Defective adhesion of blood platelets to vascular microfibrils in the Bernard-Soulier syndrome.

abstract：:Most patients with chronic lymphocytic leukemia (CLL) are diagnosed with early-stage disease and managed with active surveillance. The individual course of patients with early-stage CLL is heterogeneous, and their probability of needing treatment is hardly anticipated at diagnosis. We aimed at developing an internatio...

journal_title：Blood

authors： Condoluci A,Terzi di Bergamo L,Langerbeins P,Hoechstetter MA,Herling CD,De Paoli L,Delgado J,Rabe KG,Gentile M,Doubek M,Mauro FR,Chiodin G,Mattsson M,Bahlo J,Cutrona G,Kotaskova J,Deambrogi C,Smedby KE,Spina V,Brusc

更新日期：2020-05-21 00:00:00

abstract：:Fas (CD95/Apo-1) mutations were previously reported as the genetic defect responsible for human lymphoproliferative syndrome associated with autoimmune manifestations (also known as autoimmune lymphoproliferative syndrome or Canale-Smith syndrome). We have identified 14 new heterozygous Fas mutations. Analysis of pati...

journal_title：Blood

authors： Rieux-Laucat F,Blachère S,Danielan S,De Villartay JP,Oleastro M,Solary E,Bader-Meunier B,Arkwright P,Pondaré C,Bernaudin F,Chapel H,Nielsen S,Berrah M,Fischer A,Le Deist F

更新日期：1999-10-15 00:00:00

abstract：:Human herpesvirus 6 activity (HHV-6) was studied in 15 allogeneic and 11 autologous marrow transplantation patients. After transplantation, HHV-6 was isolated from the peripheral blood mononuclear cells of 12 of 26 patients (6 allogeneic and 6 autologous). All isolates were variant B. Eleven of 26 and 12 of 19 patient...

journal_title：Blood

authors： Kadakia MP,Rybka WB,Stewart JA,Patton JL,Stamey FR,Elsawy M,Pellett PE,Armstrong JA

更新日期：1996-06-15 00:00:00

abstract：:Recent studies have identified nonredundant roles for basophils in immune responses including allergy and protective immunity. It is well known that activated basophils release granule contents such as histamine and proteases as do mast cells. However, the functional significance of basophil-derived proteases remains ...

journal_title：Blood

authors： Iki M,Tanaka K,Deki H,Fujimaki M,Sato S,Yoshikawa S,Yamanishi Y,Karasuyama H

更新日期：2016-12-22 00:00:00

abstract：:Hand mirror cells are a morphological configuration that are seen in immunologically stimulated lymphocytes and can be induced by antigen--antibody complexes. Therefore, the bone marrow and peripheral blood plasma of two patients with acute lymphoblastic leukemia--hand mirror variant were evaluated for the presence of...

journal_title：Blood

authors： Stass SA,Phillips TM,Weislow OS,Perlin E,Schumacher HR

更新日期：1980-10-01 00:00:00

abstract：:Because of the expression of inhibitory receptors (KIR) for major histocompatibility complex (MHC) class I allotypes, a person's natural killer (NK) cells will not recognize and will, therefore, kill cells from individuals lacking his/her KIR epitopes. This study investigated the role of NK cell alloreactivity in huma...

journal_title：Blood

authors： Ruggeri L,Capanni M,Casucci M,Volpi I,Tosti A,Perruccio K,Urbani E,Negrin RS,Martelli MF,Velardi A

更新日期：1999-07-01 00:00:00

abstract：:Since their discovery in patients with autosomal dominant (AD) chronic mucocutaneous candidiasis (CMC) in 2011, heterozygous STAT1 gain-of-function (GOF) mutations have increasingly been identified worldwide. The clinical spectrum associated with them needed to be delineated. We enrolled 274 patients from 167 kindreds...

journal_title：Blood

authors： Toubiana J,Okada S,Hiller J,Oleastro M,Lagos Gomez M,Aldave Becerra JC,Ouachée-Chardin M,Fouyssac F,Girisha KM,Etzioni A,Van Montfrans J,Camcioglu Y,Kerns LA,Belohradsky B,Blanche S,Bousfiha A,Rodriguez-Gallego C,Meyts

更新日期：2016-06-23 00:00:00

abstract：:The present studies were initiated to define the coding region of a 34 kilodalton (kd) protein (p34) frequently observed with antibodies from HTLV-III/LAV-infected people by immunoblotting and radioimmunoprecipitation (RIP) techniques. We have directly mapped this viral protein to the pol gene of HTLV-III/LAV by radio...

journal_title：Blood

authors： Allan JS,Coligan JE,Lee TH,Barin F,Kanki PJ,M'Boup S,McLane MF,Groopman JE,Essex M

