Defective targeting of hemojuvelin to plasma membrane is a common pathogenetic mechanism in juvenile hemochromatosis.

abstract：:Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with defective complement regulation caused by multiple factors. We previously described the deficiency of factor H-related proteins CFHR1 and CFHR3 as predisposing factor for aHUS. Here we identify in an extended cohort of 147 aHUS...

journal_title：Blood

authors： Józsi M,Licht C,Strobel S,Zipfel SL,Richter H,Heinen S,Zipfel PF,Skerka C

更新日期：2008-02-01 00:00:00

abstract：:The replating capability of human multipotential (colony-forming unit-granulocyte-erythrocyte-macrophage-megakaryocyte [CFU-GEMM]) and erythroid (burst-forming unit-erythroid [BFU-E]) progenitors was assessed in vitro as a potential measure of self-renewal using purified, recombinant (r) human (hu) or murine (mu) mast...

journal_title：Blood

authors： Carow CE,Hangoc G,Cooper SH,Williams DE,Broxmeyer HE

更新日期：1991-11-01 00:00:00

abstract：:Combinations of proinflammatory and procoagulant reactions are the unifying principle for a variety of disorders affecting the cardiovascular system. The factor XII-driven contact system starts coagulation and inflammatory mechanisms via the intrinsic pathway of coagulation and the bradykinin-producing kallikrein-kini...

journal_title：Blood

authors： Maas C,Renné T

更新日期：2018-04-26 00:00:00

abstract：:At the intersection between children and older adults, the care of adolescent and young adult (AYA) patients with acute lymphoblastic leukemia (ALL) poses unique challenges and issues beyond those faced by other age groups. Although the survival of AYA patients is inferior to younger children, growing evidence suggest...

journal_title：Blood

authors： Curran E,Stock W

更新日期：2015-06-11 00:00:00

abstract：:Current knowledge is inadequate to explain the different patterns of blast cell accumulation in myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML). We compared the growth patterns of blast cell progenitors (CFU-L) in 23 patients with advanced MDS and 32 patients with de novo AML. Circulating blast progen...

journal_title：Blood

authors： Aul C,Gattermann N,Schneider W

更新日期：1992-08-01 00:00:00

abstract：:Disseminated intravascular coagulation (DIC) is a condition characterized by systemic activation of coagulation, potentially leading to thrombotic obstruction of small and midsize vessels, thereby contributing to organ dysfunction. At the same time, ongoing consumption of platelets and coagulation proteins results in ...

journal_title：Blood

authors： Levi M,Scully M

更新日期：2018-02-22 00:00:00

abstract：:Recent phenotypic analysis of plasma cells showed that normal plasma cells do express the B-cell lineage-specific molecule CD19, but their malignant counterpart (myeloma cells) are CD19-. To clarify the meaning of loss of CD19 antigen on myeloma cells, we first compared the expression of CD19 and Pax-5 genes among B c...

journal_title：Blood

authors： Mahmoud MS,Huang N,Nobuyoshi M,Lisukov IA,Tanaka H,Kawano MM

更新日期：1996-05-15 00:00:00

abstract：:Analysis of fresh human tumors have indicated that patients with B type lymphoproliferative diseases and the majority of patients with acute lymphoblastic leukemia (ALL) express elevated levels of p53 production. It is suggested that in these human malignancies, p53 may provide a novel tool for monitoring cancer activ...

journal_title：Blood

authors： Prokocimer M,Shaklai M,Bassat HB,Wolf D,Goldfinger N,Rotter V

更新日期：1986-07-01 00:00:00

abstract：:Two families of Epstein-Barr virus (EBV), type A and type B, have been defined on the basis of sequence divergence in the EBNA-2 gene. Type A EBV immortalizes B cells more efficiently in vitro and infects immunocompetent individuals more commonly than type B EBV. However, increased rates of infection by type B EBV are...

journal_title：Blood

authors： Frank D,Cesarman E,Liu YF,Michler RE,Knowles DM

更新日期：1995-03-01 00:00:00

abstract：:Natural killer (NK) cells can alter the outcome of hematopoietic cell transplantation (HCT) if donor alloreactivity targets the recipient. Since most NK cells express inhibitory killer-immunoglobulin receptors (KIRs), we hypothesized that the susceptibility of recipient cells to donor NK cell-mediated lysis is genetic...

journal_title：Blood

authors： Miller JS,Cooley S,Parham P,Farag SS,Verneris MR,McQueen KL,Guethlein LA,Trachtenberg EA,Haagenson M,Horowitz MM,Klein JP,Weisdorf DJ

