abstract：:Autoimmune lymphoproliferative syndrome (ALPS) caused by impaired FAS-mediated apoptosis of lymphocytes is characterized by lymphoproliferation, autoimmunity, but also an increased risk of invasive bacterial infection, notably following splenectomy. We surveyed a cohort of 100 ALPS patients (including 33 splenectomize...

journal_title：Blood

authors： Neven B,Bruneau J,Stolzenberg MC,Meyts I,Magerus-Chatinet A,Moens L,Lanzarotti N,Weller S,Amiranoff D,Florkin B,Bader-Meunier B,Leverger G,Ferster A,Chantrain C,Blanche S,Picard C,Molina TJ,Brousse N,Durandy A,Rizzi

更新日期：2014-09-04 00:00:00

abstract：:Peripheral T-cell lymphomas (PTCLs) are rare lymphomas with mostly poor outcome with current treatment. The addition of etoposide to cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and upfront consolidation with autologous stem cell transplantation (auto-SCT) have shown promising results but have nev...

journal_title：Blood

authors： Ellin F,Landström J,Jerkeman M,Relander T

更新日期：2014-09-04 00:00:00

abstract：:Anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL) is a CD30-positive T-cell non-Hodgkin lymphoma that morphologically resembles ALK-positive ALCL but lacks chromosomal rearrangements of the ALK gene. The genetic and clinical heterogeneity of ALK-negative ALCL has not been delineated. We p...

journal_title：Blood

authors： Parrilla Castellar ER,Jaffe ES,Said JW,Swerdlow SH,Ketterling RP,Knudson RA,Sidhu JS,Hsi ED,Karikehalli S,Jiang L,Vasmatzis G,Gibson SE,Ondrejka S,Nicolae A,Grogg KL,Allmer C,Ristow KM,Wilson WH,Macon WR,Law ME,Ce

更新日期：2014-08-28 00:00:00

abstract：:Loss-of-function mutation in the heme oxygenase 1 (Hmox1) gene causes a rare and lethal disease in children, characterized by severe anemia and intravascular hemolysis, with damage to endothelia and kidneys. Previously, we found that macrophages engaged in recycling of red cells were depleted from the tissues of Hmox1...

journal_title：Blood

authors： Kovtunovych G,Ghosh MC,Ollivierre W,Weitzel RP,Eckhaus MA,Tisdale JF,Yachie A,Rouault TA

更新日期：2014-08-28 00:00:00

abstract：:Treatment outcomes for older patients with acute myeloid leukemia (AML) have remained dismal. This randomized, phase 2 trial in AML patients not considered suitable for intensive induction therapy compared low-dose cytarabine (LDAC) with or without volasertib, a highly potent and selective inhibitor of polo-like kinas...

journal_title：Blood

authors： Döhner H,Lübbert M,Fiedler W,Fouillard L,Haaland A,Brandwein JM,Lepretre S,Reman O,Turlure P,Ottmann OG,Müller-Tidow C,Krämer A,Raffoux E,Döhner K,Schlenk RF,Voss F,Taube T,Fritsch H,Maertens J

更新日期：2014-08-28 00:00:00

abstract：:Hematopoietic stem cell (HSC)-based gene therapy holds promise for the cure of many diseases. The field is now moving toward the use of lentiviral vectors (LVs) as evidenced by 4 successful clinical trials. These trials used vesicular-stomatitis-virus-G protein (VSV-G)-LVs at high doses combined with strong cytokine-c...

journal_title：Blood

authors： Girard-Gagnepain A,Amirache F,Costa C,Lévy C,Frecha C,Fusil F,Nègre D,Lavillette D,Cosset FL,Verhoeyen E

更新日期：2014-08-21 00:00:00

abstract：:We studied the impact of driver mutations of JAK2, CALR, (calreticulin gene) or MPL on clinical course, leukemic transformation, and survival of patients with primary myelofibrosis (PMF). Of the 617 subjects studied, 399 (64.7%) carried JAK2 (V617F), 140 (22.7%) had a CALR exon 9 indel, 25 (4.0%) carried an MPL (W515)...

journal_title：Blood

authors： Rumi E,Pietra D,Pascutto C,Guglielmelli P,Martínez-Trillos A,Casetti I,Colomer D,Pieri L,Pratcorona M,Rotunno G,Sant'Antonio E,Bellini M,Cavalloni C,Mannarelli C,Milanesi C,Boveri E,Ferretti V,Astori C,Rosti V,Cerva

更新日期：2014-08-14 00:00:00

abstract：:Proteasome inhibition is an effective treatment strategy for multiple myeloma. With improving survival, attention is increasingly focusing on ease of administration and toxicity profile. Ixazomib is an investigational, orally bioavailable 20S proteasome inhibitor. Sixty patients with relapsed and/or refractory multipl...

journal_title：Blood

authors： Kumar SK,Bensinger WI,Zimmerman TM,Reeder CB,Berenson JR,Berg D,Hui AM,Gupta N,Di Bacco A,Yu J,Shou Y,Niesvizky R

