Results of a phase 2 trial of an oral CXCR4 antagonist, mavorixafor, for treatment of WHIM syndrome.

abstract：:We have investigated the role of adenosine diphosphate (ADP) receptors in the adhesion, activation, and aggregation of platelets perfused over immobilized von Willebrand factor (VWF) under high shear stress. Blocking P2Y(1) prevented stable platelet adhesion and aggregation, indicative of a complete inhibition of alph...

journal_title：Blood

authors： Mazzucato M,Cozzi MR,Pradella P,Ruggeri ZM,De Marco L

更新日期：2004-11-15 00:00:00

abstract：:We have previously demonstrated a significant inverse correlation between circulating thrombopoietin (TPO) levels and peripheral platelet (PLT) counts in patients with thrombocytopenia secondary to megakaryocytic hypoplasia but not in patients with immune thrombocytopenic purpura (ITP; Chang et al, Blood 88:3354, 1996...

journal_title：Blood

authors： Chang M,Qian JX,Lee SM,Joubran J,Fernandez G,Nichols J,Knoppel A,Buzby JS

更新日期：1999-04-15 00:00:00

abstract：:Most patients with acute myeloid leukemia (AML) enter complete remission (CR) after treatment with chemotherapy, but a large number of them experience relapse with resistant disease. To identify genes that are associated with their prognoses, we analyzed gene expression in 54 pediatric patients with AML using an oligo...

journal_title：Blood

authors： Yagi T,Morimoto A,Eguchi M,Hibi S,Sako M,Ishii E,Mizutani S,Imashuku S,Ohki M,Ichikawa H

更新日期：2003-09-01 00:00:00

abstract：:A cohort of MDS patients was examined for mutations affecting 4 splice genes (SF3B1, SRSF2, ZRSR2, and U2AF35) and evaluated in the context of clinical and molecular markers. Splice gene mutations were detected in 95 of 221 patients. These mutations were mutually exclusive and less likely to occur in patients with com...

journal_title：Blood

authors： Damm F,Kosmider O,Gelsi-Boyer V,Renneville A,Carbuccia N,Hidalgo-Curtis C,Della Valle V,Couronné L,Scourzic L,Chesnais V,Guerci-Bresler A,Slama B,Beyne-Rauzy O,Schmidt-Tanguy A,Stamatoullas-Bastard A,Dreyfus F,Prébet T,

更新日期：2012-04-05 00:00:00

abstract：:Hematopoietic stem cells (HSCs) show pronounced heterogeneity in self-renewal and differentiation behavior, which is reflected in their repopulation kinetics. Here, a single-cell-based mathematical model of HSC organization is used to examine the basis of HSC heterogeneity. Our modeling results, which are based on the...

journal_title：Blood

authors： Roeder I,Horn K,Sieburg HB,Cho R,Muller-Sieburg C,Loeffler M

更新日期：2008-12-15 00:00:00

abstract：:Bortezomib (PS-341), a selective inhibitor of proteasomes, induces apoptosis in multiple myeloma (MM) cells; however, prolonged drug exposure may result in cumulative toxicity and the development of chemoresistance. Here we show that combining PK-11195 (PK), an antagonist to mitochondrial peripheral benzodiazepine rec...

journal_title：Blood

authors： Chauhan D,Li G,Podar K,Hideshima T,Mitsiades C,Schlossman R,Munshi N,Richardson P,Cotter FE,Anderson KC

更新日期：2004-10-15 00:00:00

abstract：:Sardinian beta-thalassemia patients all are homozygotes for the same null allele in the beta-globin gene, but the clinical manifestations are extremely variable in severity. Previous studies have shown that the coinheritance of alpha-thalassemia or the presence of genetic variants that sustain fetal hemoglobin product...

journal_title：Blood

authors： Galanello R,Sanna S,Perseu L,Sollaino MC,Satta S,Lai ME,Barella S,Uda M,Usala G,Abecasis GR,Cao A

更新日期：2009-10-29 00:00:00

abstract：:Elevated circulatory levels of many blood coagulation factors are known to be a risk factor for deep vein thrombosis in humans. Here we report the first direct demonstration of a close association between elevated circulatory factor IX levels in mice with thrombosis as well as myocardial fibrosis. Transgenic mice over...

journal_title：Blood

authors： Ameri A,Kurachi S,Sueishi K,Kuwahara M,Kurachi K

更新日期：2003-03-01 00:00:00

abstract：:Gastrointestinal (GI) bleeding is distinctive of severe von Willebrand disease (VWD), generally arising in older patients; in most cases, blood transfusion and hospitalization are required. The presence of arteriovenous malformations is often described when endoscopic examinations are performed. Patients with congenit...

journal_title：Blood

authors： Biguzzi E,Siboni SM,Peyvandi F

更新日期：2020-09-03 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

authors： Daenthanasanmak A,Iamsawat S,Chakraborty P,Nguyen HD,Bastian D,Liu C,Mehrotra S,Yu XZ

