abstract：:Patients with immune thrombocytopenia (ITP) commonly have antiplatelet antibodies that cause thrombocytopenia through Fcγ receptors (FcγRs). Antibodies specific for FcγRs, designed to inhibit antibody-FcγR interaction, had been shown to improve ITP in refractory human patients. However, the development of such FcγR-sp...

journal_title：Blood

authors： Yu X,Menard M,Prechl J,Bhakta V,Sheffield WP,Lazarus AH

更新日期：2016-01-07 00:00:00

abstract：:The congenital sideroblastic anemias (CSAs) are relatively uncommon diseases characterized by defects in mitochondrial heme synthesis, iron-sulfur (Fe-S) cluster biogenesis, or protein synthesis. Here we demonstrate that mutations in HSPA9, a mitochondrial HSP70 homolog located in the chromosome 5q deletion syndrome 5...

journal_title：Blood

authors： Schmitz-Abe K,Ciesielski SJ,Schmidt PJ,Campagna DR,Rahimov F,Schilke BA,Cuijpers M,Rieneck K,Lausen B,Linenberger ML,Sendamarai AK,Guo C,Hofmann I,Newburger PE,Matthews D,Shimamura A,Snijders PJ,Towne MC,Niemeyer CM,

更新日期：2015-12-17 00:00:00

abstract：:The bone marrow microenvironment contains a heterogeneous population of stromal cells organized into niches that support hematopoietic stem cells (HSCs) and other lineage-committed hematopoietic progenitors. The stem cell niche generates signals that regulate HSC self-renewal, quiescence, and differentiation. Here, we...

journal_title：Blood

authors： Calvi LM,Link DC

更新日期：2015-11-26 00:00:00

abstract：:Nijmegen breakage syndrome 1 (NBS1) is a component of the MRE11 complex, which is a sensor of DNA double-strand breaks and plays a crucial role in the DNA damage response. Because activated macrophages produce large amounts of reactive oxygen species (ROS) that can cause DNA lesions, we examined the role of NBS1 in ma...

journal_title：Blood

authors： Pereira-Lopes S,Tur J,Calatayud-Subias JA,Lloberas J,Stracker TH,Celada A

更新日期：2015-11-26 00:00:00

abstract：:Histone deacetylase (HDAC) inhibitors (HDACis) have demonstrated activity in hematological and solid malignancies. Vorinostat, romidepsin, belinostat, and panobinostat are Food and Drug Administration-approved for hematological malignancies and inhibit class II and/or class I HDACs, including HDAC1, 2, 3, and 6. We co...

journal_title：Blood

authors： Matthews GM,Mehdipour P,Cluse LA,Falkenberg KJ,Wang E,Roth M,Santoro F,Vidacs E,Stanley K,House CM,Rusche JR,Vakoc CR,Zuber J,Minucci S,Johnstone RW

更新日期：2015-11-19 00:00:00

abstract：:Joint bleeding after (sports) trauma, after major joint surgery, or as seen in hemophilia in general leads to arthropathy. Joint degeneration is considered to result from the direct effects of blood components on cartilage and indirectly from synovial inflammation. Blood-provided proinflammatory cytokines trigger chon...

journal_title：Blood

authors： van Vulpen LF,Schutgens RE,Coeleveld K,Alsema EC,Roosendaal G,Mastbergen SC,Lafeber FP

更新日期：2015-11-05 00:00:00

abstract：:Drug-dependent antibodies (DDAbs) that cause acute thrombocytopenia upon drug exposure are nonreactive in the absence of the drug but bind tightly to a platelet membrane glycoprotein, usually α(IIb)/β3 integrin (GPIIb/IIIa) when the drug is present. How a drug promotes binding of antibody to its target is unknown and ...

journal_title：Blood

authors： Bougie DW,Peterson J,Rasmussen M,Aster RH

更新日期：2015-10-29 00:00:00

abstract：:Monitoring unfractionated heparin (UFH) is crucial to prevent over- or under-anticoagulation. However, the optimal parameters for monitoring UFH in children are not well established. The study objectives were to investigate (1) the relationship between UFH dose and its anticoagulant effect as assessed by anti-Xa, acti...

