Genetic basis of hemolytic anemia caused by pyrimidine 5' nucleotidase deficiency.

abstract：:T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive malignancy that accounts for ∼20% of ALL cases. Intensive chemotherapy regimens result in cure rates >85% in children and <50% in adults, warranting a search of novel therapeutic strategies. Although immune-based therapies have tremendously improved the trea...

journal_title：Blood

authors： Tran Quang C,Zaniboni B,Humeau R,Lengliné E,Dourthe ME,Ganesan R,Singh S,Scheer JM,Asnafi V,Ghysdael J

更新日期：2020-09-10 00:00:00

abstract：:P-selectin, an endothelial leukocyte adhesion receptor, is rapidly translocated to the cell surface upon release from storage granules called Weibel-Palade bodies and is also transcriptionally upregulated upon cytokine stimulation of endothelial cells (ECs). These two pathways of surface expression are coincident with...

journal_title：Blood

authors： Barkalow FJ,Goodman MJ,Gerritsen ME,Mayadas TN

更新日期：1996-12-15 00:00:00

abstract：:Six hundred twelve eligible, previously untreated patients with active multiple myeloma and at least some data available for analysis were entered into a randomized trial (Southwest Oncology Group [SWOG] Phase III myeloma study 8229/30), in which the prognostic significance of pretreatment serum beta 2 microglobulin l...

journal_title：Blood

authors： Durie BG,Stock-Novack D,Salmon SE,Finley P,Beckord J,Crowley J,Coltman CA

更新日期：1990-02-15 00:00:00

abstract：:Ristocetin and vancomycin are structurally similar glycopeptide antibiotics. Both vancomycin and ristocetin in high concentrations (3.0 mg/ml) cause the precipitation of fibrinogen, plasminogen, and IgG from platelet-poor plasma (PPP). In contrast to ristocetin, vanomycin (0.5-1.5 mg/ml) does not agglutinate platelets...

journal_title：Blood

authors： Moake JL,Cimo PL,Peterson DM,Roper P,Natelson EA

更新日期：1977-09-01 00:00:00

abstract：:In the past 5 years we have witnessed significant advances in both the diagnostic process and optimal therapy for patients with essential thrombocythemia (ET). Insights into the underlying molecular mechanisms have been accompanied by the development of new diagnostic tests and by an improved understanding of the rela...

journal_title：Blood

authors： Beer PA,Erber WN,Campbell PJ,Green AR

更新日期：2011-02-03 00:00:00

abstract：:Neonatal alloimmune thrombocytopenia (NAIT) can cause severe bleeding in the central nervous system (CNS) and death or severe neurologic sequelae. The expression of the PLA1 antigen is detectable as early as 19 weeks of gestation. Alloimmunization can therefore lead to fetal thrombocytopenia very early in pregnancy. U...

journal_title：Blood

authors： Kaplan C,Daffos F,Forestier F,Cox WL,Lyon-Caen D,Dupuy-Montbrun MC,Salmon C

更新日期：1988-07-01 00:00:00

abstract：:The production of interleukin-1beta (IL-1beta), a powerful mediator of inflammation, is tightly regulated at several levels. However, in some pathologic conditions, a pharmacologic treatment is required to control the toxicity of excessive extracellular IL-1beta. Because of the heavy side effects of most therapies use...

journal_title：Blood

authors： Hamon Y,Luciani MF,Becq F,Verrier B,Rubartelli A,Chimini G

更新日期：1997-10-15 00:00:00

abstract：:The putative chemokine receptor BLR1 has been identified as the first G-protein-coupled receptor involved in B-cell migration and in microenvironmental homing to B-cell follicles and to germinal centers. In healthy individuals, expression of BLR1 is restricted to all mature recirculating B cells and to a subpopulation...

journal_title：Blood

authors： Förster R,Schweigard G,Johann S,Emrich T,Kremmer E,Nerl C,Lipp M

更新日期：1997-07-15 00:00:00

abstract：:Steroid-resistant or steroid-refractory acute graft-versus-host disease (SR-aGVHD) poses one of the most vexing challenges faced by providers who care for patients after allogeneic hematopoietic cell transplantation. For the past 4 decades, research in the field has been driven by the premise that persistent graft-ver...

journal_title：Blood

authors： Martin PJ

更新日期：2020-05-07 00:00:00

abstract：:Intracellular oxidative stress in cells transformed by the BCR-ABL oncogene is associated with increased DNA double-strand breaks. Imprecise repair of these breaks can result in the accumulation of mutations, leading to therapy-related drug resistance and disease progression. Using several BCR-ABL model systems, we fo...

journal_title：Blood

authors： Fernandes MS,Reddy MM,Gonneville JR,DeRoo SC,Podar K,Griffin JD,Weinstock DM,Sattler M

更新日期：2009-08-27 00:00:00

abstract：:Activated neutrophils have the ability to upregulate the expression of many genes, in particular those encoding cytokines and chemokines, and to subsequently release the corresponding proteins. Although little is known to date concerning the regulation of gene transcription in neutrophils, it is noteworthy that many o...

