to thrombopoietin (TPO), and exhibited full efficac...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-10-3623
更新日期:2004-07-01 00:00:00
abstract::The reduction of nitroblue tetrazolium dye by human neutrophils was measured in the presence of serum in which the complement system had been activated through the alternate pathway by interaction with inulin. Neutrophils incubated with serum inulin supernatants reduced the dye and showed a general increase in oxidati...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1975-06-01 00:00:00
abstract::The deregulated Bcr/Abl tyrosine kinase is responsible for the development of Philadelphia (Ph)-positive leukemia in humans. To investigate the significance of the C-terminal Abl actin-binding domain within Bcr/Abl p190 in the development of leukemia/lymphoma in vivo, mutant p190 DNA constructs were used to generate t...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:2000-09-15 00:00:00
abstract::In a previous study, we reported that a single injection of cyclophosphamide (CTX) in tumor-bearing mice resulted in tumor eradication when the animals were subsequently injected with tumor-sensitized lymphocytes. Notably, CTX acted by inducing bystander effects on T cells, and the response to the combined CTX/adoptiv...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:2000-03-15 00:00:00
abstract::Donor-matched transplantation of hematopoietic stem cells (HSCs) is widely used to treat hematologic malignancies but is associated with high mortality. The expansion of HSC numbers and their mobilization into the bloodstream could significantly improve therapy. We report here that adult mice conditionally deficient f...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-04-280305
更新日期:2010-12-09 00:00:00
abstract::CD44 is a widely distributed cell surface glycoprotein whose principal ligand has been identified as hyaluronic acid (HA), a major component of the extracellular matrix (ECM). Recent studies have demonstrated that activation through CD44 leads to induction of effector function in T cells and macrophages. In the curren...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1997-04-15 00:00:00
abstract::Autosomal recessive STAT1 deficiency is associated with impaired cellular responses to interferons and susceptibility to intracellular bacterial and viral infections. We report here a new form of partial STAT1 deficiency in 2 siblings presenting mycobacterial and viral diseases. Both carried a homozygous missense muta...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-04-280586
更新日期:2010-12-23 00:00:00
abstract::Acute myeloid leukemia (AML) is an aggressive malignancy with a relapse rate approaching 50%, despite aggressive chemotherapy. New therapies for AML are targeted at signal transduction pathways known to support blast survival, such as the Stat3 pathway. Aberrant activation of Stat3 has been demonstrated in many differ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-04-280123
更新日期:2011-05-26 00:00:00
abstract::We investigated 17 patients (12 males and 5 females, ages 2 to 57 years old) with posttransfusion non-A, non-B hepatitis to determine relationships between clinical courses and hepatitis C virus (HCV) markers. The patients were grouped according to time course of abnormal serum alanine aminotransferase (ALT) levels in...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1991-03-15 00:00:00
abstract::Most patients with chronic lymphocytic leukemia (CLL) are diagnosed with early-stage disease and managed with active surveillance. The individual course of patients with early-stage CLL is heterogeneous, and their probability of needing treatment is hardly anticipated at diagnosis. We aimed at developing an internatio...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2019003453
更新日期:2020-05-21 00:00:00
abstract::Human herpesvirus 6 (HHV-6) is a potentially immunosuppressive agent that has been suggested to act as a cofactor in the progression of HIV disease. Exposure of human macrophages to HHV-6A or HHV-6B profoundly impaired their ability to produce interleukin 12 (IL-12) upon stimulation with interferon-gamma (IFN-gamma) a...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2002-10-3152
更新日期:2003-10-15 00:00:00
abstract::Glanzmann thrombasthenia (GT) is a rare bleeding disorder resulting from mutations in either glycoprotein (GP) IIb or GPIIIa genes. The disease is relatively frequent in highly inbred populations such as Iraqi Jews. The molecular basis of GT in 6 unrelated Iraqi-Jewish patients was previously identified as an 11-bp de...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1997-05-15 00:00:00
abstract::Therapy for childhood acute lymphoblastic leukemia (ALL) is associated with 5-year survival rates of ∼90% even after largely eliminating cranial radiation. This meta-analysis assesses the long-term neurocognitive functioning after chemotherapy-only regimens among survivors of childhood ALL. We conducted a systematic r...
journal_title:Blood
pub_type: 杂志文章,meta分析,评审
doi:10.1182/blood-2015-02-627414
更新日期:2015-07-16 00:00:00
abstract::Serial erythrocyte volume distribution curves were used to characterize changes in erythrocyte subpopulations following the induction of Heinz body hemolytic anemia in cats. Macrocytes produced in response to hemolysis were observed after loss of reticulum. After recovery from hemolysis, a gradual reduction in mean ce...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1982-08-01 00:00:00
abstract::FTY720 is an immunosuppressant developed to prevent organ transplant rejection. Recent studies indicate an additional role for FTY720 in inducing cell apoptosis. We demonstrate here that FTY720 mediates toxic effects in cell lines representing different B-cell malignancies and primary B cells from patients with chroni...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-10-053884
更新日期:2008-01-01 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::We have recently demonstrated that IgD(hi) B cells can occupy an extravascular perisinusoidal niche in the bone marrow in addition to the well-established follicular niche in conventional secondary lymphoid organs. The spleen has long been considered to be the site at which newly formed B lymphocytes mature into IgD(h...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-05-021089
更新日期:2007-03-15 00:00:00
abstract::Expression of the myeloperoxidase (MPO) gene is tightly regulated in a tissue- and development-specific manner. Accumulation of MPO messenger RNA (mRNA) occurs only at the late myeloblastic and promyelocytic stages of myeloid differentiation and is negligible at other stages of myeloid development and in other tissues...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1991-07-15 00:00:00
abstract::Mice carrying a conditional prothrombin knockout allele (fII(lox)) were established to develop an experimental setting for exploring the importance of thrombin in the maintenance of vascular integrity, the inflammatory response, and disease processes in adult animals. In the absence of Cre-mediated recombination, homo...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-07-169003
更新日期:2009-01-15 00:00:00
abstract::Although well characterized in the mouse, the role of Notch signaling in the human T-cell receptor alphabeta (TCR-alphabeta) versus TCR-gammadelta lineage decision is still unclear. Although it is clear in the mouse that TCR-gammadelta development is less Notch dependent compared with TCR-alphabeta differentiation, re...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-06-164871
更新日期:2009-03-26 00:00:00
abstract::Stem cell factor (SCF) and interleukin-3 (IL-3) both act on several target hematopoietic populations, including mast cells. We have isolated a unique murine mast cell line, B6M, that is phenotypically similar to immature mast cells. For B6M cells, IL-3 is a survival factor and alone does not stimulate proliferation. S...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1996-05-01 00:00:00
abstract::Several patients with clinical features of chronic myeloid leukemia (CML) have fusion of the TEL (ETV6) gene on 12p13 with ABL on 9q34 and express a chimeric Tel-Abl protein that contains the same portion of the Abl tyrosine kinase fused to Tel, an Ets family transcription factor, rather than Bcr. In a murine retrovir...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2001-12-0244
更新日期:2002-06-15 00:00:00