Arg2074Cys missense mutation in the C2 domain of factor V causing moderately severe factor V deficiency: molecular characterization by expression of the recombinant protein.

abstract：:Venous limb gangrene (VLG) can occur in cancer patients, but the clinical picture and pathogenesis remain uncertain. We identified 10 patients with metastatic cancer (7 pathologically proven) who developed severe venous limb ischemia (phlegmasia/VLG) after initiating treatment of deep-vein thrombosis (DVT); in 8 patie...

journal_title：Blood

authors： Warkentin TE,Cook RJ,Sarode R,Sloane DA,Crowther MA

更新日期：2015-07-23 00:00:00

abstract：:The terminal differentiation of B cells into antibody-secreting cells (ASCs) is a critical component of adaptive immune responses. However, it is a very sensitive process, which dysfunctions lead to a great variety of lymphoproliferative neoplasia including germinal center-derived lymphomas. To better characterize the...

journal_title：Blood

authors： Pignarre A,Chatonnet F,Caron G,Haas M,Desmots-Loyer F,Fest T

更新日期：2020-11-04 00:00:00

abstract：:Allogeneic hematopoietic cell transplantation in multiple myeloma is limited by prior reports of high treatment-related mortality. We analyzed outcomes after allogeneic hematopoietic cell transplantation for multiple myeloma in 1207 recipients in 3 cohorts based on the year of transplantation: 1989-1994 (n = 343), 199...

journal_title：Blood

authors： Kumar S,Zhang MJ,Li P,Dispenzieri A,Milone GA,Lonial S,Krishnan A,Maiolino A,Wirk B,Weiss B,Freytes CO,Vogl DT,Vesole DH,Lazarus HM,Meehan KR,Hamadani M,Lill M,Callander NS,Majhail NS,Wiernik PH,Nath R,Kamble RT

更新日期：2011-08-18 00:00:00

abstract：:Gene therapy for inherited disorders of blood cells will require both efficient methods for stable gene transfer and nonablative bone marrow conditioning regimens to allow engraftment of modified hematopoietic progenitor cells (HPCs). We have used a sensitive murine system for detecting HPC engraftment using congenic ...

journal_title：Blood

authors： Mardiney M 3rd,Malech HL

更新日期：1996-05-15 00:00:00

abstract：:The pathophysiology of microthrombocytopenia in the Wiskott-Aldrich syndrome (WAS) and its milder form, X-linked thrombocytopenia (XLT), is unclear. Although quantitative defects are correctable by splenectomy, residual platelet abnormalities are suggestive of intrinsic disturbances of production. In contrast to human...

journal_title：Blood

authors： Sabri S,Foudi A,Boukour S,Franc B,Charrier S,Jandrot-Perrus M,Farndale RW,Jalil A,Blundell MP,Cramer EM,Louache F,Debili N,Thrasher AJ,Vainchenker W

更新日期：2006-07-01 00:00:00

abstract：:Complement plays a key role in host defense, but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins, including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic...

journal_title：Blood

authors： Ueda Y,Mohammed I,Song D,Gullipalli D,Zhou L,Sato S,Wang Y,Gupta S,Cheng Z,Wang H,Bao J,Mao Y,Brass L,Zheng XL,Miwa T,Palmer M,Dunaief J,Song WC

更新日期：2017-03-02 00:00:00

abstract：:Successful immune reconstitution (IR) is associated with improved outcomes following pediatric cord blood transplantation (CBT). Usage and timing of anti-thymocyte globulin (ATG), introduced to the conditioning to prevent graft-versus-host disease and graft failure, negatively influences T-cell IR. We studied the rela...

journal_title：Blood

authors： Admiraal R,Lindemans CA,van Kesteren C,Bierings MB,Versluijs AB,Nierkens S,Boelens JJ

更新日期：2016-12-08 00:00:00

abstract：:The mechanism leading to the formation of antiphospholipid antibodies (aPL) is still unknown. Because an in vitro study suggested that aPL may derive from pro-oxidant conditions, we sought a relationship between aPL and isoprostanes, indices of lipid peroxidation in vivo. Thirty patients with systemic lupus erythemato...

journal_title：Blood

authors： Iuliano L,Praticò D,Ferro D,Pittoni V,Valesini G,Lawson J,FitzGerald GA,Violi F

更新日期：1997-11-15 00:00:00

abstract：:Leukocyte rolling precedes firm adhesion and emigration in inflammatory cell recruitment. Both P-selectin, an endothelial lectin that binds to sialylated O-glycans containing sialyl-Lewisx (sLex) on the granulocyte surface, and leukocyte L-selectin have been shown to mediate leukocyte rolling in vivo. Here, we investi...

journal_title：Blood

authors： Ley K,Zakrzewicz A,Hanski C,Stoolman LM,Kansas GS

更新日期：1995-06-15 00:00:00

abstract：:Peripheral blood and bone marrow mononuclear cells from patients with refractory anemia (RA) or RA with sideroblasts (defined according to the revised French-American-British classification with less than 5% blast cells in the bone marrow) were analyzed using a panel of monoclonal antibodies directed against leukocyte...

