Molecular basis for the recently described hereditary hyperferritinemia-cataract syndrome: a mutation in the iron-responsive element of ferritin L-subunit gene (the "Verona mutation")

abstract：:Hand mirror cells are a morphological configuration that are seen in immunologically stimulated lymphocytes and can be induced by antigen--antibody complexes. Therefore, the bone marrow and peripheral blood plasma of two patients with acute lymphoblastic leukemia--hand mirror variant were evaluated for the presence of...

journal_title：Blood

authors： Stass SA,Phillips TM,Weislow OS,Perlin E,Schumacher HR

更新日期：1980-10-01 00:00:00

abstract：:A study in 121 infants with severe combined immunodeficiency (SCID) was performed to determine the prevalence of an engraftment by transplacentally acquired maternal T cells and to explore clinical and immunological findings related to this abnormality. Each newly diagnosed patient with SCID presenting with circulatin...

journal_title：Blood

authors： Müller SM,Ege M,Pottharst A,Schulz AS,Schwarz K,Friedrich W

更新日期：2001-09-15 00:00:00

abstract：:Chromosomal rearrangements affecting RUNX1 and CBFB are common in acute leukemias. These mutations result in the expression of fusion proteins that act dominant-negatively to suppress the normal function of the Runt-related transcription factor 1 (RUNX)/core binding factor beta (CBFbeta) complexes. In addition, loss-o...

journal_title：Blood

authors： Kundu M,Compton S,Garrett-Beal L,Stacy T,Starost MF,Eckhaus M,Speck NA,Liu PP

更新日期：2005-11-15 00:00:00

abstract：:Erythropoiesis was investigated in 32 children wih short stature and in eight children with skeletal dysplasia by studying blood hemoglobin in relation to growth and to serum concentrations of insulin-like growth factor I (IGF-I), IGF binding protein-3 (IGFBP-3), and erythropoietin (EPO) before, during, and after 12 m...

journal_title：Blood

authors： Vihervuori E,Virtanen M,Koistinen H,Koistinen R,Seppälä M,Siimes MA

更新日期：1996-03-01 00:00:00

abstract：:A postulated role of the contact system in anaphylactic reactions to insect stings was investigated. During prospective, in-hospital sting challenge, we collected serial blood samples from five normal volunteers and 16 patients with a history of insect-sting anaphylaxis. Activation of the contact system was assessed b...

journal_title：Blood

authors： van der Linden PW,Hack CE,Eerenberg AJ,Struyvenberg A,van der Zwan JK

更新日期：1993-09-15 00:00:00

abstract：:Phytohemagglutin-stimulated child and adult leukocytes equally supported CCR5-dependent (R5) and CXCR4-dependent (X4) HIV-1 replication. In contrast, when phytohemagglutin-stimulated leukocytes from either healthy or congenitally immunodeficient children were cultured on feeder cells, they well supported R5, but not X...

journal_title：Blood

authors： Mariani SA,Brigida I,Kajaste-Rudnitski A,Ghezzi S,Rocchi A,Plebani A,Vicenzi E,Aiuti A,Poli G

更新日期：2012-03-01 00:00:00

abstract：:The Graffi murine leukemia virus induces a large spectrum of leukemias in mice and thus provides a good model to compare the transcriptome of all types of leukemias. We analyzed the gene expression profiles of both T and B leukemias induced by the virus with DNA microarrays. Given that we considered that a 4-fold chan...

journal_title：Blood

authors： Charfi C,Voisin V,Levros LC Jr,Edouard E,Rassart E

更新日期：2011-02-10 00:00:00

abstract：:Vaccination of patients with cancer using dendritic cells (DCs) was shown to be effective for B-cell lymphoma and malignant melanoma. Here we provide evidence that patients with advanced breast and ovarian cancer can be efficiently vaccinated with autologous DCs pulsed with HER-2/neu- or MUC1-derived peptides. Ten pat...

