abstract：:Tissue factor pathway inhibitor (TFPI) inhibits proteases in the blood coagulation cascade that lead to the production of thrombin, including prothrombinase (factor Xa [FXa]/FVa), the catalytic complex that directly generates thrombin. Thus, TFPI and FV are directly linked in regulating the procoagulant response. Stud...

journal_title：Blood

authors： Maroney SA,Westrick RJ,Cleuren AC,Martinez ND,Siebert AE,Zogg M,Ginsburg D,Weiler H,Mast AE

更新日期：2021-01-14 00:00:00

abstract：:Plasminogen activation rates are enhanced by cell surface binding. We previously demonstrated that exogenous plasminogen binds to phosphatidylserine-exposing and spread platelets. Platelets contain plasminogen in their α-granules, but secretion of plasminogen from platelets has not been studied. Recently, a novel tran...

journal_title：Blood

authors： Whyte CS,Morrow GB,Baik N,Booth NA,Jalal MM,Parmer RJ,Miles LA,Mutch NJ

更新日期：2021-01-14 00:00:00

abstract：:Graft-versus-host disease (GVHD) is a prominent barrier to allogeneic hematopoietic stem cell transplantation (AHSCT). Definitive diagnosis of GVHD is invasive, and biopsies of involved tissues pose a high risk of bleeding and infection. T cells are central to GVHD pathogenesis, and our previous studies in a chronic G...

journal_title：Blood

authors： Assmann JC,Farthing DE,Saito K,Maglakelidze N,Oliver B,Warrick KA,Sourbier C,Ricketts CJ,Meyer TJ,Pavletic SZ,Linehan WM,Krishna MC,Gress RE,Buxbaum NP

更新日期：2021-01-07 00:00:00

abstract：:Natural killer (NK) cells play critical roles in protection against hematological malignancies but can acquire a dysfunctional state, which limits antitumor immunity. However, the underlying reasons for this impaired NK cell function remain to be uncovered. We found that NK cells in aggressive B-cell lymphoma underwen...

journal_title：Blood

authors： Kobayashi T,Lam PY,Jiang H,Bednarska K,Gloury R,Murigneux V,Tay J,Jacquelot N,Li R,Tuong ZK,Leggatt GR,Gandhi MK,Hill MM,Belz GT,Ngo S,Kallies A,Mattarollo SR

更新日期：2020-12-24 00:00:00

abstract：:Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare primary immunodeficiency caused by gain-of-function mutations in the CXCR4 gene. We report the safety, tolerability, pharmacokinetics, pharmacodynamics, and preliminary efficacy of mavorixafor from a phase 2 open-label dose-escalatio...

journal_title：Blood

authors： Dale DC,Firkin F,Bolyard AA,Kelley M,Makaryan V,Gorelick KJ,Ebrahim T,Garg V,Tang W,Jiang H,Skerlj R,Beaussant Cohen S

更新日期：2020-12-24 00:00:00

abstract：:Adult-onset hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease of immune hyperactivation. Unlike pediatric HLH, adult HLH is rarely driven by germline genetic variants. Although numerous precipitating etiologies have been identified, the reason that HLH occurs in only a subset of individuals ...

journal_title：Blood

authors： Miller PG,Sperling AS,Gibson CJ,Viswanathan K,Castellano C,McConkey M,Ceremsak J,Taylor MS,Birndt S,Perner F,Arnason J,Agrawal M,Schram AM,Nikiforow S,Pihan G,Hasserjian RP,Aster JC,La Rosée P,Morgan EA,Berliner N,

更新日期：2020-12-24 00:00:00

abstract：:Numerous studies have reported significant associations between ABO blood group and risk of cardiovascular disease. These studies have consistently demonstrated that thrombotic risk is significantly reduced in individuals in blood group O. Nevertheless, the biological mechanisms through which ABO influences hemostasis...

journal_title：Blood

authors： Ward SE,O'Sullivan JM,O'Donnell JS

更新日期：2020-12-17 00:00:00

abstract：:Hematopoietic transplantation is the preferred treatment for many patients with hematologic malignancies. Some patients may develop invasive fungal diseases (IFDs) during initial chemotherapy, which need to be considered when assessing patients for transplantation and treatment posttransplantation. Given the associate...

journal_title：Blood

authors： Puerta-Alcalde P,Champlin RE,Kontoyiannis DP

更新日期：2020-12-10 00:00:00

abstract：:Acquired thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterized by thrombotic microangiopathy leading to end-organ damage. The standard of care (SOC) treatment is therapeutic plasma exchange (TPE) alongside immunomodulation with steroids, with increasing use of rituximab +/- other immuno...

