Identical mutations in RAG1 or RAG2 genes leading to defective V(D)J recombinase activity can cause either T-B-severe combined immune deficiency or Omenn syndrome.

abstract：:Measurements of hepatic iron concentration (HIC) are important predictors of transfusional iron burden and long-term outcome in patients with transfusion-dependent anemias. The goal of this work was to develop a readily available, noninvasive method for clinical HIC measurement. The relaxation rates R2 (1/T2) and R2* ...

journal_title：Blood

authors： Wood JC,Enriquez C,Ghugre N,Tyzka JM,Carson S,Nelson MD,Coates TD

更新日期：2005-08-15 00:00:00

abstract：:Cutaneous involvement is seen in ~ 50% of adult T-cell leukemia/lymphoma (ATLL) patients. We investigated the association between skin eruption type and prognosis in 119 ATLL patients. ATLL eruptions were categorized into patch (6.7%), plaque (26.9%), multipapular (19.3%), nodulotumoral (38.7%), erythrodermic (4.2%), ...

journal_title：Blood

authors： Sawada Y,Hino R,Hama K,Ohmori S,Fueki H,Yamada S,Fukamachi S,Tajiri M,Kubo R,Yoshioka M,Nakashima D,Sugita K,Yoshiki R,Shimauchi T,Mori T,Izu K,Kobayashi M,Nakamura M,Tokura Y

更新日期：2011-04-14 00:00:00

abstract：:This study examines the role of neutrophils (PMN) in the pathogenesis of endotoxin-induced microclot formation. It is intended to clarify whether granulocytes are involved in endotoxin-induced activation of intravascular coagulation (generation of soluble fibrin) and/or in endotoxin-induced precipitation of soluble fi...

journal_title：Blood

authors： Müller-Berghaus G,Eckhardt T

更新日期：1975-05-01 00:00:00

abstract：:Our previous study demonstrated that 2-methoxyestradiol (2ME2), an estrogen derivative, induces apoptosis in multiple myeloma (MM) cells; however, the related transcriptional events are unclear. In the present study, we used oligonucleotide microarrays to identify genes altered during 2ME2-induced apoptosis in MM cell...

journal_title：Blood

authors： Chauhan D,Li G,Auclair D,Hideshima T,Richardson P,Podar K,Mitsiades N,Mitsiades C,Li C,Kim RS,Munshi N,Chen LB,Wong W,Anderson KC

更新日期：2003-05-01 00:00:00

abstract：:In a phase I/II study, 19 patients with advanced tumors but normal hematopoiesis and nine patients with bone marrow failure and prolonged severe cytopenias were treated with recombinant human interleukin-3 (rhIL-3) to assess the toxicity and biological effects of this multipotential hematopoietic growth factor. Doses ...

journal_title：Blood

authors： Ganser A,Lindemann A,Seipelt G,Ottmann OG,Herrmann F,Eder M,Frisch J,Schulz G,Mertelsmann R,Hoelzer D

更新日期：1990-08-15 00:00:00

abstract：:Glanzmann thrombasthenia (GT) is the most common inherited disorder of platelets. Most of the molecular defects previously identified in GT have been caused by point (or other small) mutations in the genes for glycoprotein (GP) IIb or GPIIIa. We have used single-strand conformation polymorphism (SSCP) analysis to rapi...

journal_title：Blood

authors： Jin Y,Dietz HC,Nurden A,Bray PF

更新日期：1993-10-15 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：:Polycythemia vera (PV) treatment with interferon α (IFNα) is frequently limited by dose-related toxicity. PV CD34(+) cells are characterized by overexpression of Bcl-xL, which can be antagonized by ABT-737 leading to apoptosis. We explored the effects of ABT-737 and IFNα on PV hematopoiesis. Both IFNα and ABT-737 alon...

journal_title：Blood

authors： Lu M,Wang J,Li Y,Berenzon D,Wang X,Mascarenhas J,Xu M,Hoffman R

更新日期：2010-11-18 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：:Plasma fibronectin (pFN) cross-linked to fibrin during the injury response provides a provisional matrix required for cells to begin tissue repair. Using a synthetic matrix of pFN and fibrin as a substrate for cell adhesion and spreading, we have determined that pFN covalently cross-linked to fibrin into a complex mul...