journal_title：Blood

authors： Brossart P,Wirths S,Stuhler G,Reichardt VL,Kanz L,Brugger W

更新日期：2000-11-01 00:00:00

abstract：:Human blood contains 2 populations of dendritic cells (DCs): plasmacytoid and myeloid (mDC). mDCs are subdivided into 3 subsets using the surface markers CD16, CD1c, and BDCA-3. Their role as pathogen sentinels and adjuvant targets was tested by phenotypic and functional analysis. We show that mDC subsets are immature...

journal_title：Blood

authors： Piccioli D,Tavarini S,Borgogni E,Steri V,Nuti S,Sammicheli C,Bardelli M,Montagna D,Locatelli F,Wack A

更新日期：2007-06-15 00:00:00

abstract：:ADAMTS13 metalloprotease cleaves von Willebrand factor (VWF), thereby inhibiting platelet aggregation and arterial thrombosis. An inability to cleave ultralarge VWF resulting from hereditary or acquired deficiency of plasma ADAMTS13 activity leads to a potentially fatal syndrome, thrombotic thrombocytopenic purpura (T...

journal_title：Blood

authors： Pickens B,Mao Y,Li D,Siegel DL,Poncz M,Cines DB,Zheng XL

更新日期：2015-05-21 00:00:00

abstract：:Lipids extracted with chloroform-methanol from red blood cell membranes of 7 PNH and 13 control subjects were used for the preparation of liposomes, which were then examined with the reactive lysis test. PNH liposomes lysed to a higher extent than control liposomes as indicated by the higher dilution of the limiting c...

journal_title：Blood

authors： Tedesco F,Kahane I,Zanella A,Giovanetti AM,Sirchia G

更新日期：1981-05-01 00:00:00

abstract：:The monoclonal antibodies Tab and AP3 are directed, respectively, against GPIIb and GPIIIa, the subunits of the platelet fibrinogen receptor. When added together to platelets, these antibodies prevent adenosine diphosphate (ADP)-induced platelet aggregation, despite normal fibrinogen binding (Newman et al, Blood 69:66...

journal_title：Blood

authors： Isenberg WM,Bainton DF,Newman PJ

更新日期：1990-10-15 00:00:00

abstract：:Using data from ARISTOTLE, we describe the periprocedural management of anticoagulation and rates of subsequent clinical outcomes among patients chronically anticoagulated with warfarin or apixaban. We recorded whether (and for how long) anticoagulant therapy was interrupted preprocedure, whether bridging therapy was ...

journal_title：Blood

authors： Garcia D,Alexander JH,Wallentin L,Wojdyla DM,Thomas L,Hanna M,Al-Khatib SM,Dorian P,Ansell J,Commerford P,Flaker G,Lanas F,Vinereanu D,Xavier D,Hylek EM,Held C,Verheugt FW,Granger CB,Lopes RD

更新日期：2014-12-11 00:00:00

abstract：:We investigated the functions of adiponectin, an adipocyte-specific secretory protein and a new member of the family of soluble defense collagens, in hematopoiesis and immune responses. Adiponectin suppressed colony formation from colony-forming units (CFU)-granulocyte-macrophage, CFU-macrophage, and CFU-granulocyte, ...

journal_title：Blood

authors： Yokota T,Oritani K,Takahashi I,Ishikawa J,Matsuyama A,Ouchi N,Kihara S,Funahashi T,Tenner AJ,Tomiyama Y,Matsuzawa Y

更新日期：2000-09-01 00:00:00

abstract：:Fanconi anemia (FA) is a genetic disease characterized by congenital abnormalities, bone marrow failure, and susceptibility to leukemia and other cancers. FANCJ, one of 13 genes linked to FA, encodes a DNA helicase proposed to operate in homologous recombination repair and replicational stress response. The pathogenic...