journal_title：Blood

authors： Hanslik A,Kitzmüller E,Tran US,Thom K,Karapetian H,Prutsch N,Voitl J,Michel-Behnke I,Newall F,Male C

更新日期：2015-10-29 00:00:00

abstract：:Drug-induced immune thrombocytopenia (DITP) is caused by antibodies that react with specific platelet-membrane glycoproteins when the provoking drug is present. More than 100 drugs have been implicated as triggers for this condition, quinine being one of the most common. The cause of DITP in most cases appears to be a...

journal_title：Blood

authors： Zhu J,Zhu J,Bougie DW,Aster RH,Springer TA

更新日期：2015-10-29 00:00:00

abstract：:In this issue of Blood, Prasad et al describe a mouse model with a mutation in the Aα chain of fibrinogen such that no fibrin polymer is formed in vivo, allowing for the first time the differentiation of the role of fibrinogen vs fibrin oligomer or polymer in antimicrobial host defense and in hemostasis/thrombosis. ...

journal_title：Blood

authors： Litvinov RI,Weisel JW

更新日期：2015-10-22 00:00:00

abstract：:Fetal hemoglobin (HbF, α2γ2) induction is a well-validated strategy for sickle cell disease (SCD) treatment. Using a small-molecule screen, we found that UNC0638, a selective inhibitor of EHMT1 and EHMT2 histone methyltransferases, induces γ-globin expression. EHMT1/2 catalyze mono- and dimethylation of lysine 9 on hi...

journal_title：Blood

authors： Renneville A,Van Galen P,Canver MC,McConkey M,Krill-Burger JM,Dorfman DM,Holson EB,Bernstein BE,Orkin SH,Bauer DE,Ebert BL

更新日期：2015-10-15 00:00:00

abstract：:This phase 2 study evaluated whether substituting bortezomib for vincristine in frontline rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) therapy could improve efficacy in non-germinal center B-cell-like diffuse large B-cell lymphoma (non-GCB DLBCL), centrally confirmed by immunohist...

journal_title：Blood

authors： Offner F,Samoilova O,Osmanov E,Eom HS,Topp MS,Raposo J,Pavlov V,Ricci D,Chaturvedi S,Zhu E,van de Velde H,Enny C,Rizo A,Ferhanoglu B

更新日期：2015-10-15 00:00:00

abstract：:In this issue of Blood, Wang et al describe the occurrence and pathogenetic relevance of IDH2R172 mutations in angioimmunoblastic T-cell lymphoma (AITL). ...

journal_title：Blood

authors： Pileri SA

更新日期：2015-10-08 00:00:00

abstract：:In Wiskott-Aldrich syndrome (WAS), immunodeficiency and autoimmunity often comanifest, yet how WAS mutations misregulate chromatin-signaling in Thelper (TH) cells favoring development of auto-inflammation over protective immunity is unclear. Previously, we identified an essential promoter-specific, coactivator role of...

journal_title：Blood

authors： Sarkar K,Sadhukhan S,Han SS,Vyas YM

更新日期：2015-10-01 00:00:00

abstract：:Von Willebrand factor (VWF) is a key hemostatic protein synthesized in both endothelial cells and megakaryocytes. Megakaryocyte-derived VWF is stored in α-granules of platelets and is enriched in hyperactive "ultra-large" VWF multimers. To elucidate the specific contribution of platelet VWF in hemostasis and thrombosi...

journal_title：Blood

authors： Verhenne S,Denorme F,Libbrecht S,Vandenbulcke A,Pareyn I,Deckmyn H,Lambrecht A,Nieswandt B,Kleinschnitz C,Vanhoorelbeke K,De Meyer SF

更新日期：2015-10-01 00:00:00

abstract：:Omenn syndrome (OS) is a severe immunodeficiency associated with erythroderma, lymphoproliferation, elevated IgE, and hyperactive oligoclonal T cells. A restricted T-cell repertoire caused by defective thymic T-cell development and selection, lymphopenia with homeostatic proliferation, and lack of regulatory T cells a...

journal_title：Blood

authors： Fuchs S,Rensing-Ehl A,Pannicke U,Lorenz MR,Fisch P,Jeelall Y,Rohr J,Speckmann C,Vraetz T,Farmand S,Schmitt-Graeff A,Krüger M,Strahm B,Henneke P,Enders A,Horikawa K,Goodnow C,Schwarz K,Ehl S