journal_title：Blood

authors： McDonald PP,Bald A,Cassatella MA

更新日期：1997-05-01 00:00:00

abstract：:Previous studies demonstrated that Kit activation confers radioprotection. However, the mechanism by which Kit signaling interferes with cellular response to ionizing radiation (IR) has not been firmly established. Based on the role of the sphingomyelin (SM) cycle apoptotic pathway in IR-induced apoptosis, we hypothes...

journal_title：Blood

authors： Maddens S,Charruyer A,Plo I,Dubreuil P,Berger S,Salles B,Laurent G,Jaffrézou JP

更新日期：2002-08-15 00:00:00

abstract：:The p21-activated kinases (Paks) are serine/threonine kinases that are major effectors of the Rho guanosine 5'\x{2011}triphosphatase, Rac, and Cdc42. Rac and Cdc42 are known regulators of hematopoietic stem and progenitor cell (HSPC) function, however, a direct role for Paks in HSPCs has yet to be elucidated. Lin(-)Sc...

journal_title：Blood

authors： Dorrance AM,De Vita S,Radu M,Reddy PN,McGuinness MK,Harris CE,Mathieu R,Lane SW,Kosoff R,Milsom MD,Chernoff J,Williams DA

更新日期：2013-03-28 00:00:00

abstract：:We have isolated and characterized the human cardiac mast cell (CMC) and compared this novel mast cell (MC type with MC obtained from uterus, skin, and lung. Heart tissue was obtained from 14 patients with cardiomyopathy (CMP, heart transplantation). CMC were isolated by enzymatic digestion using collagenase, pronase-...

journal_title：Blood

authors： Sperr WR,Bankl HC,Mundigler G,Klappacher G,Grossschmidt K,Agis H,Simon P,Laufer P,Imhof M,Radaszkiewicz T,Glogar D,Lechner K,Valent P

更新日期：1994-12-01 00:00:00

abstract：:Murine autoreactive anti-Smith (Sm) B cells are negatively regulated by anergy and developmental arrest, but are also positively selected into the marginal zone (MZ) and B-1 B-cell populations. Despite positive selection, anti-Sm production occurs only in autoimmune-prone mice. To investigate autoreactive B-cell activ...

journal_title：Blood

authors： Qian Y,Conway KL,Lu X,Seitz HM,Matsushima GK,Clarke SH

更新日期：2006-08-01 00:00:00

abstract：:Early B-cell factor 1 (Ebf1) is a transcription factor with documented dose-dependent functions in normal and malignant B-lymphocyte development. To understand more about the roles of Ebf1 in malignant transformation, we investigated the impact of reduced functional Ebf1 dosage on mouse B-cell progenitors. Gene expres...

journal_title：Blood

authors： Prasad MA,Ungerbäck J,Åhsberg J,Somasundaram R,Strid T,Larsson M,Månsson R,De Paepe A,Lilljebjörn H,Fioretos T,Hagman J,Sigvardsson M

更新日期：2015-06-25 00:00:00

abstract：:Human promyelocytic leukemia (HL-60) cells were induced to differentiate into macrophage-like cells by treatment with 10(-7) mol/L 1,25-dihydroxyvitamin D3 (VD3). A monoclonal antibody (MoAb, 60B8), reactive with the particulate of the differentiated cells but not of the untreated cells, was isolated. The antigen reco...

journal_title：Blood

authors： Nozawa R,Kato H,Ito T,Yokota T

更新日期：1988-05-01 00:00:00

abstract：:We treated 5 patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and multifocal bone lesions or diffuse bone marrow plasmacytic infiltration with high-dose therapy (HDT) and autologous blood stem cell transplantation. In all cases, the treatment produced...

journal_title：Blood

authors： Jaccard A,Royer B,Bordessoule D,Brouet JC,Fermand JP

更新日期：2002-04-15 00:00:00

abstract：:While studying patient plasma containing an unusual pattern of von Willebrand factor (VWF) multimers, we discovered a previously unreported phenomenon: heavy predominance of dimeric VWF. Genomic analysis revealed a new congenital mutation (Tyr87Ser) that altered the final stages of VWF biosynthesis. This mutation in t...

journal_title：Blood

authors： Rosenberg JB,Haberichter SL,Jozwiak MA,Vokac EA,Kroner PA,Fahs SA,Kawai Y,Montgomery RR

更新日期：2002-09-01 00:00:00

abstract：:The present study focuses on a large family with an X-linked immunodeficiency in which there are variable clinical and laboratory phenotypes, including recurrent viral and bacterial infections, hypogammaglobulinemia, Epstein-Barr virus-driven lymphoproliferation, splenomegaly, colitis, and liver disease. Molecular and...

journal_title：Blood

authors： Rigaud S,Lopez-Granados E,Sibéril S,Gloire G,Lambert N,Lenoir C,Synaeve C,Stacey M,Fugger L,Stephan JL,Fischer A,Picard C,Durandy A,Chapel H,Latour S