更新日期：2019-01-17 00:00:00

abstract：:In this investigation, we studied the importance of cellular glutathione (GSH) in the hexose monophosphate shunt (HMPS) activity of unstimulated human erythrocytes and the mechanism by which pyruvate stimulates the HMPS. The rate of HMPS activity was measured by the production of radioactive CO2 from 14C-1-glucose or ...

journal_title：Blood

authors： Sagone AL Jr,Balcerzak SP,Metz EN

更新日期：1975-01-01 00:00:00

abstract：:Signaling through the Notch pathway plays an essential role in inducing T-lineage commitment and promoting the maturation of immature thymocytes. Using an in vitro culture system, we show that 2 different classes of Notch ligands, Jagged1 or Delta1, transmit distinct signals to T-cell progenitors. OP9 stromal cells ex...

journal_title：Blood

authors： Lehar SM,Dooley J,Farr AG,Bevan MJ

更新日期：2005-02-15 00:00:00

abstract：:Adaptive immunity is triggered at the immune synapse, where peptide-major histocompatibility complexes and costimulatory molecules expressed by dendritic cells (DCs) are physically presented to T cells. Here we describe transmission of the inflammatory monoamine serotonin (5-hydroxytryptamine [5-HT]) between these cel...

journal_title：Blood

authors： O'Connell PJ,Wang X,Leon-Ponte M,Griffiths C,Pingle SC,Ahern GP

更新日期：2006-02-01 00:00:00

abstract：:The common γ chain (CD132) is a subunit of the interleukin (IL) receptors for IL-2, IL-4, IL-7, IL-9, IL-15, and IL-21. Because levels of several of these cytokines were shown to be increased in the serum of patients developing acute and chronic graft-versus-host disease (GVHD), we reasoned that inhibition of CD132 co...

journal_title：Blood

authors： Hechinger AK,Smith BA,Flynn R,Hanke K,McDonald-Hyman C,Taylor PA,Pfeifer D,Hackanson B,Leonhardt F,Prinz G,Dierbach H,Schmitt-Graeff A,Kovarik J,Blazar BR,Zeiser R

更新日期：2015-01-15 00:00:00

abstract：:We have been transducing mouse hematopoietic cells with the human MDR1 (MDR) gene in retroviral vectors to determine the optimal conditions for retroviral gene transfer as a model system for potential human gene therapy. In these studies, we have demonstrated transduction and expression of the human MDR gene using eco...

journal_title：Blood

authors： Richardson C,Ward M,Podda S,Bank A

更新日期：1994-07-15 00:00:00

abstract：:We have used two in vitro models to identify genes whose expression may serve as markers of lineage commitment during the development of hematopoietic stem cells. One system involves the development in vitro of blastocyst-derived embryonic stem cells into embryoid bodies. The second involves culturing of day 3.5 blast...

journal_title：Blood

authors： McClanahan T,Dalrymple S,Barkett M,Lee F

更新日期：1993-06-01 00:00:00

abstract：:The human leukocyte integrin subunit CD11b is expressed predominantly on myelomonocytic cells. To identify CD11b promoter sequences important for myelomonocytic gene expression and to assess the utility of the CD11b promoter for expressing heterologous genes in vivo, we generated transgenic mice with a human CD4 repor...

journal_title：Blood

authors： Back A,East K,Hickstein D

更新日期：1995-02-15 00:00:00

abstract：:We describe glycoprotein (GP) Ib as a mediator of adhesion to fibronectin, specifically in flow. A monoclonal antibody (MoAb) directed to the von Willebrand factor (vWF)-binding site on this receptor or the absence of this receptor on the platelet membrane, in the case of a patient with the Bernard-Soulier syndrome, r...

journal_title：Blood

authors： Beumer S,Heijnen HF,IJsseldijk MJ,Orlando E,de Groot PG,Sixma JJ

更新日期：1995-11-01 00:00:00

abstract：:Monoclonal gammopathy of undetermined significance (MGUS) is present in ∼2% of individuals age >50 years. The increased risk of multiple myeloma (MM) in relatives of individuals with MGUS is consistent with MGUS being a marker of inherited genetic susceptibility to MM. Common single-nucleotide polymorphisms (SNPs) at ...

journal_title：Blood

authors： Weinhold N,Johnson DC,Rawstron AC,Försti A,Doughty C,Vijayakrishnan J,Broderick P,Dahir NB,Begum DB,Hosking FJ,Yong K,Walker BA,Hoffmann P,Mühleisen TW,Langer C,Dörner E,Jöckel KH,Eisele L,Nöthen MM,Hose D,Davies

更新日期：2014-04-17 00:00:00

abstract：:Lym-1 is a murine IgG2a monoclonal antibody that recognizes a polymorphic variant of HLA-DR antigens on malignant B cells, with minimal cross-reactivity with normal tissues. Because it can be safely administered in vivo, a detailed knowledge of its ability to recruit and trigger the antitumor immune effector systems i...