更新日期：1987-01-01 00:00:00

abstract：:The presence of circulating tumor idiotype interferes with the in vivo effectiveness of anti-idiotype antibodies. We developed two assays that permit identification of patients with high levels of serum idiotype without the need for first producing an anti-idiotype antibody. A cell suspension made from the tumor was c...

journal_title：Blood

authors： Miller RA,Lowder JN,Gralow J,Meeker T,Levy R

更新日期：1987-04-01 00:00:00

abstract：:Juvenile chronic myelocytic leukemia (JCML) is a rare disorder of early childhood. Characteristic of JCML are the progressive appearance of high levels of fetal hemoglobin (HbF), reflecting a true reversion to a fetal type of erythropoiesis, and the presence of colony-forming cells able to grow in vitro spontaneously ...

journal_title：Blood

authors： Privitera E,Schirò R,Longoni D,Ronchi A,Rambaldi A,Bernasconi S,Ottolenghi S,Masera G,Biondi A

更新日期：1995-07-01 00:00:00

abstract：:Increased expression levels of miR-181 family members have been shown to be associated with favorable outcome in patients with cytogenetically normal acute myeloid leukemia. Here we show that increased expression of miR-181a and miR-181b is also significantly (P < .05; Cox regression) associated with favorable overall...

journal_title：Blood

authors： Li Z,Huang H,Li Y,Jiang X,Chen P,Arnovitz S,Radmacher MD,Maharry K,Elkahloun A,Yang X,He C,He M,Zhang Z,Dohner K,Neilly MB,Price C,Lussier YA,Zhang Y,Larson RA,Le Beau MM,Caligiuri MA,Bullinger L,Valk PJ,Del

更新日期：2012-03-08 00:00:00

abstract：:Natural killer (NK) cells are large granular lymphocytes (LGLs) that contain distinct lysosomal granules. The present study was undertaken to determine if these lysosomes contain glycosaminoglycans (GAGs) similar to those previously described in myeloid cells. Mononuclear cells from human blood were stained with HNK-1...

journal_title：Blood

authors： Parmley RT,Rahemtulla F,Cooper MD,Rodén L

更新日期：1985-07-01 00:00:00

abstract：:Heme biosynthesis was examined in erythroid tissue of a 4-yr-old girl with severe sideroblastic anemia since infancy, as documented by the presence of intramitochondrial deposits of iron in erythroblasts. Free red cell protoporphyrin, urinary porphyrins, and activities of erythrocyte porphobilinogen synthase, uroporph...

journal_title：Blood

authors： Buchanan GR,Bottomley SS,Nitschke R

更新日期：1980-01-01 00:00:00

abstract：:Studies of neutrophil nicotinamide adenine dinucleotide phosphate (NADPH) oxidase activation in a cell-free system showed that the low molecular-weight guanosine triphosphatase (GTPase) Rac was required, and that Rap1a may participate in activation of the catalytic complex. Full-length posttranslationally modified Rac...

journal_title：Blood

authors： Gabig TG,Crean CD,Mantel PL,Rosli R

更新日期：1995-02-01 00:00:00

abstract：:P-selectin (CD62P) is a member of the selectin family of adhesion molecules involved in the regulation of leukocyte traffic. P-selectin glycoprotein ligand-1 (PSGL-1) is a mucin-like molecule that is thought to be a primary ligand for P-selectin. The interaction of P-selectin with PSGL-1 results in leukocyte rolling a...

journal_title：Blood

authors： Snapp KR,Wagers AJ,Craig R,Stoolman LM,Kansas GS

更新日期：1997-02-01 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：: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：:Fetal hemoglobin (Hb F) production in sickle cell (SS) disease and in normal individuals varies over a 20-fold range and is under genetic control. Previous studies suggested that variant Hb F levels might be controlled by genetic loci separate from the beta-globin complex on chromosome 11. Using microscopic radial imm...

journal_title：Blood

authors： Dover GJ,Smith KD,Chang YC,Purvis S,Mays A,Meyers DA,Sheils C,Serjeant G

更新日期：1992-08-01 00:00:00

abstract：:One hundred eight consecutive patients with indolent lymphoproliferative diseases were stratified into chronic lymphocytic leukemia (CLL), stage III and IV well-differentiated lymphocytic lymphoma (WDLL), and stage III and IV follicular lymphoma (FL). Within each stratum, patients were prospectively and randomly assig...

journal_title：Blood

authors： Jacobs P,King HS

更新日期：1987-06-01 00:00:00

abstract：:Failure of a superoxide generating system, the NADPH oxidase, present in neutrophils and other phagocytes gives rise to chronic granulomatous disease (CGD), a group of single-gene inherited disorders all characterized by an extreme susceptibility to pyogenic infection, with potentially fatal consequences. About 30% of...

journal_title：Blood

authors： Thrasher A,Chetty M,Casimir C,Segal AW

更新日期：1992-09-01 00:00:00

abstract：:Myeloma immunosurveillance remains incompletely understood. We have demonstrated proteolytic processing of the matrix proteoglycan, versican (VCAN), in myeloma tumors. Whereas intact VCAN exerts tolerogenic activities through Toll-like receptor 2 (TLR2) binding, the immunoregulatory consequences of VCAN proteolysis re...