更新日期：2011-07-14 00:00:00

abstract：:Graft-versus-host disease (GVHD) is a major complication of allogeneic bone marrow transplantation (BMT). When GVHD is controlled by T-cell-depleted grafts or immunosuppressants, BM transplant recipients often suffer from an increased rate of leukemic relapse and impaired reconstitution of immunity. Using a mouse BMT ...

journal_title：Blood

authors： Imado T,Iwasaki T,Kataoka Y,Kuroiwa T,Hara H,Fujimoto J,Sano H

更新日期：2004-09-01 00:00:00

abstract：:Insights into immune-mediated thrombotic thrombocytopenic purpura (iTTP) pathophysiology have led to novel targeted therapies. Immunomodulatory strategies target anti-ADAMTS13 antibodies: rituximab is effective in inducing responses in refractory/relapsed TTP and increasing relapse-free survival; caplacizumab targets ...

journal_title：Blood

authors： Mazepa MA,Masias C,Chaturvedi S

更新日期：2019-08-01 00:00:00

abstract：:Ad.CMV-CD is a replication incompetent adenoviral vector carrying a cytomegalovirus (CMV)-driven transcription unit of the cytosine deaminase (CD) gene. The CD transcription unit in this vector catalyzes the deamination of the nontoxic pro-drug, 5-fluorocytosine (5-FC), thus converting it to the cytotoxic drug 5-fluor...

journal_title：Blood

authors： Garcia-Sanchez F,Pizzorno G,Fu SQ,Nanakorn T,Krause DS,Liang J,Adams E,Leffert JJ,Yin LH,Cooperberg MR,Hanania E,Wang WL,Won JH,Peng XY,Cote R,Brown R,Burtness B,Giles R,Crystal R,Deisseroth AB

更新日期：1998-07-15 00:00:00

abstract：:Recurrent mutations in isocitrate dehydrogenase 2 (IDH2) occur in ∼12% of patients with acute myeloid leukemia (AML). Mutated IDH2 proteins neomorphically synthesize 2-hydroxyglutarate resulting in DNA and histone hypermethylation, which leads to blocked cellular differentiation. Enasidenib (AG-221/CC-90007) is a firs...

journal_title：Blood

authors： Stein EM,DiNardo CD,Pollyea DA,Fathi AT,Roboz GJ,Altman JK,Stone RM,DeAngelo DJ,Levine RL,Flinn IW,Kantarjian HM,Collins R,Patel MR,Frankel AE,Stein A,Sekeres MA,Swords RT,Medeiros BC,Willekens C,Vyas P,Tosolini A

更新日期：2017-08-10 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：:To evaluate outcome and prognosis of patients with T-cell lymphoma we analyzed 343 patients treated within trials of the German High-Grade Non-Hodgkin Lymphoma Study Group (DSHNHL). Two hundred eighty-nine patients belonged to 1 of the 4 major T-cell lymphoma subtypes: anaplastic large cell lymphoma (ALCL), anaplastic...

journal_title：Blood

authors： Schmitz N,Trümper L,Ziepert M,Nickelsen M,Ho AD,Metzner B,Peter N,Loeffler M,Rosenwald A,Pfreundschuh M

更新日期：2010-11-04 00:00:00

abstract：:Thrombopoietin (TPO) is a hematopoietic growth factor that regulates megakaryocytopoiesis and platelet production through binding to its receptor, Mpl, encoded by the c-mpl proto-oncogene. Circulating levels of TPO are regulated by receptor-mediated uptake and degradation. To better understand this mode of TPO regulat...

journal_title：Blood

authors： Cohen-Solal K,Vitrat N,Titeux M,Vainchenker W,Wendling F

更新日期：1999-05-01 00:00:00

abstract：:Activation of Janus tyrosine kinases (Jak) and Signal transducers and activators of transcription (Stat) after ligation of major histocompatibility complex class I (MHC-I) was explored in Jurkat T cells. Cross-linking of MHC-I mediated tyrosine phosphorylation of Tyk2, but not Jak1, Jak2, and Jak3. In addition, the tr...

journal_title：Blood

authors： Skov S,Nielsen M,Bregenholt S,Odum N,Claesson MH

更新日期：1998-05-15 00:00:00

abstract：:T cell-based therapies have much promise in cancer treatment. This approach may be enhanced if used in combination with radiotherapy provided that tumor-specific T cells can be protected against the effects of radiotherapy. Previously, we demonstrated that administration of TLR9 ligand into mice decreased activation- ...

journal_title：Blood

authors： Zheng L,Asprodites N,Keene AH,Rodriguez P,Brown KD,Davila E

更新日期：2008-03-01 00:00:00

abstract：:Mantle cell lymphoma (MCL) is a highly aggressive B-cell lymphoma resistant to conventional chemotherapy. Although defined by the characteristic t(11;14) translocation, MCL has not been recapitulated in transgenic mouse models of cyclin D1 overexpression alone. Indeed, several genetic aberrations have been identified ...

journal_title：Blood

authors： Katz SG,Labelle JL,Meng H,Valeriano RP,Fisher JK,Sun H,Rodig SJ,Kleinstein SH,Walensky LD

更新日期：2014-02-06 00:00:00