abstract：:The importance of glutamine (Gln) metabolism in multiple myeloma (MM) cells and its potential role as a therapeutic target are still unknown, although it has been reported that human myeloma cell lines (HMCLs) are highly sensitive to Gln depletion. In this study, we found that both HMCLs and primary bone marrow (BM) C...

journal_title：Blood

authors： Bolzoni M,Chiu M,Accardi F,Vescovini R,Airoldi I,Storti P,Todoerti K,Agnelli L,Missale G,Andreoli R,Bianchi MG,Allegri M,Barilli A,Nicolini F,Cavalli A,Costa F,Marchica V,Toscani D,Mancini C,Martella E,Dall'Asta V

更新日期：2016-08-04 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：:Clonal hematopoiesis (CH) in aplastic anemia (AA) has been closely linked to the evolution of late clonal disorders, including paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML), which are common complications after successful immunosuppressive therapy (IST). With the ...

journal_title：Blood

authors： Ogawa S

更新日期：2016-07-21 00:00:00

abstract：:Daratumumab targets CD38-expressing myeloma cells through a variety of immune-mediated mechanisms (complement-dependent cytotoxicity, antibody-dependent cell-mediated cytotoxicity, and antibody-dependent cellular phagocytosis) and direct apoptosis with crosslinking. These mechanisms may also target nonplasma cells tha...

journal_title：Blood

authors： Krejcik J,Casneuf T,Nijhof IS,Verbist B,Bald J,Plesner T,Syed K,Liu K,van de Donk NW,Weiss BM,Ahmadi T,Lokhorst HM,Mutis T,Sasser AK

更新日期：2016-07-21 00:00:00

abstract：:The majority of diffuse large B-cell lymphoma (DLBCL) tumors contain mutations in histone-modifying enzymes (HMEs), indicating a potential therapeutic benefit of histone deacetylase inhibitors (HDIs), and preclinical data suggest that HDIs augment the effect of rituximab. In this randomized phase 2 study, we evaluated...

journal_title：Blood

authors： Assouline SE,Nielsen TH,Yu S,Alcaide M,Chong L,MacDonald D,Tosikyan A,Kukreti V,Kezouh A,Petrogiannis-Haliotis T,Albuquerque M,Fornika D,Alamouti S,Froment R,Greenwood CM,Oros KK,Camglioglu E,Sharma A,Christodoulopoul

更新日期：2016-07-14 00:00:00

abstract：:Krüppel-like factor 5 (Klf5) encodes a zinc-finger transcription factor and has been reported to be a direct target of C/EBPα, a master transcription factor critical for formation of granulocyte-macrophage progenitors (GMP) and leukemic GMP. Using an in vivo hematopoietic-specific gene ablation model, we demonstrate t...

journal_title：Blood

authors： Shahrin NH,Diakiw S,Dent LA,Brown AL,D'Andrea RJ

更新日期：2016-07-07 00:00:00

abstract：:Adoptive transfer of T cells genetically modified to express chimeric antigen receptors (CARs) targeting CD19 has produced impressive results in treating patients with B-cell malignancies. Although these CAR-modified T cells target the same antigen, the designs of CARs vary as well as several key aspects of the clinic...

journal_title：Blood

authors： Park JH,Geyer MB,Brentjens RJ

更新日期：2016-06-30 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：:Fanconi anemia (FA) is the most frequent inherited cause of bone marrow failure (BMF). Most FA patients experience hematopoietic stem cell attrition and cytopenia during childhood, which along with intrinsic chromosomal instability, favor clonal evolution and the frequent emergence in their teens or young adulthood of...

journal_title：Blood

authors： Peffault de Latour R,Soulier J

更新日期：2016-06-16 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：:Neutrophilic differentiation is dependent on CCAAT enhancer-binding protein α (C/EBPα), a transcription factor expressed in multiple organs including the bone marrow. Using functional genomic technologies in combination with clustered regularly-interspaced short palindromic repeat (CRISPR)/CRISPR-associated protein 9 ...

journal_title：Blood

authors： Avellino R,Havermans M,Erpelinck C,Sanders MA,Hoogenboezem R,van de Werken HJ,Rombouts E,van Lom K,van Strien PM,Gebhard C,Rehli M,Pimanda J,Beck D,Erkeland S,Kuiken T,de Looper H,Gröschel S,Touw I,Bindels E,Delwel

更新日期：2016-06-16 00:00:00

abstract：:Light-mediated release of signaling ligands, such as chemoattractants, growth factors, and cytokines is an attractive strategy for investigation and therapeutic targeting of leukocyte communication and immune responses. We introduce a versatile optogenetic method to control ligand secretion, combining UV-conditioned e...