更新日期：2014-08-14 00:00:00

abstract：:An estimated 22 350 patients had multiple myeloma diagnosed in 2013, representing 1.3% of all new cancers; 10 710 deaths are projected, representing 1.8% of cancer deaths. Approximately 0.7% of US men and women will have a myeloma diagnosis in their lifetime, and with advances in therapy, 77 600 US patients are living...

journal_title：Blood

authors： Gertz MA,Dingli D

更新日期：2014-08-07 00:00:00

abstract：:Myeloid-derived suppressor cells (MDSCs) represent a heterogeneous population that shares certain characteristics including an aberrant myeloid phenotype and the ability to suppress T cells. MDSCs have been predominantly studied in malignant diseases and findings suggest involvement in tumor-associated immune suppress...

journal_title：Blood

authors： Jitschin R,Braun M,Büttner M,Dettmer-Wilde K,Bricks J,Berger J,Eckart MJ,Krause SW,Oefner PJ,Le Blanc K,Mackensen A,Mougiakakos D

更新日期：2014-07-31 00:00:00

abstract：:In this issue of Blood, Lu et al describe the cooperation between an orally bioavailable mouse double minute 2 (MDM2) antagonist (RG7112) and the pegylated interferon α (Peg-IFNα 2a) to target JAK2V617F hematopoietic progenitors and stem cells. Their work provides a rationale for the treatment of patients suffering fr...

journal_title：Blood

authors： Plo I

更新日期：2014-07-31 00:00:00

abstract：:Viral infections have been associated with reduced platelet counts, the biological significance of which has remained elusive. Here, we show that infection with encephalomyocarditis virus (EMCV) rapidly reduces platelet count, and this response is attributed to platelet Toll-like receptor 7 (TLR7). Platelet-TLR7 stimu...

journal_title：Blood

authors： Koupenova M,Vitseva O,MacKay CR,Beaulieu LM,Benjamin EJ,Mick E,Kurt-Jones EA,Ravid K,Freedman JE

更新日期：2014-07-31 00:00:00

abstract：:The phagocytic function of macrophages plays a pivotal role in eliminating apoptotic cells and invading pathogens. Evidence implicating plasminogen (Plg), the zymogen of plasmin, in phagocytosis is extremely limited with the most recent in vitro study showing that plasmin acts on prey cells rather than on macrophages....

journal_title：Blood

authors： Das R,Ganapathy S,Settle M,Plow EF

更新日期：2014-07-31 00:00:00

abstract：:Allogeneic hematopoietic stem cell transplantation (HSCT) is widely used to treat hematopoietic cell disorders but is often complicated by graft-versus-host disease (GVHD), which causes severe epithelial damage. Here we have investigated longitudinally the effects of induction chemotherapy, conditioning radiochemother...

journal_title：Blood

authors： Munneke JM,Björklund AT,Mjösberg JM,Garming-Legert K,Bernink JH,Blom B,Huisman C,van Oers MH,Spits H,Malmberg KJ,Hazenberg MD

更新日期：2014-07-31 00:00:00

abstract：:The interplay between bone marrow stromal cells (BMSCs) and multiple myeloma (MM) cells performs a crucial role in MM pathogenesis by secreting growth factors, cytokines, and extracellular vesicles. Exosomes are membranous vesicles 40 to 100 nm in diameter constitutively released by almost all cell types, and they med...

journal_title：Blood

authors： Wang J,Hendrix A,Hernot S,Lemaire M,De Bruyne E,Van Valckenborgh E,Lahoutte T,De Wever O,Vanderkerken K,Menu E

更新日期：2014-07-24 00:00:00

abstract：:Signal transducer and activator of transcription 3 (STAT3) is considered a negative regulator of inflammation, as inhibition of STAT3 signaling enhances antitumor immunity. However, STAT3 activation is a key oncogenic pathway in natural killer (NK)-lineage large granular lymphomas, and we recently reported enhanced pr...

journal_title：Blood

authors： Zhu S,Phatarpekar PV,Denman CJ,Senyukov VV,Somanchi SS,Nguyen-Jackson HT,Mace EM,Freeman AF,Watowich SS,Orange JS,Holland SM,Lee DA

更新日期：2014-07-17 00:00:00

abstract：:Over the past 5 years, many novel approaches to early diagnosis, prevention, and treatment of acute graft-versus-host disease (aGVHD) have been translated from the bench to the bedside. In this review, we highlight recent discoveries in the context of current aGVHD care. The most significant innovations that have alre...

journal_title：Blood

authors： Holtan SG,Pasquini M,Weisdorf DJ

更新日期：2014-07-17 00:00:00

abstract：:Pregnancy can precipitate thrombotic thrombocytopenic purpura (TTP). We present a prospective study of TTP cases from the United Kingdom Thrombotic Thrombocytopenic Purpura (UK TTP) Registry with clinical and laboratory data from the largest cohort of pregnancy-associated TTP and describe management through pregnancy,...

journal_title：Blood

authors： Scully M,Thomas M,Underwood M,Watson H,Langley K,Camilleri RS,Clark A,Creagh D,Rayment R,Mcdonald V,Roy A,Evans G,McGuckin S,Ni Ainle F,Maclean R,Lester W,Nash M,Scott R,O Brien P,collaborators of the UK TTP Registr