journal_title：Blood

authors： Goshua G,Sinha P,Hendrickson JE,Tormey CA,Bendapudi P,Lee AI

更新日期：2020-12-06 00:00:00

abstract：:Digital protein assays have great potential to advance immunodiagnostics because of their single-molecule sensitivity, high precision, and robust measurements. However, translating digital protein assays to acute clinical care has been challenging because it requires their deployment with a rapid turnaround. Herein, w...

journal_title：Blood

authors： Song Y,Sandford E,Tian Y,Yin Q,Kozminski AG,Su SH,Cai T,Ye Y,Chung MT,Lindstrom R,Goicochea A,Barabas J,Olesnavich M,Rozwadowski M,Li Y,Alam HB,Singer BH,Ghosh M,Choi SW,Tewari M,Kurabayashi K

更新日期：2020-12-04 00:00:00

abstract：:Adeno-associated virus (AAV)-based gene therapies can restore endogenous factor VIII (FVIII) expression in hemophilia A (HA). AAV vectors typically use a B-domain-deleted FVIII transgene, such as human FVIII-SQ in valoctocogene roxaparvovec (AAV5-FVIII-SQ). Surprisingly, the activity of transgene-produced FVIII-SQ was...

journal_title：Blood

authors： Rosen S,Tiefenbacher S,Robinson M,Huang M,Srimani J,Mackenzie D,Christianson T,Pasi KJ,Rangarajan S,Symington E,Giermasz A,Pierce GF,Kim B,Zoog SJ,Vettermann C

更新日期：2020-11-26 00:00:00

abstract：:Aberrant megakaryopoiesis is a hallmark of the myeloproliferative neoplasms (MPNs), a group of clonal haematological malignancies originating from haematopoietic stem cells, leading to an increase in mature blood cells in the peripheral blood. Sialylated derivatives of the glycan structure β4-N-acetyllactosamine (Galβ...

journal_title：Blood

authors： Di Buduo CA,Giannini S,Abbonante V,Rosti V,Hoffmeister K,Balduini A

更新日期：2020-11-25 00:00:00

abstract：:The interaction of menin (MEN1) and MLL (MLL1, KMT2A) is a dependency and provides a potential opportunity for treatment of NPM1-mutant (NPM1mut) and MLL-rearranged (MLL-r) leukemias. Concomitant activating driver mutations in the gene encoding the tyrosine kinase FLT3 occur in both leukemias and are particularly comm...

journal_title：Blood

authors： Dzama MM,Steiner M,Rausch J,Sasca D,Schönfeld J,Kunz K,Taubert MC,McGeehan GM,Chen CW,Mupo A,Hähnel P,Theobald M,Kindler T,Koche RP,Vassiliou GS,Armstrong SA,Kühn MWM

更新日期：2020-11-19 00:00:00

abstract：:Primary central nervous system lymphoma (PCNSL) is confined to the brain, eyes, and cerebrospinal fluid without evidence of systemic spread. Rarely, PCNSL occurs in the context of immunosuppression, e.g. post-transplant lymphoproliferative disorders (PTLD) or HIV (AIDS-related PCNSL). These cases are poorly characteri...

journal_title：Blood

authors： Gandhi MK,Hoang T,Law SC,Brosda S,O'Rourke K,Tobin JWD,Vari F,Murigneux V,Fink JL,Gunawardana J,Gould CM,Oey H,Bednarska K,Delecluse S,Trappe RU,de Long LM,Sabdia MB,Bhagat G,Hapgood G,Blyth E,Clancy LE,Wight J

更新日期：2020-11-17 00:00:00

abstract：:Burkitt lymphoma (BL) is a highly aggressive, B-cell, non-Hodgkin lymphoma (NHL) categorized into endemic, sporadic and immunodeficiency-associated subtypes. BL has distinct pathologic and clinical features, characterized by rapidly progressive tumors with high rates of extranodal involvement. Next generation sequenci...

journal_title：Blood

authors： Crombie JL,LaCasce AS

更新日期：2020-11-10 00:00:00

abstract：:Lymphomas afflict all age groups of people, with certain types demonstrating a female predilection in adolescents and young adults. A proportion of lymphomas that are diagnosed in this population demographic occur in the setting of pregnancy. Most of these behave aggressively at presentation and require immediate or u...