journal_title：Blood

authors： Sarris M,Olekhnovitch R,Bousso P

更新日期：2016-06-09 00:00:00

abstract：:The development of neutralizing antibodies (inhibitors) against coagulation factor VIII (FVIII) is the most problematic and costly complication of FVIII replacement therapy that affects up to 30% of previously untreated patients with severe hemophilia A. The development of inhibitors is a multifactorial complication i...

journal_title：Blood

authors： Gorski MM,Blighe K,Lotta LA,Pappalardo E,Garagiola I,Mancini I,Mancuso ME,Fasulo MR,Santagostino E,Peyvandi F

更新日期：2016-06-09 00:00:00

abstract：:Inherited bleeding, thrombotic, and platelet disorders (BPDs) are diseases that affect ∼300 individuals per million births. With the exception of hemophilia and von Willebrand disease patients, a molecular analysis for patients with a BPD is often unavailable. Many specialized tests are usually required to reach a put...

journal_title：Blood

authors： Simeoni I,Stephens JC,Hu F,Deevi SV,Megy K,Bariana TK,Lentaigne C,Schulman S,Sivapalaratnam S,Vries MJ,Westbury SK,Greene D,Papadia S,Alessi MC,Attwood AP,Ballmaier M,Baynam G,Bermejo E,Bertoli M,Bray PF,Bury L,

更新日期：2016-06-09 00:00:00

abstract：:Uncontrolled activation of the complement system against endothelial and blood cells is central to the pathogenesis of atypical hemolytic uremic syndrome (aHUS). aHUS patients frequently carry mutations in the inhibitory complement regulator factor H (FH). Mutations cluster in domains 19 and 20 (FH19-20), which are cr...

journal_title：Blood

authors： Hyvärinen S,Meri S,Jokiranta TS

更新日期：2016-06-02 00:00:00

abstract：:HIV/AIDS has long been at the forefront of the development of gene- and cell-based therapies. Although conventional gene therapy approaches typically involve the addition of anti-HIV genes to cells using semirandomly integrating viral vectors, newer genome editing technologies based on engineered nucleases are now all...

journal_title：Blood

authors： Wang CX,Cannon PM

更新日期：2016-05-26 00:00:00

abstract：:Administration of lymphodepletion chemotherapy followed by CD19-specific chimeric antigen receptor (CAR)-modified T cells is a remarkably effective approach to treating patients with relapsed and refractory CD19(+) B-cell malignancies. We treated 7 patients with B-cell acute lymphoblastic leukemia (B-ALL) harboring re...

journal_title：Blood

authors： Gardner R,Wu D,Cherian S,Fang M,Hanafi LA,Finney O,Smithers H,Jensen MC,Riddell SR,Maloney DG,Turtle CJ

更新日期：2016-05-19 00:00:00

abstract：:Maintenance of myeloid cell homeostasis requires continuous turnover of phagocytes from the bloodstream, yet whether environmental signals influence phagocyte longevity in the absence of inflammation remains unknown. Here, we show that the gut microbiota regulates the steady-state cellular lifespan of neutrophils and ...

journal_title：Blood

authors： Hergott CB,Roche AM,Tamashiro E,Clarke TB,Bailey AG,Laughlin A,Bushman FD,Weiser JN

更新日期：2016-05-19 00:00:00

abstract：:von Willebrand disease (VWD) is the most common inherited bleeding disorder, and type 1 VWD is the most common VWD variant. Despite its frequency, diagnosis of type 1 VWD remains the subject of debate. In order to study the spectrum of type 1 VWD in the United States, the Zimmerman Program enrolled 482 subjects with a...

journal_title：Blood

authors： Flood VH,Christopherson PA,Gill JC,Friedman KD,Haberichter SL,Bellissimo DB,Udani RA,Dasgupta M,Hoffmann RG,Ragni MV,Shapiro AD,Lusher JM,Lentz SR,Abshire TC,Leissinger C,Hoots WK,Manco-Johnson MJ,Gruppo RA,Boggio LN

更新日期：2016-05-19 00:00:00

abstract：:Doxorubicin, bleomycin, vinblastine sulfate, and dacarbazine (ABVD) is associated with severe toxicity in older patients, particularly from bleomycin-induced lung toxicity (BLT). Therefore, using bleomycin has been questioned in older Hodgkin lymphoma (HL) patients, especially in early-stage HL. We therefore analyzed ...

journal_title：Blood

authors： Böll B,Goergen H,Behringer K,Bröckelmann PJ,Hitz F,Kerkhoff A,Greil R,von Tresckow B,Eichenauer DA,Bürkle C,Borchmann S,Fuchs M,Diehl V,Engert A,Borchmann P