journal_title：Blood

authors： Corbett SA,Wilson CL,Schwarzbauer JE

更新日期：1996-07-01 00:00:00

abstract：:The effect of recombinant factor VIII (rFVIII) brand on inhibitor development was investigated in all 407 severe hemophilia A previously untreated patients born in the United Kingdom (UK) between 1 January 2000 and 31 December 2011. Eighty-eight (22%) had been in the RODIN study. Information was extracted from the Nat...

journal_title：Blood

authors： Collins PW,Palmer BP,Chalmers EA,Hart DP,Liesner R,Rangarajan S,Talks K,Williams M,Hay CR,UK Haemophilia Centre Doctors’ Organization.

更新日期：2014-11-27 00:00:00

abstract：:Toll-like receptors (TLRs) are able to interact with pathogen-derived products and their signals induce the coordinated activation of innate and adaptive immune mechanisms. Dendritic cells (DCs) play a central role in these events. As the different TLRs are able to trigger MyD88/TRIF-dependent and -independent signali...

journal_title：Blood

authors： Warger T,Osterloh P,Rechtsteiner G,Fassbender M,Heib V,Schmid B,Schmitt E,Schild H,Radsak MP

更新日期：2006-07-15 00:00:00

abstract：:Allogeneic stem cell transplantation (SCT) is a unique procedure, primarily in patients with hematopoietic malignancies, involving chemoradiotherapy followed by the introduction of donor hematopoietic and immune cells into an inflamed and lymphopenic environment. Interruption of the process by which recipient alloanti...

journal_title：Blood

authors： Koyama M,Hill GR

更新日期：2016-06-16 00:00:00

abstract：:Concentrations of plasma fibrinopeptide A (FPA) were measured by radioimmunoassay in 50 patients with venous thromboembolism or disseminated intravascular coagulation or both. A consistent discrepancy was observed in values obtained with two anti-FPA antisera. Analysis of extracts from plasma of these patients by high...

journal_title：Blood

authors： Leeksma OC,Meijer-Huizinga F,Stoepman-van Dalen EA,van Ginkel CJ,van Aken WG,van Mourik JA

更新日期：1986-05-01 00:00:00

abstract：:The POEMS syndrome (polyradiculoneuropathy, organomegaly, multiple endocrinopathies, monoclonal protein, skin changes) is a rare disease associated with a plasma cell dyscrasia. Patients with disseminated POEMS can be treated with high-dose therapy and autologous stem cell transplantation (ASCT). While clinical improv...

journal_title：Blood

authors： D'Souza A,Lacy M,Gertz M,Kumar S,Buadi F,Hayman S,Dingli D,Zeldenrust S,Kyle R,Ansell S,Inwards D,Johnston P,Micallef I,Porrata L,Litzow M,Gastineau D,Hogan W,Dispenzieri A

更新日期：2012-07-05 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：:De novo presentation of acute myeloid leukemia (AML) expressing the Philadelphia (Ph) chromosomal abnormality is rare and is associated with a dismal prognosis. To date, reported cases of Ph(+) AML have expressed either the e13a2 or e14a2 BCR-ABL fusion transcripts. We report a unique case of de novo AML expressing th...

journal_title：Blood

authors： Ritchie DS,McBean M,Westerman DA,Kovalenko S,Seymour JF,Dobrovic A

更新日期：2008-03-01 00:00:00

abstract：:Severe congenital neutropenia (SCN) is a syndrome characterized by an isolated block in granulocytic differentiation and an increased risk of developing acute myeloid leukemia (AML). Recent studies have demonstrated that the majority of patients with SCN and cyclic neutropenia, a related disorder characterized by peri...

journal_title：Blood

authors： Grenda DS,Johnson SE,Mayer JR,McLemore ML,Benson KF,Horwitz M,Link DC

更新日期：2002-11-01 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：:Although hereditary spherocytosis (HS) is a common disorder of the red cell membrane, its clinical and biologic expression at birth and in early infancy has received little attention. In order to obtain insights into the natural history of HS during infancy, we studied 46 neonates, 39 from families in which 1 of the p...

journal_title：Blood

authors： Delhommeau F,Cynober T,Schischmanoff PO,Rohrlich P,Delaunay J,Mohandas N,Tchernia G