journal_title：Blood

authors： Hope C,Foulcer S,Jagodinsky J,Chen SX,Jensen JL,Patel S,Leith C,Maroulakou I,Callander N,Miyamoto S,Hematti P,Apte SS,Asimakopoulos F

更新日期：2016-08-04 00:00:00

abstract：:Two sisters in whom a diagnosis of Fanconi's anemia was made at ages 12 and 18 subsequently developed acute nonlymphocytic leukemia (ANLL). A third sibling had previously died at age 11 of apparent sepsis. Both sisters had cytogenetic studies that showed increased chromosomal breakage and a 46,XX karyotype, but subseq...

journal_title：Blood

authors： Stivrins TJ,Davis RB,Sanger W,Fritz J,Purtilo DT

更新日期：1984-07-01 00:00:00

abstract：:As the principal component of the membrane skeleton, spectrin confers integrity and flexibility to red cell membranes. Although this network involves many interactions, the most common hemolytic anemia mutations that disrupt erythrocyte morphology affect the spectrin tetramerization domains. Although much is known cli...

journal_title：Blood

authors： Ipsaro JJ,Harper SL,Messick TE,Marmorstein R,Mondragón A,Speicher DW

更新日期：2010-06-10 00:00:00

abstract：:Leukemia inhibitory factor (LIF)/differentiation-inhibiting activity (DIA)/human interleukin for DA cells (HILDA) is a cytokine with biologic activities involving a variety of different types of target cells. Here we have tested LIF/DIA for possible effects on the growth and differentiation of normal human hematopoiet...

journal_title：Blood

authors： Leary AG,Wong GG,Clark SC,Smith AG,Ogawa M

更新日期：1990-05-15 00:00:00

abstract：:Monocytes from patients with sickle cell disease (SCD) are in an activated state. However, the mechanism of activation of monocytes in SCD is not known. Our studies showed that placenta growth factor (PlGF) activated monocytes and increased mRNA levels of cytokines (tumor necrosis factor-alpha [TNF-alpha] and interleu...

journal_title：Blood

authors： Selvaraj SK,Giri RK,Perelman N,Johnson C,Malik P,Kalra VK

更新日期：2003-08-15 00:00:00

abstract：:The signals that mediate T-cell infiltration during T-cell autoimmune diseases are poorly understood. The chemokine CCL21 (originally isolated by us and others as Exodus-2/6Ckine/SLC/TCA4) is highly potent and highly specific for stimulating T-cell migration. However, it is thought to be expressed only in secondary ly...

journal_title：Blood

authors： Christopherson KW 2nd,Hood AF,Travers JB,Ramsey H,Hromas RA

更新日期：2003-02-01 00:00:00

abstract：:Molecular mechanisms by which the Src homology 2 domain-containing inositol 5-phosphatase (SHIP) negatively regulates phagocytosis in macrophages are unclear. We addressed the issue using bone marrow-derived macrophages from FcgammaR- or SHIP-deficient mice. Phagocytic activities of macrophages from FcgammaRII(b)(-/-)...

journal_title：Blood

authors： Nakamura K,Malykhin A,Coggeshall KM

更新日期：2002-11-01 00:00:00

abstract：:Class A scavenger receptor (SRA), also known as CD204, has been shown to participate in the pathogenesis of atherosclerosis and the pattern recognition of pathogen infection. However, its role in adaptive immune responses has not been well defined. In this study, we report that the lack of SRA/CD204 promotes Toll-like...

journal_title：Blood

authors： Yi H,Yu X,Gao P,Wang Y,Baek SH,Chen X,Kim HL,Subjeck JR,Wang XY

更新日期：2009-06-04 00:00:00

abstract：:Endothelial progenitor cells are critically involved in essential biologic processes, such as vascular homeostasis, regeneration, and tumor angiogenesis. Endothelial colony-forming cells (ECFCs) are endothelial progenitor cells with robust proliferative potential. Their profound vessel-forming capacity makes them a pr...

journal_title：Blood

authors： Reinisch A,Hofmann NA,Obenauf AC,Kashofer K,Rohde E,Schallmoser K,Flicker K,Lanzer G,Linkesch W,Speicher MR,Strunk D

更新日期：2009-06-25 00:00:00

abstract：:Although CD4(+)CD25(+) T cells (T regulatory cells [Tregs]) and natural killer T cells (NKT cells) each protect against graft-versus-host disease (GVHD), interactions between these 2 regulatory cell populations after allogeneic bone marrow transplantation (BMT) have not been studied. We show that host NKT cells can in...

journal_title：Blood

authors： Pillai AB,George TI,Dutt S,Strober S

更新日期：2009-04-30 00:00:00