journal_title：Blood

authors： Dunleavy K,McLintock C

更新日期：2020-11-05 00:00:00

abstract：:Truncation of asparaginase treatment due to asparaginase related toxicities or silent inactivation (SI) is common and may increase relapse risk in acute lymphoblastic leukemia (ALL). We investigated relapse risk following suboptimal asparaginase exposure among 1401 children aged 1-17 years, diagnosed with ALL between ...

journal_title：Blood

authors： Gottschalk Højfeldt S,Grell K,Abrahamsson J,Lund B,Vettenranta K,Jonsson OG,Frandsen TL,Wolthers BO,Marquart HVH,Vaitkeviciene G,Lepik K,Heyman M,Schmiegelow K,Albertsen BK

更新日期：2020-11-04 00:00:00

abstract：:The terminal differentiation of B cells into antibody-secreting cells (ASCs) is a critical component of adaptive immune responses. However, it is a very sensitive process, which dysfunctions lead to a great variety of lymphoproliferative neoplasia including germinal center-derived lymphomas. To better characterize the...

journal_title：Blood

authors： Pignarre A,Chatonnet F,Caron G,Haas M,Desmots-Loyer F,Fest T

更新日期：2020-11-04 00:00:00

abstract：:Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) that leads to progressive bone marrow (BM) fibrosis. Although the cellular mutations involved in the pathogenesis of PMF have been extensively investigated, the sequential events that drive stromal activation and fibrosis by hematopoietic-stromal cross...

journal_title：Blood

authors： Gleitz HFE,Dugourd AJF,Leimkühler NB,Snoeren IAM,Fuchs SNR,Menzel S,Ziegler S,Kröger N,Triviai I,Büsche G,Kreipe H,Banjanin B,Pritchard JE,Hoogenboezem R,Bindels EM,Schumacher N,Rose-John S,Elf S,Saez-Rodriguez J,Kr

更新日期：2020-10-29 00:00:00

abstract：:Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. HSTCL is more common in adolescent and young adults and has a rapidly progressive clinical course and poor outcome due to its refractoriness to conventional chemo...

journal_title：Blood

authors： Pro B,Allen P,Behdad A

更新日期：2020-10-29 00:00:00

abstract：:Additional sex combs-like 1 (ASXL1), an epigenetic modulator, is frequently mutated in myeloid neoplasms. Recent analyses of mutant ASXL1 conditional knockin (ASXL1-MT-KI) mice suggested that ASXL1-MT alone is insufficient for myeloid transformation. In our previous study, we used retrovirus-mediated insertional mutag...

journal_title：Blood

authors： Takeda R,Asada S,Park SJ,Yokoyama A,Becker HJ,Kanai A,Visconte V,Hershberger C,Hayashi Y,Yonezawa T,Tamura M,Fukushima T,Tanaka Y,Fukuyama T,Matsumoto A,Yamasaki S,Nakai K,Yamazaki S,Inaba T,Shibata T,Inoue D,Ho

更新日期：2020-10-01 00:00:00

abstract：:Anaplastic large cell lymphoma (ALCL) is a T-cell malignancy predominantly driven by a hyperactive anaplastic lymphoma kinase (ALK) fusion protein. ALK inhibitors, such as crizotinib, provide alternatives to standard chemotherapy with reduced toxicity and side effects. Children with lymphomas driven by nucleophosmin 1...

journal_title：Blood

authors： Prokoph N,Probst NA,Lee LC,Monahan JM,Matthews JD,Liang HC,Bahnsen K,Montes-Mojarro IA,Karaca-Atabay E,Sharma GG,Malik V,Larose H,Forde SD,Ducray SP,Lobello C,Wang Q,Luan SL,Pospíšilová Š,Gambacorti-Passerini C,Burk

更新日期：2020-10-01 00:00:00

abstract：:Heme is an essential cofactor for numerous cellular functions, but release of free heme during hemolysis results in oxidative tissue damage, vascular dysfunction, and inflammation. Macrophages play a key protective role in heme clearance; however, the mechanisms that regulate metabolic adaptations that are required fo...

journal_title：Blood

authors： Bories GFP,Yeudall S,Serbulea V,Fox TE,Isakson BE,Leitinger N

更新日期：2020-09-24 00:00:00

abstract：:The nuclear receptors (NR) retinoid X receptors (RXRs) exert immunomodulatory functions to control inflammation and metabolism via homodimers and heterodimers with several other NRs including retinoic acid receptors. IRX4204 is a novel, highly specific RXR agonist in clinical trials that potently and selectively activ...