journal_title：Blood

authors： Hokland P,Kerndrup G,Griffin JD,Ellegaard J

更新日期：1986-04-01 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：:Interleukin-2 (IL-2) and IL-15 exert similar biological actions, which largely reflect the fact that their receptors share common beta and gamma subunits; in contrast, distinct subunits are required for high-affinity binding of either cytokine to a heterotrimeric receptor complex. Human neutrophils are known to expres...

journal_title：Blood

authors： McDonald PP,Russo MP,Ferrini S,Cassatella MA

更新日期：1998-12-15 00:00:00

abstract：:Shear-induced platelet aggregation (SIPA) involves the sequential interaction of von Willebrand factor (VWF) with both glycoprotein Ib (GPIb) and alphaIIbbeta3 receptors. Type 2B recombinant VWF (2B-rVWF), characterized by an increased affinity for GPIb, induces strong SIPA at a high shear rate (4000 s-1). Despite the...

journal_title：Blood

authors： Mekrache M,Bachelot-Loza C,Ajzenberg N,Saci A,Legendre P,Baruch D

更新日期：2003-06-01 00:00:00

abstract：:The influence of intestinal bacterial decontamination on the occurrence of grades II to IV acute graft-versus-host disease (GVHD) was retrospectively analyzed in 194 predominantly adult patients treated by genotypically identical sibling marrow transplantation under conditions of strict protective isolation and intest...

journal_title：Blood

authors： Beelen DW,Haralambie E,Brandt H,Linzenmeier G,Müller KD,Quabeck K,Sayer HG,Graeven U,Mahmoud HK,Schaefer UW

更新日期：1992-11-15 00:00:00

abstract：:The proto-oncogene c-fos was transiently upregulated in primitive hematopoietic stem (Lin-Sca-1(+)) cells stimulated with stem cell factor, interleukin-3 (IL-3), and IL-6. To investigate a role of the c-fos in hematopoietic stem cells, we used bone marrow (BM) cells from transgenic mice carrying the c-fos gene under t...

journal_title：Blood

authors： Okada S,Fukuda T,Inada K,Tokuhisa T

更新日期：1999-02-01 00:00:00

abstract：:Techniques for the quantitation of virus-specific and alloantigen-reactive T cells vary in their measurement of clinically relevant T-cell effector populations, their sensitivity and quantitative accuracy, and the time required to obtain measurable results. We compared frequencies of Epstein-Barr virus (EBV)-specific ...

journal_title：Blood

authors： Koehne G,Smith KM,Ferguson TL,Williams RY,Heller G,Pamer EG,Dupont B,O'Reilly RJ

更新日期：2002-03-01 00:00:00

abstract：:Tumors have several mechanisms to escape from the immune system. One of these involves expression of intracellular anticytotoxic proteins that modulate the execution of cell death. Previously, we have shown that the serine protease inhibitor (serpin) SPI-6, which inactivates the cytotoxic protease granzyme B (GrB), is...

journal_title：Blood

authors： Bots M,Kolfschoten IG,Bres SA,Rademaker MT,de Roo GM,Krüse M,Franken KL,Hahne M,Froelich CJ,Melief CJ,Offringa R,Medema JP

更新日期：2005-02-01 00:00:00

abstract：:Myelodysplastic syndromes (MDSs) are hematopoietic stem cell disorders in which recurrent mutations define clonal hematopoiesis. The origin of the phenotypic diversity of non-del(5q) MDS remains unclear. Here, we investigated the clonal architecture of the CD34+CD38- hematopoietic stem/progenitor cell (HSPC) compartme...

journal_title：Blood

authors： Chesnais V,Arcangeli ML,Delette C,Rousseau A,Guermouche H,Lefevre C,Bondu S,Diop M,Cheok M,Chapuis N,Legros L,Raynaud S,Willems L,Bouscary D,Lauret E,Bernard OA,Kosmider O,Pflumio F,Fontenay M

更新日期：2017-01-26 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：:Clinical and cytogenetic findings in 10 patients with deletions of the long arm of chromosomes 5 (5q-) are reported. Five cases had refractory anemia, the preleukemic syndrome, or refractory anemia with an excess of blasts; in all but one, the 5q- was the single initial abnormality. Three patients had overt leukemia; ...

journal_title：Blood

authors： Swolin B,Weinfeld A,Ridell B,Waldenström J,Westin J

更新日期：1981-11-01 00:00:00

abstract：:Rh(o)(D) immune globulin intravenous (anti-D IGIV) was licensed by the United States Food and Drug Administration (FDA) in March 1995 to treat patients with immune thrombocytopenic purpura (ITP). Anti-D IGIV induces extravascular hemolysis, an expected adverse reaction that is consistent with the presumed mechanism of...

