The architecture of the IgG anti-carbohydrate repertoire in primary antibody deficiencies.

abstract：:Hairy cell leukemia variant (HCLv) presents with high disease burden, lack of typical antigens like CD25, and poor response to standard treatments like cladribine. Occasionally, patients with classic HCL respond poorly. Clinical and molecular features of HCL and HCLv has not been compared. Rearrangements expressing im...

journal_title：Blood

authors： Arons E,Suntum T,Stetler-Stevenson M,Kreitman RJ

更新日期：2009-11-19 00:00:00

abstract：:Bcr-Abl tyrosine kinase activity initiates a number of intracellular signaling cascades that result in leukemogenesis. Imatinib mesylate, a specific Bcr-Abl tyrosine kinase inhibitor, has been highly successful in the treatment of chronic myelogenous leukemia (CML). However, the emergence of imatinib resistance and th...

journal_title：Blood

authors： Gu JJ,Santiago L,Mitchell BS

更新日期：2005-04-15 00:00:00

abstract：:Mutations of IDH1 and IDH2, which produce the oncometabolite 2-hydroxyglutarate (2HG), have been identified in several tumors, including acute myeloid leukemia. Recent studies have shown that expression of the IDH mutant enzymes results in high levels of 2HG and a block in cellular differentiation that can be reversed...

journal_title：Blood

authors： Kernytsky A,Wang F,Hansen E,Schalm S,Straley K,Gliser C,Yang H,Travins J,Murray S,Dorsch M,Agresta S,Schenkein DP,Biller SA,Su SM,Liu W,Yen KE

更新日期：2015-01-08 00:00:00

abstract：:The ultrastructure of the lymphocytes from three children with severe combined immunodeficiency disease (SCID) is described. Parallel tubular arrays (PTA) were found in a large percentage of circulating lymphocytes (53%, 41%, and 13%) in three SCID patients when compared to age-matched controls. The size of these incl...

journal_title：Blood

authors： Payne CM,Jones JF,Sieber OF Jr,Fulginiti VA

更新日期：1977-07-01 00:00:00

abstract：:Tissue-derived adenosine, acting via the adenosine A(2A) receptor (A(2A)R), is emerging as an important negative regulator of T-cell function. In this report, we demonstrate that A(2A)R stimulation not only inhibits the generation of adaptive effector T cells but also promotes the induction of adaptive regulatory T ce...

journal_title：Blood

authors： Zarek PE,Huang CT,Lutz ER,Kowalski J,Horton MR,Linden J,Drake CG,Powell JD

更新日期：2008-01-01 00:00:00

abstract：:Heparin-induced thrombocytopenia (HIT) with thrombosis is a serious complication of heparin use. HIT sera can generate platelet-derived microparticles, which are produced in a heparin-dependent manner and are hypothesized to be important initial pathological participants because they promote vascular occlusion. To dat...

journal_title：Blood

authors： Hughes M,Hayward CP,Warkentin TE,Horsewood P,Chorneyko KA,Kelton JG

更新日期：2000-07-01 00:00:00

abstract：:Sickle cell disease is a common hemolytic disorder with a broad range of complications, including vaso-occlusive episodes, acute chest syndrome (ACS), pain, and stroke. Heme oxygenase-1 (gene HMOX1; protein HO-1) is the inducible, rate-limiting enzyme in the catabolism of heme and might attenuate the severity of outco...

journal_title：Blood

authors： Bean CJ,Boulet SL,Ellingsen D,Pyle ME,Barron-Casella EA,Casella JF,Payne AB,Driggers J,Trau HA,Yang G,Jones K,Ofori-Acquah SF,Hooper WC,DeBaun MR

更新日期：2012-11-01 00:00:00

abstract：:Sickle cell anemia (SCA) is an inherited disorder of beta-globin, resulting in red blood cell rigidity, anemia, painful crises, organ infarctions, and reduced life expectancy. Allogeneic blood or marrow transplantation (BMT) can cure SCA but is associated with an 8% to 10% mortality rate, primarily from complications ...