更新日期：2007-06-01 00:00:00

abstract：:We examined the role of the T-cell antigen CD2 in the regulation of erythropoiesis by the lymphokine cascade. T-cell interleukin-2 (IL-2) receptors (p55) were induced via triggering of the antigen receptor-associated CD3 epitope. Before CD3 triggering T cells were preincubated with a CD2-blocking (Leu-5b) or isotype c...

journal_title：Blood

authors： Burdach S,Shatsky M,Wagenhorst B,Levitt L

更新日期：1988-08-01 00:00:00

abstract：:Sequencing the complete factor IX gene of 2 sisters with hemophilia B with different phenotypes and no family history of hemorrhagic diathesis revealed a common 5' splice site mutation in intron 3 (T6704C) in both and an additional missense mutation (I344T) in one. The presence of dysfunctional antigen in the latter s...

journal_title：Blood

authors： Costa JM,Vidaud D,Laurendeau I,Vidaud M,Fressinaud E,Moisan JP,David A,Meyer D,Lavergne JM

更新日期：2000-08-15 00:00:00

abstract：:Adhesive interactions between CD34+ hematopoietic progenitor cells (HPC) and bone marrow stroma are crucial for normal hematopoiesis, yet their molecular bases are still poorly elucidated. We have investigated whether cell surface proteoglycan CD44 can mediate adhesion of human CD34+ HPC to immobilized hyaluronan (HA)...

journal_title：Blood

authors： Legras S,Levesque JP,Charrad R,Morimoto K,Le Bousse C,Clay D,Jasmin C,Smadja-Joffe F

更新日期：1997-03-15 00:00:00

abstract：:The hereditary stomatocytoses are a series of dominantly inherited hemolytic anemias in which the permeability of the erythrocyte membrane to monovalent cations is pathologically increased. The causative mutations for some forms of hereditary stomatocytosis have been found in the transporter protein genes, RHAG and SL...

journal_title：Blood

authors： Flatt JF,Guizouarn H,Burton NM,Borgese F,Tomlinson RJ,Forsyth RJ,Baldwin SA,Levinson BE,Quittet P,Aguilar-Martinez P,Delaunay J,Stewart GW,Bruce LJ

更新日期：2011-11-10 00:00:00

abstract：:Chronic myelogenous leukemia (CML) is a malignant disease of the hematopoietic stem cell characterized by abnormal circulation and proliferation of malignant progenitors. In contrast to their normal counterparts, CML progenitors adhere poorly to bone marrow stroma or fibronectin (FN). Aside from anchoring progenitors ...

journal_title：Blood

authors： Lundell BI,McCarthy JB,Kovach NL,Verfaillie CM

更新日期：1996-03-15 00:00:00

abstract：:When overexpressed in primary erythroid progenitors, oncogenic Ras leads to the constitutive activation of its downstream signaling pathways, severe block of terminal erythroid differentiation, and cytokine-independent growth of primary erythroid progenitors. However, whether high-level expression of oncogenic Ras is ...

journal_title：Blood

authors： Zhang J,Liu Y,Beard C,Tuveson DA,Jaenisch R,Jacks TE,Lodish HF

更新日期：2007-06-15 00:00:00

abstract：:CD22 is an important immunotherapeutic target on B-cell malignancies, particularly hairy cell leukemia (HCL), but its soluble extracellular domain, sCD22, has not yet been reported in the blood. By immunoaffinity and enzyme-linked immunosorbent assay techniques using anti-CD22 monoclonal antibodies, we identified the ...

journal_title：Blood

authors： Matsushita K,Margulies I,Onda M,Nagata S,Stetler-Stevenson M,Kreitman RJ

更新日期：2008-09-15 00:00:00

abstract：:Mutations in the MYH9 gene encoding the nonmuscle myosin heavy chain IIA result in bleeding disorders characterized by a macrothrombocytopenia. To understand the role of myosin in normal platelet functions and in pathology, we generated mice with disruption of MYH9 in megakaryocytes. MYH9Delta mice displayed macrothro...

journal_title：Blood

authors： Léon C,Eckly A,Hechler B,Aleil B,Freund M,Ravanat C,Jourdain M,Nonne C,Weber J,Tiedt R,Gratacap MP,Severin S,Cazenave JP,Lanza F,Skoda R,Gachet C

更新日期：2007-11-01 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：:Macrophage colony stimulating factor (CSF-1) is one of several cytokines that control the differentiation, survival, and proliferation of monocytes and macrophages. A set of 11 human T-cell clones, chosen for their phenotypic diversity, were tested for their ability to express CSF-1 mRNA. After 5 hours of stimulation ...