journal_title：Blood

authors： Gaines AR

更新日期：2000-04-15 00:00:00

abstract：:Important predictors of adverse outcomes of thrombosis in children, including postthrombotic syndrome (PTS), have recently been identified. Given this knowledge and the encouraging preliminary pediatric experience with systemic thrombolysis, we sought to retrospectively analyze our institutional experience with a thro...

journal_title：Blood

authors： Goldenberg NA,Durham JD,Knapp-Clevenger R,Manco-Johnson MJ

更新日期：2007-07-01 00:00:00

abstract：:High efficiency retroviral-mediated gene transfer to rhesus CD4+ peripheral blood lymphocytes (PBL) was accomplished using an optimized transduction protocol using a gibbon ape leukemia virus (GaLV) envelope-containing packaging cell line PG13. Engineered CD4+ PBL were administered to three nonmyeloablated animals in ...

journal_title：Blood

authors： Bunnell BA,Metzger M,Byrne E,Morgan RA,Donahue RE

更新日期：1997-03-15 00:00:00

abstract：:Sepsis and septic shock remain an important medical problem, emphasizing the need to identify novel therapeutic opportunities. Hypovolemic hypotension, coagulation dysfunction, disturbed microcirculation, and multiorgan failure resulting from vascular leakage are often observed in these severe conditions. In the prese...

journal_title：Blood

authors： Oehmcke S,Shannon O,von Köckritz-Blickwede M,Mörgelin M,Linder A,Olin AI,Björck L,Herwald H

更新日期：2009-07-09 00:00:00

abstract：:In the present study, we investigated disease characteristics and clinical outcome in young patients (< 40 years) with World Health Organization (WHO)-defined essential thrombocythemia (ET) compared with early/prefibrotic primary myelofibrosis (PMF) with presenting thrombocythemia. We recruited 213 young patients (med...

journal_title：Blood

authors： Barbui T,Thiele J,Carobbio A,Passamonti F,Rumi E,Randi ML,Bertozzi I,Vannucchi AM,Gisslinger H,Gisslinger B,Finazzi G,Ruggeri M,Rodeghiero F,Rambaldi A,Gangat N,Tefferi A

更新日期：2012-07-19 00:00:00

abstract：:IL-17 and its receptor are founding members of a novel family of inflammatory cytokines. IL-17 plays a pathogenic role in rheumatoid arthritis (RA)-associated bone destruction. However, IL-17 is also an important regulator of host defense through granulopoiesis and neutrophil trafficking. Therefore, the role of IL-17 ...

journal_title：Blood

authors： Yu JJ,Ruddy MJ,Wong GC,Sfintescu C,Baker PJ,Smith JB,Evans RT,Gaffen SL

更新日期：2007-05-01 00:00:00

abstract：:Chronic granulomatous disease (CGD) is characterized by the absence of a respiratory burst in activated phagocytes. Defects in at least four different genes lead to CGD. Patients with the X-linked form of CGD have mutations in the gene for the beta-subunit of cytochrome b558 (gp91-phox). We studied the molecular defec...

journal_title：Blood

authors： de Boer M,Bolscher BG,Dinauer MC,Orkin SH,Smith CI,Ahlin A,Weening RS,Roos D

更新日期：1992-09-15 00:00:00

abstract：:There is growing evidence that lymphocyte trafficking contributes to the clinical course of chronic lymphocytic leukemia (CLL), but to date, only static in vitro cultures have been used to study these phenomena. To address this lack of data, we have developed a dynamic in vitro model in which CLL cells experience shea...

journal_title：Blood

authors： Walsby E,Buggins A,Devereux S,Jones C,Pratt G,Brennan P,Fegan C,Pepper C

更新日期：2014-06-05 00:00:00

abstract：:We designed a phase 1-2 study to evaluate the safety and the efficacy of increasing doses of bendamustine (160 mg/m², 180 mg/m², and 200 mg/m² given on days -7 and -6) coupled with fixed doses of etoposide, cytarabine, and melphalan (BeEAM regimen) as the conditioning regimen to autologous stem cell transplantation fo...

journal_title：Blood

authors： Visani G,Malerba L,Stefani PM,Capria S,Galieni P,Gaudio F,Specchia G,Meloni G,Gherlinzoni F,Giardini C,Falcioni S,Cuberli F,Gobbi M,Sarina B,Santoro A,Ferrara F,Rocchi M,Ocio EM,Caballero MD,Isidori A

更新日期：2011-09-22 00:00:00

abstract：:Platelet factor 4 (PF4) is an abundant platelet alpha-granule C-X-C chemokine that has weak chemotactic potency but strongly inhibits hematopoiesis through an unknown mechanism. We find that PF4 binds to human CD34+ hematopoietic progenitor cells (HPCs) with a median effective concentration of 1 microg/mL but not afte...

journal_title：Blood

authors： Dudek AZ,Nesmelova I,Mayo K,Verfaillie CM,Pitchford S,Slungaard A

更新日期：2003-06-15 00:00:00