journal_title：Blood

authors： Iannone R,Luznik L,Engstrom LW,Tennessee SL,Askin FB,Casella JF,Kickler TS,Goodman SN,Hawkins AL,Griffin CA,Noffsinger L,Fuchs EJ

更新日期：2001-06-15 00:00:00

abstract：:Although recent data suggests that osteoblasts play a key role within the hematopoietic stem cell (HSC) niche, the mechanisms underpinning this remain to be fully defined. The studies described herein examine the role in hematopoiesis of Osteopontin (Opn), a multidomain, phosphorylated glycoprotein, synthesized by ost...

journal_title：Blood

authors： Nilsson SK,Johnston HM,Whitty GA,Williams B,Webb RJ,Denhardt DT,Bertoncello I,Bendall LJ,Simmons PJ,Haylock DN

更新日期：2005-08-15 00:00:00

abstract：:Peripheral T-cell lymphomas (PTCLs) are aggressive tumors in which the current therapy based on multiagent chemotherapy is not successful. Since cytochrome P450 3A subfamily (CYP3A) enzymes are involved in the inactivation of chemotherapy drugs, we hypothesized that CYP3A and P-glycoprotein (MDR1) expression in these ...

journal_title：Blood

authors： Rodríguez-Antona C,Leskelä S,Zajac M,Cuadros M,Alvés J,Moneo MV,Martín C,Cigudosa JC,Carnero A,Robledo M,Benitez J,Martínez-Delgado B

更新日期：2007-11-01 00:00:00

abstract：:The prognostic implications of t(9;22)(q34;q11) were assessed at a median follow-up of 3.5 years in 434 children receiving intensive treatment for acute lymphoblastic leukemia (ALL). Four-year event-free and overall survivals were 81% and 88%, respectively, in 419 children lacking t(9;22), but were 0% and 20%, respect...

journal_title：Blood

authors： Fletcher JA,Lynch EA,Kimball VM,Donnelly M,Tantravahi R,Sallan SE

更新日期：1991-02-01 00:00:00

abstract：:Mice lacking all 3 Vav proteins fail to produce significant numbers of recirculating follicular or marginal zone B cells. Those B cells that do mature have shortened lifespans. The constitutive nuclear factor-kappaB (NF-kappaB) activity of resting naive B cells required Vav function and expression of cellular reticulo...

journal_title：Blood

authors： Vigorito E,Gambardella L,Colucci F,McAdam S,Turner M

更新日期：2005-10-01 00:00:00

abstract：:Types A and B Niemann-Pick disease (NPD) result from the deficient activity of acid sphingomyelinase (ASM; E.C. 3.1.4.12) and the resultant lysosomal accumulation of sphingomyelin. Type A disease is a fatal, neurodegenerative disorder of infancy, whereas type B disease has no neurologic manifestations and is character...

journal_title：Blood

authors： Levran O,Desnick RJ,Schuchman EH

更新日期：1992-10-15 00:00:00

abstract：:It has been shown that histamine induces early changes on endothelial cells (EC), such as a transient expression of P-selectin and secretion and/or surface expression of early mediators (eg, prostacyclin [PG1(2)], platelet-activating factor [PAF], and leukotriene B4 [LTB4]). However, delayed effects of histamine on EC...

journal_title：Blood

authors： Jeannin P,Delneste Y,Gosset P,Molet S,Lassalle P,Hamid Q,Tsicopoulos A,Tonnel AB

更新日期：1994-10-01 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：:A global phase 3 study evaluated the pharmacokinetics, efficacy, and safety of recombinant fusion protein linking coagulation factor IX with albumin (rIX-FP) in 63 previously treated male patients (12-61 years) with severe hemophilia B (factor IX [FIX] activity ≤2%). The study included 2 groups: group 1 patients recei...