journal_title：Blood

authors： Wu Y,Sommers JA,Suhasini AN,Leonard T,Deakyne JS,Mazin AV,Shin-Ya K,Kitao H,Brosh RM Jr

更新日期：2010-11-11 00:00:00

abstract：:Eighty-three patients with circulating anticoagulants were studied at The New York Hospital. The lupus-type anticoagulant, an inhibitor of the prothrombin activator complex, was demonstrated in 58 patients. The inhibitor was identified using the blood and tissue thromboplastin inhibition tests. Inhibition by the lupus...

journal_title：Blood

authors： Schleider MA,Nachman RL,Jaffe EA,Coleman M

更新日期：1976-10-01 00:00:00

abstract：:The administration of cytokines that modulate endogenous or transferred T-cell immunity could improve current approaches to clinical immunotherapy. Interleukin-2 (IL-2) is used most commonly for this purpose, but causes systemic toxicity and preferentially drives the expansion of CD4(+)CD25(+)Foxp3(+) regulatory T cel...

journal_title：Blood

authors： Berger C,Berger M,Hackman RC,Gough M,Elliott C,Jensen MC,Riddell SR

更新日期：2009-09-17 00:00:00

abstract：:Because of continuous blood transfusions, thalassemia patients are subjected to peroxidative tissue injury by the secondary iron overload. In accordance, analysis of serum from 42 beta-thalassemia patients, aged 4 to 40 years, showed that the mean concentrations of conjugated diene lipid hydroperoxides (CD), lipoperox...

journal_title：Blood

authors： Livrea MA,Tesoriere L,Pintaudi AM,Calabrese A,Maggio A,Freisleben HJ,D'Arpa D,D'Anna R,Bongiorno A

更新日期：1996-11-01 00:00:00

abstract：:Toll-like receptors orchestrate rapid local protective innate-immune responses to invading pathogens and optimize leukocyte priming of subsequent adaptive responses. Paradoxically, systemic excess of the TLR2 ligand, bacterial lipoprotein (BLP), suppresses peripheral inflammatory responses. Here, we demonstrate that t...

journal_title：Blood

authors： McKimmie CS,Moore M,Fraser AR,Jamieson T,Xu D,Burt C,Pitman NI,Nibbs RJ,McInnes IB,Liew FY,Graham GJ

更新日期：2009-04-30 00:00:00

abstract：:Patients with antineutrophil cytoplasmic antibodies (ANCAs) frequently develop severe vasculitis and glomerulonephritis. Although ANCAs, particularly antimyeloperoxidase (anti-MPO), have been shown to promote leukocyte adhesion in postcapillary venules, their ability to promote adhesion in the glomerular vasculature i...

journal_title：Blood

authors： Kuligowski MP,Kwan RY,Lo C,Wong C,James WG,Bourges D,Ooi JD,Abeynaike LD,Hall P,Kitching AR,Hickey MJ

更新日期：2009-06-18 00:00:00

abstract：:Cytogenetics is considered one of the most valuable prognostic determinants in acute myeloid leukemia (AML). However, many studies on which this assertion is based were limited by relatively small sample sizes or varying treatment approach, leading to conflicting data regarding the prognostic implications of specific ...

journal_title：Blood

authors： Grimwade D,Walker H,Oliver F,Wheatley K,Harrison C,Harrison G,Rees J,Hann I,Stevens R,Burnett A,Goldstone A

更新日期：1998-10-01 00:00:00

abstract：:Leukemia-cell expression of ZAP-70, CD38, or unmutated immunoglobulin heavy chain variable region genes (U-IGHV) each is associated with aggressive disease in patients with chronic lymphocytic leukemia (CLL). To assess the relative strength of each marker, we defined thresholds for designating a case as positive for C...

journal_title：Blood

authors： Rassenti LZ,Jain S,Keating MJ,Wierda WG,Grever MR,Byrd JC,Kay NE,Brown JR,Gribben JG,Neuberg DS,He F,Greaves AW,Rai KR,Kipps TJ