journal_title：Blood

authors： Thangavelu G,Wang C,Loschi M,Saha A,Osborn M,Furlan SN,Aoyama K,McDonald-Hyman C,Aguilar EG,Janesick AS,Chandraratna RA,Refaeli Y,Panoskaltsis-Mortari A,MacDonald KP,Hill GR,Zeiser R,Maillard I,Serody J,Murphy WJ,Mu

更新日期：2020-09-24 00:00:00

abstract：:The initiation and progression of diffuse large B-cell lymphoma (DLBCL) is governed by genetic and epigenetic aberrations. As the most abundant eukaryotic message RNA modification, N6-methyladenosine (m6A) is known to influence various fundamental bioprocesses by regulating target gene; however, the function of m6A mo...

journal_title：Blood

authors： Han H,Fan G,Song S,Jiang Y,Qian C,Zhang W,Su Q,Xue X,Zhuang W,Li B

更新日期：2020-09-23 00:00:00

abstract：:Mantle cell lymphoma (MCL) is a mature B-cell neoplasm initially driven by CCND1 rearrangement with 2 molecular subtypes, conventional MCL (cMCL) and leukemic non-nodal MCL (nnMCL), that differ in their clinicobiological behavior. To identify the genetic and epigenetic alterations determining this diversity, we used w...

journal_title：Blood

authors： Nadeu F,Martin-Garcia D,Clot G,Díaz-Navarro A,Duran-Ferrer M,Navarro A,Vilarrasa-Blasi R,Kulis M,Royo R,Gutiérrez-Abril J,Valdés-Mas R,López C,Chapaprieta V,Puiggros M,Castellano G,Costa D,Aymerich M,Jares P,Espinet B

更新日期：2020-09-17 00:00:00

abstract：:The cells and mechanisms involved in blood clot resorption are only partially known. We show that regulatory T (Treg) cells accumulate in venous blood clots and regulate thrombolysis by controlling the recruitment, differentiation and matrix metalloproteinase (MMP) activity of monocytes. We describe a clot Treg popula...

journal_title：Blood

authors： Shahneh F,Alexandra G,Klein M,Frauhammer F,Bopp T,Schäfer K,Raker V,Becker C

更新日期：2020-09-15 00:00:00

abstract：:T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive malignancy that accounts for ∼20% of ALL cases. Intensive chemotherapy regimens result in cure rates >85% in children and <50% in adults, warranting a search of novel therapeutic strategies. Although immune-based therapies have tremendously improved the trea...

journal_title：Blood

authors： Tran Quang C,Zaniboni B,Humeau R,Lengliné E,Dourthe ME,Ganesan R,Singh S,Scheer JM,Asnafi V,Ghysdael J

更新日期：2020-09-10 00:00:00

abstract：:Congenital dyserythropoietic anemias (CDAs) are a heterogeneous group of inherited anemias that affect the normal differentiation-proliferation pathways of the erythroid lineage. They belong to the wide group of ineffective erythropoiesis conditions that mainly result in monolinear cytopenia. CDAs are classified into ...

journal_title：Blood

authors： Iolascon A,Andolfo I,Russo R

更新日期：2020-09-10 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：:Gastrointestinal (GI) bleeding is distinctive of severe von Willebrand disease (VWD), generally arising in older patients; in most cases, blood transfusion and hospitalization are required. The presence of arteriovenous malformations is often described when endoscopic examinations are performed. Patients with congenit...

journal_title：Blood

authors： Biguzzi E,Siboni SM,Peyvandi F

更新日期：2020-09-03 00:00:00

abstract：:Recombinant erythropoietin (EPO) and iron substitution are a standard of care for treatment of anemias associated with chronic inflammation, including anemia of chronic kidney disease. A black box warning for EPO therapy and concerns about negative side effects related to high-dose iron supplementation as well as the ...

journal_title：Blood

authors： Petzer V,Tymoszuk P,Asshoff M,Carvalho J,Papworth J,Deantonio C,Bayliss L,Wake MS,Seifert M,Brigo N,Valente de Souza L,Hilbe R,Grubwieser P,Demetz E,Dichtl S,Volani C,Berger S,Böhm F,Hoffmann A,Pfeifhofer-Obermair C

更新日期：2020-08-27 00:00:00

abstract：:Lenalidomide, bortezomib, and dexamethasone (RVd) followed by autologous stem cell transplantation (ASCT) is standard frontline therapy for transplant-eligible patients with newly diagnosed multiple myeloma (NDMM). The addition of daratumumab (D) to RVd (D-RVd) in transplant-eligible NDMM patients was evaluated. Patie...