更新日期：2016-05-05 00:00:00

abstract：:Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease defined by transcriptional classifications, specific signaling and survival pathways, and multiple low-frequency genetic alterations. Preclinical model systems that capture the genetic and functional heterogeneity of DLBCL are urgently needed. Here, we g...

journal_title：Blood

authors： Chapuy B,Cheng H,Watahiki A,Ducar MD,Tan Y,Chen L,Roemer MG,Ouyang J,Christie AL,Zhang L,Gusenleitner D,Abo RP,Farinha P,von Bonin F,Thorner AR,Sun HH,Gascoyne RD,Pinkus GS,van Hummelen P,Wulf GG,Aster JC,Weinst

更新日期：2016-05-05 00:00:00

abstract：:Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disease. Inherited forms of HLH are caused by biallelic mutations in several effectors of granule-dependent lymphocyte-mediated cytotoxicity. A small proportion of patients with a so-called "secondary" form of HLH, which develops in the a...

journal_title：Blood

authors： Sepulveda FE,Garrigue A,Maschalidi S,Garfa-Traore M,Ménasché G,Fischer A,de Saint Basile G

更新日期：2016-04-28 00:00:00

abstract：:Chronic graft-versus-host disease (cGVHD) remains a major complication following allogeneic bone marrow transplantation (BMT). The discovery of novel therapeutics is dependent on assessment in preclinical murine models of cGVHD. Rho-associated kinase 2 (ROCK2) recently was shown to be implicated in regulation of inter...

journal_title：Blood

authors： Flynn R,Paz K,Du J,Reichenbach DK,Taylor PA,Panoskaltsis-Mortari A,Vulic A,Luznik L,MacDonald KK,Hill GR,Nyuydzefe MS,Weiss JM,Chen W,Trzeciak A,Serody JS,Aguilar EG,Murphy WJ,Maillard I,Munn D,Koreth J,Cutler CS

更新日期：2016-04-28 00:00:00

abstract：:Epstein-Barr virus (EBV) is a ubiquitous virus that establishes a latent infection within the host and in some cases can lead to the development of EBV-associated lymphomas, lymphoproliferative disorders, hemophagocytic lymphohistiocytosis, solid tumors, and other diseases. We studied the clinical significance of dete...

journal_title：Blood

authors： Kanakry JA,Hegde AM,Durand CM,Massie AB,Greer AE,Ambinder RF,Valsamakis A

更新日期：2016-04-21 00:00:00

abstract：:Caveolin-1 (Cav-1) is a key organizer of membrane specializations and a scaffold protein that regulates signaling in multiple cell types. We found increased Cav-1 expression in human and murine T cells after allogeneic hematopoietic cell transplantation. Indeed, Cav-1(-/-)donor T cells caused less severe acute graft-v...

journal_title：Blood

authors： Schönle A,Hartl FA,Mentzel J,Nöltner T,Rauch KS,Prestipino A,Wohlfeil SA,Apostolova P,Hechinger AK,Melchinger W,Fehrenbach K,Guadamillas MC,Follo M,Prinz G,Ruess AK,Pfeifer D,del Pozo MA,Schmitt-Graeff A,Duyster J,H

更新日期：2016-04-14 00:00:00

abstract：:In this issue of Blood, Rowley et al report that noncoding RNAs precisely regulate the messenger RNA (mRNA) profile in platelets. Interfering in this process using genetically engineered mice affects hemostatic and thrombotic functions of platelets. ...

journal_title：Blood

authors： Boilard E,Belleannée C

更新日期：2016-04-07 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：:In this issue of Blood, Palomo et al provide new insight into the mechanism of action of defibrotide as an endothelial protective agent, while Richardson et al present the encouraging final results of the phase 3 clinical study of defibrotide for the treatment of severe hepatic veno-occlusive disease (VOD), showing a ...

journal_title：Blood

authors： Arai S

更新日期：2016-03-31 00:00:00

abstract：:Megakaryopoiesis is the hierarchical differentiation of hematopoietic stem cells into megakaryocytes. Differentiating megakaryocytes undergo maturation characterized by endomitosis and produce numerous platelets through proplatelet formation. C-type lectin-like receptor 2 (CLEC-2) is a podoplanin (PDPN) receptor mainl...

journal_title：Blood

authors： Tamura S,Suzuki-Inoue K,Tsukiji N,Shirai T,Sasaki T,Osada M,Satoh K,Ozaki Y

更新日期：2016-03-31 00:00:00

abstract：:The transcription factor c-Maf is extensively involved in the pathophysiology of multiple myeloma (MM), a fatal malignancy of plasma cells. In the present study, affinity chromatography and mass spectrometry were used to identify c-Maf ubiquitination-associated proteins, from which the E3 ligase HERC4 was found to int...