更新日期：2000-01-15 00:00:00

abstract：:In this issue of Blood, Martin et al report poor outcomes for ibrutinib-refractory patients with mantle cell lymphoma (MCL). MCL, an uncommon B-cell lymphoma driven by dysregulated cyclin D1, responds well to initial therapy but is destined to relapse. Subsequent single-agent treatments had modest response rates and d...

journal_title：Blood

authors： Smith MR

更新日期：2016-03-24 00:00:00

abstract：:Human transferrin receptor-2 (TFR-2) is a protein highly homologous to TFR-1/CD71 and is endowed with the ability to bind transferrin (TF) with low affinity. High levels of TFR-2 mRNA were found in the liver and in erythroid precursors. Mutations affecting the TFR-2 gene led to hemochromatosis type 3, a form of inheri...

journal_title：Blood

authors： Deaglio S,Capobianco A,Calì A,Bellora F,Alberti F,Righi L,Sapino A,Camaschella C,Malavasi F

更新日期：2002-11-15 00:00:00

abstract：:Functionally inhibitory antibody to the plasma membrane complement inhibitor CD59 has been used to investigate control of the terminal complement proteins at the endothelial cell surface. Antibodies against purified human erythrocyte CD59 (polyclonal anti-CD59 and monoclonal antibodies [MoAbs] 1F1 and 1F5) were found ...

journal_title：Blood

authors： Hamilton KK,Ji Z,Rollins S,Stewart BH,Sims PJ

更新日期：1990-12-15 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：:In this issue of Blood, Usmani et al provide important information on second primary malignancies in patients treated with thalidomide and lenalidomide in the Arkansas total therapy trials. ...

journal_title：Blood

authors： Rajkumar SV

更新日期：2012-08-23 00:00:00

abstract：:Agammaglobulinemia is the most profound primary antibody deficiency that can occur due to an early termination of B-cell development. We here investigated 3 novel patients, including the first known adult, from unrelated families with agammaglobulinemia, recurrent infections, and hypertrophic cardiomyopathy (HCM). Two...

journal_title：Blood

authors： Saettini F,Poli C,Vengoechea J,Bonanomi S,Orellana JC,Fazio G,Rodriguez FH,Noguera LP,Booth CA,Jarur-Chamy V,Shams M,Iascone M,Vukic M,Gasperini S,Quadri M,Barroeta Seijas AB,Rivers E,Mauri M,Badolato R,Cazzaniga G

更新日期：2020-09-09 00:00:00

abstract：:Humans and mice with impaired perforin-dependent cytotoxic function may develop excessive T-cell activation and the fatal disorder hemophagocytic lymphohistiocytosis (HLH) after infection. Though cytotoxic lymphocytes can kill antigen-presenting cells, the physiological mechanism of perforin-mediated immune regulation...

journal_title：Blood

authors： Terrell CE,Jordan MB

更新日期：2013-06-27 00:00:00

abstract：:Expression of vascular endothelial growth factor (VEGF) is tightly regulated to achieve normal angiogenesis. The objective was to examine regulation of VEGF by the activin-like kinase receptors (ALKs) ALK1 and ALK5. Transforming growth factor beta1 (TGFbeta1) and bone morphogenetic protein-9 (BMP-9) enhanced and suppr...

journal_title：Blood

authors： Shao ES,Lin L,Yao Y,Boström KI

更新日期：2009-09-03 00:00:00

abstract：:A novel thrombin inhibitor based on single-stranded (ss) deoxynucleotides with the sequence GGTTGGTGTGGTTGG (thrombin aptamer) has been recently discovered. In this study, we tested its efficacy in inhibiting clot-bound thrombin activity and platelet thrombus formation in an ex vivo whole artery angioplasty model. The...

journal_title：Blood

authors： Li WX,Kaplan AV,Grant GW,Toole JJ,Leung LL

更新日期：1994-02-01 00:00:00

abstract：:Reduced levels of somatic hypermutation (SHM) have recently been described in IgG-switched immunoglobulin genes in a minority of patients with common variable immunodeficiency (CVID), demonstrating a disruption of the normal linkage between isotype switch and SHM. To see if, irrespective of isotype, there is a tendenc...

journal_title：Blood

authors： Andersen P,Permin H,Andersen V,Schejbel L,Garred P,Svejgaard A,Barington T

更新日期：2005-01-15 00:00:00