journal_title：Blood

authors： Voorhees PM,Kaufman JL,Laubach J,Sborov DW,Reeves B,Rodriguez C,Chari A,Silbermann R,Costa LJ,Anderson LD Jr,Nathwani N,Shah N,Efebera YA,Holstein SA,Costello C,Jakubowiak A,Wildes TM,Orlowski RZ,Shain KH,Cowan AJ,

更新日期：2020-08-20 00:00:00

abstract：:Despite increasing use of targeted therapies to treat cancer, anemia remains a common complication of cancer therapy. Physician concerns about the safety of intravenous (IV) iron products and erythropoiesis-stimulating agents (ESAs) have resulted in many patients with cancer receiving no or suboptimal anemia therapy. ...

journal_title：Blood

authors： Gilreath JA,Rodgers GM

更新日期：2020-08-13 00:00:00

abstract：:Acute erythroleukemia (AEL or acute myeloid leukemia [AML]-M6) is a rare but aggressive hematologic malignancy. Previous studies showed that AEL leukemic cells often carry complex karyotypes and mutations in known AML-associated oncogenes. To better define the underlying molecular mechanisms driving the erythroid phen...

journal_title：Blood

authors： Fagnan A,Bagger FO,Piqué-Borràs MR,Ignacimouttou C,Caulier A,Lopez CK,Robert E,Uzan B,Gelsi-Boyer V,Aid Z,Thirant C,Moll U,Tauchmann S,Kurtovic-Kozaric A,Maciejewski J,Dierks C,Spinelli O,Salmoiraghi S,Pabst T,Shimo

更新日期：2020-08-06 00:00:00

abstract：:Several studies demonstrate that hemolysis and free heme in circulation cause endothelial barrier dysfunction and are associated with severe pathological conditions such as acute respiratory distress syndrome, acute chest syndrome, and sepsis. However, the precise molecular mechanisms involved in the pathology of heme...

journal_title：Blood

authors： James J,Srivastava A,Varghese MV,Eccles CA,Zemskova M,Rafikova O,Rafikov R

更新日期：2020-08-06 00:00:00

abstract：:Fanconi anemia (FA) is a hereditary disorder caused by mutations in any 1 of 22 FA genes. The disease is characterized by hypersensitivity to interstrand crosslink (ICL) inducers such as mitomycin C (MMC). In addition to promoting ICL repair, FA proteins such as RAD51, BRCA2, or FANCD2 protect stalled replication fork...

journal_title：Blood

authors： Okamoto Y,Abe M,Mu A,Tempaku Y,Rogers CB,Mochizuki AL,Katsuki Y,Kanemaki MT,Takaori-Kondo A,Sobeck AT,Bielinsky AK,Takata M

更新日期：2020-07-31 00:00:00

abstract：:We developed a risk score to predict event-free survival (EFS) after allogeneic hematopoietic cell transplantation for sickle cell disease. The study population (n = 1425) was randomly split into training (n = 1070) and validation (n = 355) cohorts. Risk factors were identified and validated via Cox regression models....

journal_title：Blood

authors： Brazauskas R,Scigliuolo GM,Wang HL,Cappelli B,Ruggeri A,Fitzhugh CD,Hankins JS,Kanter J,Meerpohl JJ,Panepinto JA,Rondelli D,Shenoy S,Walters MC,Wagner JE,Tisdale JF,Gluckman E,Eapen M

更新日期：2020-07-30 00:00:00

abstract：:The success of allogeneic hematopoietic cell transplantation depends heavily on the delicate balance between the activity of the donor immune system against malignant and nonmalignant cells of the recipient. Abrogation of alloreactivity will lead to disease relapse, whereas untamed allo-immune responses will lead to l...

journal_title：Blood

authors： Voermans C,Hazenberg MD

更新日期：2020-07-23 00:00:00

abstract：:The oxygen transport function of hemoglobin (HB) is thought to have arisen ∼500 million years ago, roughly coinciding with the divergence between jawless (Agnatha) and jawed (Gnathostomata) vertebrates. Intriguingly, extant HBs of jawless and jawed vertebrates were shown to have evolved twice, and independently, from ...

journal_title：Blood

authors： Miyata M,Gillemans N,Hockman D,Demmers JAA,Cheng JF,Hou J,Salminen M,Fisher CA,Taylor S,Gibbons RJ,Ganis JJ,Zon LI,Grosveld F,Mulugeta E,Sauka-Spengler T,Higgs DR,Philipsen S

更新日期：2020-07-16 00:00:00