更新日期：2015-10-01 00:00:00

abstract：:In this issue of Blood, Donadieu et al present what may be the most encouraging data to date on a group of patients with Langerhans cell histiocytosis (LCH), which historically has poor disease-free survival and poor overall survival. ...

journal_title：Blood

authors： Stine KC

更新日期：2015-09-17 00:00:00

abstract：:Many researchers have speculated that the clinical progression from monoclonal gammopathy of undetermined significance (MGUS) to multiple myeloma (MM) is driven by defects in dendritic cell (DC) function. However, evidence supporting this assumption is controversial, and no mechanism for the putative DC dysfunction ha...

journal_title：Blood

authors： Leone P,Berardi S,Frassanito MA,Ria R,De Re V,Cicco S,Battaglia S,Ditonno P,Dammacco F,Vacca A,Racanelli V

更新日期：2015-09-17 00:00:00

abstract：:Cancer is a leading cause of thrombosis. We identify a new procoagulant mechanism that contributes to thromboembolism in prostate cancer and allows for safe anticoagulation therapy development. Prostate cancer-mediated procoagulant activity was reduced in plasma in the absence of factor XII or its substrate of the int...

journal_title：Blood

authors： Nickel KF,Ronquist G,Langer F,Labberton L,Fuchs TA,Bokemeyer C,Sauter G,Graefen M,Mackman N,Stavrou EX,Ronquist G,Renné T

更新日期：2015-09-10 00:00:00

abstract：:Fibrinogen, coagulation factor VII (FVII), and factor VIII (FVIII) and its carrier von Willebrand factor (vWF) play key roles in hemostasis. Previously identified common variants explain only a small fraction of the trait heritabilities, and additional variations may be explained by associations with rarer variants wi...

journal_title：Blood

authors： Huffman JE,de Vries PS,Morrison AC,Sabater-Lleal M,Kacprowski T,Auer PL,Brody JA,Chasman DI,Chen MH,Guo X,Lin LA,Marioni RE,Müller-Nurasyid M,Yanek LR,Pankratz N,Grove ML,de Maat MP,Cushman M,Wiggins KL,Qi L,Sennb

更新日期：2015-09-10 00:00:00

abstract：:The Gardos channel is a Ca(2+)-sensitive, intermediate conductance, potassium selective channel expressed in several tissues including erythrocytes and pancreas. In normal erythrocytes, it is involved in cell volume modification. Here, we report the identification of a dominantly inherited mutation in the Gardos chann...

journal_title：Blood

authors： Rapetti-Mauss R,Lacoste C,Picard V,Guitton C,Lombard E,Loosveld M,Nivaggioni V,Dasilva N,Salgado D,Desvignes JP,Béroud C,Viout P,Bernard M,Soriani O,Vinti H,Lacroze V,Feneant-Thibault M,Thuret I,Guizouarn H,Badens C

更新日期：2015-09-10 00:00:00

abstract：:Cancer cells require glutamine to adapt to increased biosynthetic activity. The limiting step in intracellular glutamine catabolism involves its conversion to glutamate by glutaminase (GA). Different GA isoforms are encoded by the genes GLS1 and GLS2 in humans. Herein, we show that glutamine levels control mitochondri...

journal_title：Blood

authors： Jacque N,Ronchetti AM,Larrue C,Meunier G,Birsen R,Willems L,Saland E,Decroocq J,Maciel TT,Lambert M,Poulain L,Hospital MA,Sujobert P,Joseph L,Chapuis N,Lacombe C,Moura IC,Demo S,Sarry JE,Recher C,Mayeux P,Tambur

更新日期：2015-09-10 00:00:00

abstract：:Systemic inflammation perturbs the bone marrow environment by evicting resident B cells and favoring granulopoiesis over lymphopoiesis. Despite these conditions, a subset of marrow B cell remains to become activated and produce potent acute immunoglobulin M (IgM) responses. This discrepancy is currently unresolved and...

journal_title：Blood

authors： Moreau JM,Berger A,Nelles ME,Mielnik M,Furlonger C,Cen SY,Besla R,Robbins CS,Paige CJ