journal_title：Blood

authors： Santagostino E,Martinowitz U,Lissitchkov T,Pan-Petesch B,Hanabusa H,Oldenburg J,Boggio L,Negrier C,Pabinger I,von Depka Prondzinski M,Altisent C,Castaman G,Yamamoto K,Álvarez-Roman MT,Voigt C,Blackman N,Jacobs I,PROLONG

更新日期：2016-04-07 00:00:00

abstract：:The human erythrocyte blood group system Cromer consists of high-incidence and low-incidence antigens that reside on decay-accelerating factor (DAF; CD55), a glycosyl-phosphatidylinositol-anchored membrane protein that regulates complement activation on cell surfaces. In the Cromer phenotypes Dr(a-) and Inab there is ...

journal_title：Blood

authors： Lublin DM,Mallinson G,Poole J,Reid ME,Thompson ES,Ferdman BR,Telen MJ,Anstee DJ,Tanner MJ

更新日期：1994-08-15 00:00:00

abstract：:Wiskott-Aldrich syndrome (WAS) is an X-linked disease caused by mutations in the WAS gene, leading to thrombocytopenia, eczema, recurrent infections, autoimmune disease, and malignancy. Hematopoietic cell transplantation (HCT) is the primary curative approach, with the goal of correcting the underlying immunodeficienc...

journal_title：Blood

authors： Burroughs LM,Petrovic A,Brazauskas R,Liu X,Griffith LM,Ochs HD,Bleesing JJ,Edwards S,Dvorak CC,Chaudhury S,Prockop SE,Quinones R,Goldman FD,Quigg TC,Chandrakasan S,Smith AR,Parikh S,Dávila Saldaña BJ,Thakar MS,Phela

更新日期：2020-06-04 00:00:00

abstract：:WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is an immune deficiency linked in many cases to heterozygous mutations causing truncations in the cytoplasmic tail of CXC chemokine receptor 4 (CXCR4). Leukocytes expressing truncated CXCR4 display enhanced responses to the receptor ligand CXC...

journal_title：Blood

authors： Lagane B,Chow KY,Balabanian K,Levoye A,Harriague J,Planchenault T,Baleux F,Gunera-Saad N,Arenzana-Seisdedos F,Bachelerie F

更新日期：2008-07-01 00:00:00

abstract：:The authors have identified a 12-residue carboxyl-terminal extension of Lys-Ser-Pro-Met-Arg-Arg-Phe-Leu-Leu-Phe-Cys-Met in a dysfibrinogen derived from a woman heterozygotic for this abnormality and associated with severe bleeding. This extension is due to a T-to-A mutation that creates AAG encoding Lys at the stop (T...

journal_title：Blood

authors： Sugo T,Nakamikawa C,Yoshida N,Niwa K,Sameshima M,Mimuro J,Weisel JW,Nagita A,Matsuda M

更新日期：2000-12-01 00:00:00

abstract：:Enhanced tolerance to combination chemotherapy has been cited as an ancillary benefit of staging laparotomy and splenectomy in Hodgkin's disease. Seventeen patients with Hodgkin's disease and 15 with non-Hodgkin's lymphoma were subjected to nontherapeutic splenectomy as part of the staging procedure prior to their ini...

journal_title：Blood

authors： Ihde DC,DeVita VT,Canellos GP,Young RC

更新日期：1976-02-01 00:00:00

abstract：:Erythropoietin (Epo) binding to the Epo receptor (EpoR) elicits downstream signaling that is essential for red blood cell production. One important negative regulatory mechanism to terminate Epo signaling is Epo-induced EpoR endocytosis and degradation. Defects in this mechanism play a key role in the overproduction o...

journal_title：Blood

authors： Bulut GB,Sulahian R,Yao H,Huang LJ

更新日期：2013-12-05 00:00:00

abstract：:The median age of chronic myeloid leukemia (CML) patients is ~60 years, and age is still considered an important prognostic factor, included in Sokal and EURO risk scores. However, few data are available about the long-term outcome of older patients treated with imatinib (IM) frontline. We analyzed the relationship be...