更新日期：2008-09-01 00:00:00

abstract：:Recombinant human macrophage colony-stimulating factor (rhM-CSF) was given to cynomolgus monkeys by continuous intravenous infusion or subcutaneous injection, at a dose of 50 to 100 micrograms/kg/d in repetitive 14-day cycles. Starting within 24 to 48 hours of initiation of rhM-CSF, there was a progressive increase in...

journal_title：Blood

authors： Munn DH,Garnick MB,Cheung NK

更新日期：1990-05-15 00:00:00

abstract：:Peripheral blood mononuclear cells (PBMC) from 17 patients receiving HLA-identical sibling bone marrow grafts were stimulated with host pretransplant PBMC. Cytotoxic T-cell lines (TCL) with specificity for host pretransplant PBMC were obtained from 9 of these patients, all presenting with severe graft-versus-host dise...

journal_title：Blood

authors： Niederwieser D,Grassegger A,Auböck J,Herold M,Nachbaur D,Rosenmayr A,Gächter A,Nussbaumer W,Gaggl S,Ritter M

更新日期：1993-04-15 00:00:00

abstract：:Granulocytic colonies grown in culture from marrow and peripheral blood from five patients with Ph1-positive CML and heterozygous at the G-6-PD locus were analyzed for G-6-PD in order to identify CFU-C that do not arise from the CML clone. The patients had both B and A enzymes in normal tissues, but their CML clones t...

journal_title：Blood

authors： Singer JW,Fialkow PJ,Steinmann L,Najfeld V,Stein SJ,Robinson WA

更新日期：1979-02-01 00:00:00

abstract：:Human CD100, the first semaphorin identified in the immune system, is a transmembrane protein involved in T-cell activation. In the present study, we showed that activation of peripheral blood or tonsillar B lymphocytes induced the expression of CD100 in CD38(+)CD138(-) cell populations, including in CD148(+) subpopul...

journal_title：Blood

authors： Billard C,Delaire S,Raffoux E,Bensussan A,Boumsell L

更新日期：2000-02-01 00:00:00

abstract：:Epinephrine causes platelet aggregation and secretion by interacting with alpha 2-adrenergic receptors on the platelet surface. Platelet aggregation requires the binding of fibrinogen to a specific receptor on the membrane glycoprotein IIb-IIIa complex. Although the IIb-IIIa complex is identifiable on the surface of r...

journal_title：Blood

authors： Shattil SJ,Motulsky HJ,Insel PA,Flaherty L,Brass LF

更新日期：1986-12-01 00:00:00

abstract：:Multiple myeloma (MM) is associated with severe normochromic/normocytic anemia. This study demonstrates that the abnormal up-regulation of apoptogenic receptors, including both Fas ligand (L) and tumor necrosis factor-related apoptosis-inducing ligand (TRAIL), by highly malignant myeloma cells is involved in the patho...

journal_title：Blood

authors： Silvestris F,Cafforio P,Tucci M,Dammacco F

更新日期：2002-02-15 00:00:00

abstract：:Mitochondrial DNA (mtDNA) biosynthesis requires replication factors and adequate nucleotide pools from the mitochondria and cytoplasm. We performed gene expression profiling analysis of 542 human acute myeloid leukemia (AML) samples and identified 55% with upregulated mtDNA biosynthesis pathway expression compared wit...

journal_title：Blood

authors： Liyanage SU,Hurren R,Voisin V,Bridon G,Wang X,Xu C,MacLean N,Siriwardena TP,Gronda M,Yehudai D,Sriskanthadevan S,Avizonis D,Shamas-Din A,Minden MD,Bader GD,Laposa R,Schimmer AD

更新日期：2017-05-11 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

authors： Ajzner E,Schlammadinger A,Kerényi A,Bereczky Z,Katona E,Haramura G,Boda Z,Muszbek L

更新日期：2009-01-15 00:00:00