journal_title：Blood

authors： Hallet MM,Praloran V,Vié H,Peyrat MA,Wong G,Witek-Giannotti J,Soulillou JP,Moreau JF

更新日期：1991-02-15 00:00:00

abstract：:Expression of inhibitory killer cell immunoglobulin-like receptors (KIRs) specific for self-major histocompatibility complex (MHC) class I molecules provides an educational signal that generates functional natural killer (NK) cells. However, the effects of activating KIRs specific for self-MHC class I on NK-cell educa...

journal_title：Blood

authors： Fauriat C,Ivarsson MA,Ljunggren HG,Malmberg KJ,Michaëlsson J

更新日期：2010-02-11 00:00:00

abstract：:Human subjects maintain long-term immunologic memory against infective organisms but the mechanism is unclear. CD4+ T-helper memory (Thmem) cells are pivotal in controlling humoral and cellular responses, therefore their longevity and response to vaccination are critical for maintenance of protective immunity. To prob...

journal_title：Blood

authors： Di Genova G,Roddick J,McNicholl F,Stevenson FK

更新日期：2006-04-01 00:00:00

abstract：:CXCR4 chemokine receptors retain hematopoietic progenitors and leukemia cells within the marrow microenvironment. We prospectively evaluated the prognostic implication of CXCR4 in 90 consecutive patients with acute myelogenous leukemia (AML) by flow cytometry. Patients were divided into groups with low (n=32), interme...

journal_title：Blood

authors： Spoo AC,Lübbert M,Wierda WG,Burger JA

更新日期：2007-01-15 00:00:00

abstract：:Expression patterns of interleukin-6 (IL-6), IL-6 receptor (IL-6R), and gp130 genes in 39 patients with acute myeloid leukemia (AML), in 23 patients with acute lymphoblastic leukemia (ALL), and in 7 patients with acute mixed lineage leukemia (AMLL) were studied by quantitative reverse transcriptase-polymerase chain re...

journal_title：Blood

authors： Inoue K,Sugiyama H,Ogawa H,Yamagami T,Azuma T,Oka Y,Miwa H,Kita K,Hiraoka A,Masaoka T

更新日期：1994-10-15 00:00:00

abstract：:Eighteen patients with agnogenic myeloid metaplasia with myelofibrosis were studied for clinical and laboratory evidence of immunologic dysfunction. Clinical findings included the presence of arthritis, vasculitis, and erythema nodosum. Laboratory abnormalities included the presence of circulating immune complexes, an...

journal_title：Blood

authors： Gordon BR,Coleman M,Kohen P,Day NK

更新日期：1981-11-01 00:00:00

abstract：:During this time of transition in the federal government and the National Institutes of Health, I write to assure the Blood community of the National Heart, Lung, and Blood Institute's (NHLBI) commitment to new and established investigators as outlined in the NHLBI Strategic Plan. This perspective discusses the NHLBI ...

journal_title：Blood

authors： Nabel EG

更新日期：2009-03-26 00:00:00

abstract：:Although thalidomide (Thal) was initially used to treat multiple myeloma (MM) because of its known antiangiogenic effects, the mechanism of its anti-MM activity is unclear. These studies demonstrate clinical activity of Thal against MM that is refractory to conventional therapy and delineate mechanisms of anti-tumor a...

journal_title：Blood

authors： Hideshima T,Chauhan D,Shima Y,Raje N,Davies FE,Tai YT,Treon SP,Lin B,Schlossman RL,Richardson P,Muller G,Stirling DI,Anderson KC

更新日期：2000-11-01 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：:GATA family proteins play essential roles in development of many cell types, including hematopoietic, cardiac, and endodermal lineages. The first three factors, GATAs 1, 2, and 3, are essential for normal hematopoiesis, and their mutations are responsible for a variety of blood disorders. Acquired and inherited GATA1 ...

journal_title：Blood

authors： Crispino JD,Horwitz MS

更新日期：2017-04-13 00:00:00

abstract：:Investigators in the United Kingdom have shown that hereditary amyloidosis can be misdiagnosed as Ig light-chain (AL) amyloidosis because family history is an ineffective screen, and tissue staining used to type amyloid is unreliable. Misdiagnosis of AL can lead to inappropriate use of chemotherapy and failure to diag...

journal_title：Blood

authors： Comenzo RL,Zhou P,Fleisher M,Clark B,Teruya-Feldstein J

更新日期：2006-05-01 00:00:00