journal_title：Blood

authors： Gugliotta G,Castagnetti F,Palandri F,Breccia M,Intermesoli T,Capucci A,Martino B,Pregno P,Rupoli S,Ferrero D,Gherlinzoni F,Montefusco E,Bocchia M,Tiribelli M,Pierri I,Grifoni F,Marzocchi G,Amabile M,Testoni N,Martin

更新日期：2011-05-26 00:00:00

abstract：:A critical role for the endothelium of yolk sac and dorsal aorta has been shown in embryonic hematopoiesis. A stromal cell line derived from yolk sac, YSCL-72, has been chosen to search for a novel molecule associated with embryonic hematopoiesis. Analysis between YSCL-72 and an adult aorta-derived endothelial cell li...

journal_title：Blood

authors： Ohneda O,Ohneda K,Arai F,Lee J,Miyamoto T,Fukushima Y,Dowbenko D,Lasky LA,Suda T

更新日期：2001-10-01 00:00:00

abstract：:The pathophysiology of iron-induced compromised fertility in women with thalassemia major (TM) was evaluated in 26 adult TM females. Low gonadotropin secretion resulted in reduced ovarian antral follicle count and ovarian volume, but levels of anti-müllerian hormone (AMH), a sensitive marker for ovarian reserve indepe...

journal_title：Blood

authors： Singer ST,Vichinsky EP,Gildengorin G,van Disseldorp J,Rosen M,Cedars MI

更新日期：2011-09-08 00:00:00

abstract：:Ectopic expression of mutated K-ras or N-ras in the interleukin 6 (IL-6)-dependent ANBL6 multiple myeloma cell line induces cytokine-independent growth. To investigate the signaling pathways activated by oncogenic ras that may stimulate IL-6-independent growth, we compared ANBL6 cells stably transfected with mutated K...

journal_title：Blood

authors： Hu L,Shi Y,Hsu JH,Gera J,Van Ness B,Lichtenstein A

更新日期：2003-04-15 00:00:00

abstract：:Myeloid-derived suppressor cells (MDSCs) represent a heterogeneous population that shares certain characteristics including an aberrant myeloid phenotype and the ability to suppress T cells. MDSCs have been predominantly studied in malignant diseases and findings suggest involvement in tumor-associated immune suppress...

journal_title：Blood

authors： Jitschin R,Braun M,Büttner M,Dettmer-Wilde K,Bricks J,Berger J,Eckart MJ,Krause SW,Oefner PJ,Le Blanc K,Mackensen A,Mougiakakos D

更新日期：2014-07-31 00:00:00

abstract：:Transcriptional profiles of cultured primary human erythroid cells were examined to identify those genes involved in the control of erythroid growth during the terminal phase of maturation. Our in silico screening strategy indicated that a hypoxia-inducible proapoptotic member of the Bcl-2 gene family called Nix is ex...

journal_title：Blood

authors： Aerbajinai W,Giattina M,Lee YT,Raffeld M,Miller JL

更新日期：2003-07-15 00:00:00

abstract：:Regulated adherence of polymorphonuclear leukocytes (PMNs) to endothelium and subendothelial matrix is a critical event for PMN localization at and migration into inflammatory sites. We previously reported that human PMNs stimulated in vitro adhere to laminin, the major glycoprotein of mammalian basement membrane, by ...

journal_title：Blood

authors： Bohnsack JF

更新日期：1992-03-15 00:00:00

abstract：:Evidence supporting the efficacy of in utero hematopoietic cell transplantation (IUHCT) in a valid large animal model is needed prior to clinical application. The objective of this study was to establish clinically relevant levels of hematopoietic chimerism in a canine model of maternal-to-fetal IUHCT. We first assess...

journal_title：Blood

authors： Vrecenak JD,Pearson EG,Santore MT,Todorow CA,Li H,Radu A,Bhatti T,Peranteau WH,Johnson MP,Flake AW

更新日期：2014-09-18 00:00:00