journal_title：Blood

authors： Ottonello L,Morone P,Dapino P,Dallegri F

更新日期：1996-06-15 00:00:00

abstract：:Eph receptors and their ephrin ligands are involved in normal hematopoietic development and tumorigenesis. Using methylated CpG island amplification/DNA promoter microarray, we identified several EPH receptor and EPHRIN genes as potential hypermethylation targets in acute lymphoblastic leukemia (ALL). We subsequently ...

journal_title：Blood

authors： Kuang SQ,Bai H,Fang ZH,Lopez G,Yang H,Tong W,Wang ZZ,Garcia-Manero G

更新日期：2010-03-25 00:00:00

abstract：:The HLX gene encoding a diverged homeobox transcription factor has been found to be up-regulated by vascular endothelial growth factor-A (VEGF-A) in endothelial cells. We have now investigated the gene repertoire induced by HLX and its potential biologic function. HLX strongly increased the transcripts for several rep...

journal_title：Blood

authors： Testori J,Schweighofer B,Helfrich I,Sturtzel C,Lipnik K,Gesierich S,Nasarre P,Hofer-Warbinek R,Bilban M,Augustin HG,Hofer E

更新日期：2011-03-03 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

authors： Zippo A,De Robertis A,Bardelli M,Galvagni F,Oliviero S

更新日期：2004-06-15 00:00:00

abstract：:Natural killer (NK) cells play critical roles in protection against hematological malignancies but can acquire a dysfunctional state, which limits antitumor immunity. However, the underlying reasons for this impaired NK cell function remain to be uncovered. We found that NK cells in aggressive B-cell lymphoma underwen...

journal_title：Blood

authors： Kobayashi T,Lam PY,Jiang H,Bednarska K,Gloury R,Murigneux V,Tay J,Jacquelot N,Li R,Tuong ZK,Leggatt GR,Gandhi MK,Hill MM,Belz GT,Ngo S,Kallies A,Mattarollo SR

更新日期：2020-12-24 00:00:00

abstract：:We analyzed the involvement of Wiskott-Aldrich syndrome protein (WASp), a critical regulator of actin cytoskeleton remodeling, in the control of natural killer (NK)-cell migration. NK cells derived from patients with Wiskott-Aldrich syndrome/X-linked thrombocytopenia (WAS/XLT), carrying different mutations in the WASP...

journal_title：Blood

authors： Stabile H,Carlino C,Mazza C,Giliani S,Morrone S,Notarangelo LD,Notarangelo LD,Santoni A,Gismondi A

更新日期：2010-04-08 00:00:00

abstract：:Previous studies of follicular lymphoma (FL) patients treated heterogeneously have suggested that decreased numbers of regulatory T cells correlates with improved survival. We studied advanced-stage FL patients from a single institution phase 2 trial. All patients were treated uniformly with multiagent chemotherapy an...

journal_title：Blood

authors： Farinha P,Al-Tourah A,Gill K,Klasa R,Connors JM,Gascoyne RD

更新日期：2010-01-14 00:00:00

abstract：:The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 patients with POEMS syndrome. Minimal criteria were a...

journal_title：Blood

authors： Dispenzieri A,Kyle RA,Lacy MQ,Rajkumar SV,Therneau TM,Larson DR,Greipp PR,Witzig TE,Basu R,Suarez GA,Fonseca R,Lust JA,Gertz MA

更新日期：2003-04-01 00:00:00

abstract：:We show here that alpha2(G-Phila.) beta2(C) has an increased rate of crystal nucleation compared to alpha2 beta2(C) (HbC). We conclude from this finding that position alpha68, the mutation site of alpha2(G-Phila.) beta2 (HbG(Philadelphia)), is a contact site in the crystal of HbC. In addition, that HbS enhances HbC cr...

journal_title：Blood

authors： Lawrence C,Hirsch RE,Fataliev NA,Patel S,Fabry ME,Nagel RL

更新日期：1997-10-01 00:00:00

abstract：:Congenital thrombocytopenia may occur in isolation or accompanied by eczema and immunodeficiency, as part of the X-linked hereditary Wiskott-Aldrich syndrome (WAS). Because the clinical and immunologic picture of WAS is variable, particularly early in life, definite diagnosis cannot always be made in cases with a nega...

journal_title：Blood

authors： Puck JM,Siminovitch KA,Poncz M,Greenberg CR,Rottem M,Conley ME

更新日期：1990-06-15 00:00:00

abstract：:Interleukin-25 (IL-25) is a recently described T helper 2 (T(H)2) cell-derived cytokine that belongs to the IL-17 family and induces the production of IL-4, IL-5, and IL-13 from an unidentified non-T-cell population. Here, we show that mast cells are also potent IL-25-producing cells. When bone marrow-derived mast cel...

journal_title：Blood

authors： Ikeda K,Nakajima H,Suzuki K,Kagami S,Hirose K,Suto A,Saito Y,Iwamoto I

更新日期：2003-05-01 00:00:00