更新日期：2015-09-03 00:00:00

abstract：:Hairy cell leukemia (HCL) is marked by near 100% mutational frequency of BRAFV600E mutations. Recurrent cooperating genetic events that may contribute to HCL pathogenesis or affect the clinical course of HCL are currently not described. Therefore, we performed whole exome sequencing to explore the mutational landscape...

journal_title：Blood

authors： Dietrich S,Hüllein J,Lee SC,Hutter B,Gonzalez D,Jayne S,Dyer MJ,Oleś M,Else M,Liu X,Słabicki M,Wu B,Troussard X,Dürig J,Andrulis M,Dearden C,von Kalle C,Granzow M,Jauch A,Fröhling S,Huber W,Meggendorfer M,Hafe

更新日期：2015-08-20 00:00:00

abstract：:Rapid exclusion of heparin-induced thrombocytopenia (HIT) is needed to determine which patients can continue to receive heparin. In this prospective management study, 526 participants had a 4Ts score, rapid particle gel immunoassay (platelet factor 4/heparin [PF4/H]-PaGIA), and serotonin-release assay (SRA) performed....

journal_title：Blood

authors： Linkins LA,Bates SM,Lee AY,Heddle NM,Wang G,Warkentin TE

更新日期：2015-07-30 00:00:00

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 tumorigenicity of most cases of ALK-positive anaplastic large-cell lymphoma (ALK+ ALCL) is driven by the oncogenic fusion protein NPM-ALK in a STAT3-dependent manner. Because it has been shown that STAT3 can be inhibited by STAT1 in some experimental models, we hypothesized that the STAT1 signaling pathway is defe...

journal_title：Blood

authors： Wu C,Molavi O,Zhang H,Gupta N,Alshareef A,Bone KM,Gopal K,Wu F,Lewis JT,Douglas DN,Kneteman NM,Lai R

更新日期：2015-07-16 00:00:00

abstract：:Therapy for childhood acute lymphoblastic leukemia (ALL) is associated with 5-year survival rates of ∼90% even after largely eliminating cranial radiation. This meta-analysis assesses the long-term neurocognitive functioning after chemotherapy-only regimens among survivors of childhood ALL. We conducted a systematic r...

journal_title：Blood

authors： Iyer NS,Balsamo LM,Bracken MB,Kadan-Lottick NS

更新日期：2015-07-16 00:00:00

abstract：:We report the international experience in outcomes after related and unrelated hematopoietic transplantation for infantile osteopetrosis in 193 patients. Thirty-four percent of transplants used grafts from HLA-matched siblings, 13% from HLA-mismatched relatives, 12% from HLA-matched, and 41% from HLA-mismatched unrela...

journal_title：Blood

authors： Orchard PJ,Fasth AL,Le Rademacher J,He W,Boelens JJ,Horwitz EM,Al-Seraihy A,Ayas M,Bonfim CM,Boulad F,Lund T,Buchbinder DK,Kapoor N,O'Brien TA,Perez MA,Veys PA,Eapen M

更新日期：2015-07-09 00:00:00

abstract：:Congenital erythropoietic porphyria (CEP) is an autosomal recessive disorder of heme synthesis characterized by reduced activity of uroporphyrinogen III synthase and the accumulation of nonphysiologic isomer I porphyrin metabolites, resulting in ineffective erythropoiesis and devastating skin photosensitivity. Managem...

journal_title：Blood

authors： Egan DN,Yang Z,Phillips J,Abkowitz JL

更新日期：2015-07-09 00:00:00

abstract：:Early B-cell factor 1 (Ebf1) is a transcription factor with documented dose-dependent functions in normal and malignant B-lymphocyte development. To understand more about the roles of Ebf1 in malignant transformation, we investigated the impact of reduced functional Ebf1 dosage on mouse B-cell progenitors. Gene expres...

journal_title：Blood

authors： Prasad MA,Ungerbäck J,Åhsberg J,Somasundaram R,Strid T,Larsson M,Månsson R,De Paepe A,Lilljebjörn H,Fioretos T,Hagman J,Sigvardsson M