journal_title：Blood

authors： Zhang Z,Tong J,Tang X,Juan J,Cao B,Hurren R,Chen G,Taylor P,Xu X,Shi CX,Du J,Hou J,Wang G,Wu D,Stewart AK,Schimmer AD,Moran MF,Mao X

更新日期：2016-03-31 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：:[This corrects the article DOI: 10.1182/blood-2014-09-599423.]. ...

journal_title：Blood

authors：

更新日期：2016-03-24 00:00:00

abstract：:Transplant-associated thrombotic microangiopathy (TA-TMA) occurs frequently after hematopoietic stem cell transplantation (HSCT) and can lead to significant morbidity and mortality. There are no data addressing individual susceptibility to TA-TMA. We performed a hypothesis-driven analysis of 17 candidate genes known t...

journal_title：Blood

authors： Jodele S,Zhang K,Zou F,Laskin B,Dandoy CE,Myers KC,Lane A,Meller J,Medvedovic M,Chen J,Davies SM

更新日期：2016-02-25 00:00:00

abstract：:Internal tandem duplication of the Fms-like tyrosine kinase-3 receptor (FLT3) internal tandem duplication (ITD) is found in 30% of acute myeloid leukemia (AML) and is associated with a poor outcome. In addition to tyrosine kinase inhibitors, therapeutic strategies that modulate the expression of FLT3-ITD are also prom...

journal_title：Blood

authors： Larrue C,Saland E,Boutzen H,Vergez F,David M,Joffre C,Hospital MA,Tamburini J,Delabesse E,Manenti S,Sarry JE,Récher C

更新日期：2016-02-18 00:00:00

abstract：:Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder with a complex pathogenesis, which includes both antibody- and T-cell-mediated effector mechanisms. Rituximab (an anti-human CD20 monoclonal antibody [mAb]) is one of the treatments for ITP and is known to deplete B cells but may also work by affecting t...

journal_title：Blood

authors： Guo L,Kapur R,Aslam R,Speck ER,Zufferey A,Zhao Y,Kim M,Lazarus AH,Ni H,Semple JW

更新日期：2016-02-11 00:00:00

abstract：:Beside its classical role in the coagulation cascade, coagulation factor X (FX) is involved in several major biological processes including inflammation and enhancement of virus-induced immune responses. We recently reported that the long circulatory half-life of FX is linked to its interaction with liver-resident mac...

journal_title：Blood

authors： Muczynski V,Bazaa A,Loubière C,Harel A,Cherel G,Denis CV,Lenting PJ,Christophe OD

更新日期：2016-02-11 00:00:00

abstract：:In this issue of Blood, Hong et al advocate for use of additional US Food and Drug Administration (FDA)–approved safety measures for transfusion. Most patients transfused with contaminated platelets do not show immediate clinical signs. Active surveillance suggests patient risk 10- to 40-fold higher than passive hemov...

journal_title：Blood

authors： Benjamin RJ

更新日期：2016-01-28 00:00:00

abstract：:Essential thrombocythemia (ET) and primary myelofibrosis (PMF) are chronic diseases characterized by clonal hematopoiesis and hyperproliferation of terminally differentiated myeloid cells. The disease is driven by somatic mutations in exon 9 of CALR or exon 10 of MPL or JAK2-V617F in >90% of the cases, whereas the rem...

journal_title：Blood

authors： Milosevic Feenstra JD,Nivarthi H,Gisslinger H,Leroy E,Rumi E,Chachoua I,Bagienski K,Kubesova B,Pietra D,Gisslinger B,Milanesi C,Jäger R,Chen D,Berg T,Schalling M,Schuster M,Bock C,Constantinescu SN,Cazzola M,Kralovi

更新日期：2016-01-21 00:00:00

abstract：:BRCA1 is critical for maintenance of genomic stability and interacts directly with several proteins that regulate hematopoietic stem cell function and are part of the Fanconi anemia (FA) double-strand break DNA repair pathway. The effects of complete BRCA1 deficiency on bone marrow (BM) function are unknown. To test t...

journal_title：Blood

authors： Vasanthakumar A,Arnovitz S,Marquez R,Lepore J,Rafidi G,Asom A,Weatherly M,Davis EM,Neistadt B,Duszynski R,Vardiman JW,Le Beau MM,Godley LA,Churpek JE

更新日期：2016-01-21 00:00:00

abstract：:Somatic hypermutation and class-switch recombination of the immunoglobulin (Ig) genes occur in germinal center (GC) B cells and are initiated through deamination of cytidine to uracil by activation-induced cytidine deaminase (AID). Resulting uracil-guanine mismatches are processed by uracil DNA glycosylase (UNG)-media...

journal_title：Blood

authors： Gu X,Booth CJ,Liu Z,Strout MP

更新日期：2016-01-07 00:00:00