更新日期：2015-06-25 00:00:00

abstract：:Current treatment strategies for chronic lymphocytic leukemia (CLL) involve a combination of conventional chemotherapeutics, monoclonal antibodies, and targeted signaling inhibitors. However, CLL remains largely incurable, with drug resistance and treatment relapse a common occurrence, leading to the search for novel ...

journal_title：Blood

authors： Blunt MD,Carter MJ,Larrayoz M,Smith LD,Aguilar-Hernandez M,Cox KL,Tipton T,Reynolds M,Murphy S,Lemm E,Dias S,Duncombe A,Strefford JC,Johnson PW,Forconi F,Stevenson FK,Packham G,Cragg MS,Steele AJ

更新日期：2015-06-25 00:00:00

abstract：:In this issue of Blood, Sehgal et al report on the clinical and pharmacodynamics analysis of pomalidomide dosing strategies in multiple myeloma (MM) and their impact on immune activation and cereblon targets. The particular novelty of this study lies in the direct correlation of immune effects triggered by pomalidomid...

journal_title：Blood

authors： Podar K

更新日期：2015-06-25 00:00:00

abstract：:Eosinophils are associated with type 2 immune responses to allergens and helminths. They release various proinflammatory mediators and toxic proteins on activation and are therefore considered proinflammatory effector cells. Eosinophilia is promoted by the cytokines interleukin (IL)-3, IL-5, and granulocyte macrophage...

journal_title：Blood

authors： Schwartz C,Willebrand R,Huber S,Rupec RA,Wu D,Locksley R,Voehringer D

更新日期：2015-06-18 00:00:00

abstract：:Redox biology is fundamental to both normal cellular homeostasis and pathological states associated with excessive oxidative stress. Reactive oxygen species function not only as signaling molecules but also as redox regulators of protein function. In the vascular system, redox reactions help regulate key physiologic r...

journal_title：Blood

authors： Gu SX,Stevens JW,Lentz SR

更新日期：2015-06-18 00:00:00

abstract：:Intravascular hemolysis describes the relocalization of heme and hemoglobin (Hb) from erythrocytes to plasma. We investigated the concept that erythrocyte membrane microparticles (MPs) concentrate cell-free heme in human hemolytic diseases, and that heme-laden MPs have a physiopathological impact. Up to one-third of c...

journal_title：Blood

authors： Camus SM,De Moraes JA,Bonnin P,Abbyad P,Le Jeune S,Lionnet F,Loufrani L,Grimaud L,Lambry JC,Charue D,Kiger L,Renard JM,Larroque C,Le Clésiau H,Tedgui A,Bruneval P,Barja-Fidalgo C,Alexandrou A,Tharaux PL,Boulanger CM

更新日期：2015-06-11 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：:In this issue of Blood, Kurtz et al report the potential clinical utility of immunoglobulin high-throughput sequencing as a tool for disease monitoring and surveillance in aggressive B-cell lymphoma. ...

journal_title：Blood

authors： Sweetenham JW

更新日期：2015-06-11 00:00:00

abstract：:In this study, we randomly compared high doses of the tyrosine kinase inhibitor imatinib combined with reduced-intensity chemotherapy (arm A) to standard imatinib/hyperCVAD (cyclophosphamide/vincristine/doxorubicin/dexamethasone) therapy (arm B) in 268 adults (median age, 47 years) with Philadelphia chromosome-positiv...

journal_title：Blood

authors： Chalandon Y,Thomas X,Hayette S,Cayuela JM,Abbal C,Huguet F,Raffoux E,Leguay T,Rousselot P,Lepretre S,Escoffre-Barbe M,Maury S,Berthon C,Tavernier E,Lambert JF,Lafage-Pochitaloff M,Lhéritier V,Chevret S,Ifrah N,Dombr

更新日期：2015-06-11 00:00:00

abstract：:The Augustine-negative alias At(a-) blood type, which seems to be restricted to people of African ancestry, was identified half a century ago but remains one of the last blood types with no known genetic basis. Here we report that a nonsynonymous single nucleotide polymorphism in SLC29A1 (rs45458701) is responsible fo...

journal_title：Blood

authors： Daniels G,Ballif BA,Helias V,Saison C,Grimsley S,Mannessier L,Hustinx H,Lee E,Cartron JP,Peyrard T,Arnaud L

更新日期：2015-06-04 00:00:00