更新日期：2014-07-10 00:00:00

abstract：:Pathologic blood clotting is a leading cause of morbidity and mortality in the developed world, underlying deep vein thrombosis, myocardial infarction, and stroke. Genetic predisposition to thrombosis is still poorly understood, and we hypothesize that there are many additional risk alleles and modifying factors remai...

journal_title：Blood

authors： Liu Y,Kretz CA,Maeder ML,Richter CE,Tsao P,Vo AH,Huarng MC,Rode T,Hu Z,Mehra R,Olson ST,Joung JK,Shavit JA

更新日期：2014-07-03 00:00:00

abstract：:The outcome of patients with acute myeloid leukemia who are older than 60 years has remained poor because of unfavorable disease characteristics and patient-related factors. The randomized German-Austrian AML Study Group 06-04 protocol was designed on the basis of in vitro synergistic effects of valproic acid (VPA) an...

journal_title：Blood

authors： Tassara M,Döhner K,Brossart P,Held G,Götze K,Horst HA,Ringhoffer M,Köhne CH,Kremers S,Raghavachar A,Wulf G,Kirchen H,Nachbaur D,Derigs HG,Wattad M,Koller E,Brugger W,Matzdorff A,Greil R,Heil G,Paschka P,Gaidzik

更新日期：2014-06-26 00:00:00

abstract：:Although much of the function of von Willebrand factor (VWF) has been revealed, detailed insight into the molecular structure that enables VWF to orchestrate hemostatic processes, in particular factor VIII (FVIII) binding and stabilization in plasma, is lacking. Here, we present the high-resolution solution structure ...

journal_title：Blood

authors： Shiltagh N,Kirkpatrick J,Cabrita LD,McKinnon TA,Thalassinos K,Tuddenham EG,Hansen DF

更新日期：2014-06-26 00:00:00

abstract：:Protein S is a cofactor for tissue factor pathway inhibitor (TFPI), accelerating the inhibition of activated factor X (FXa). TFPI Kunitz domain 3 residue Glu226 is essential for enhancement of TFPI by protein S. To investigate the complementary functional interaction site on protein S, we screened 44 protein S point, ...

journal_title：Blood

authors： Reglińska-Matveyev N,Andersson HM,Rezende SM,Dahlbäck B,Crawley JT,Lane DA,Ahnström J

更新日期：2014-06-19 00:00:00

abstract：:Chronic graft-versus-host disease (cGVHD) is a leading cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation. Having shown that germinal center (GC) formation and immunoglobulin deposition are required for multiorgan system cGVHD and associated bronchiolitis obliterans syndrome (BOS...

journal_title：Blood

authors： Flynn R,Du J,Veenstra RG,Reichenbach DK,Panoskaltsis-Mortari A,Taylor PA,Freeman GJ,Serody JS,Murphy WJ,Munn DH,Sarantopoulos S,Luznik L,Maillard I,Koreth J,Cutler C,Soiffer RJ,Antin JH,Ritz J,Dubovsky JA,Byrd JC,

更新日期：2014-06-19 00:00:00

abstract：:In this issue of Blood, Bachanova et al describe how modulation of the inhibitory tumor environment may enhance natural killer (NK) cell clinical activity and produce encouraging results in the treatment of refractory acute myeloid leukemia (AML). NK cells are highly proliferative, early responders of the innate immun...

journal_title：Blood

authors： Rooney CM

更新日期：2014-06-19 00:00:00

abstract：:Sickle cell disease (SCD) is characterized by recurring episodes of vascular occlusion in which neutrophil activation plays a major role. The disease is associated with chronic hemolysis with elevated cell-free hemoglobin and heme. The ensuing depletion of heme scavenger proteins leads to nonspecific heme uptake and h...

journal_title：Blood

authors： Chen G,Zhang D,Fuchs TA,Manwani D,Wagner DD,Frenette PS

更新日期：2014-06-12 00:00:00

abstract：:There is growing evidence that lymphocyte trafficking contributes to the clinical course of chronic lymphocytic leukemia (CLL), but to date, only static in vitro cultures have been used to study these phenomena. To address this lack of data, we have developed a dynamic in vitro model in which CLL cells experience shea...

journal_title：Blood

authors： Walsby E,Buggins A,Devereux S,Jones C,Pratt G,Brennan P,Fegan C,Pepper C

更新日期：2014-06-05 00:00:00

abstract：:The existence of spontaneous heparin-induced thrombocytopenia (HIT) syndrome (or autoimmune HIT), defined as a transient prothrombotic thrombocytopenic disorder without proximate heparin exposure serologically indistinguishable from HIT, is controversial. We describe 2 new cases presenting with thrombotic stroke/throm...

journal_title：Blood

authors： Warkentin TE,Basciano PA,Knopman J,Bernstein RA

更新日期：2014-06-05 00:00:00

abstract：:Hepcidin is a 25-amino-acid peptide demonstrated to be the iron regulatory hormone capable of blocking iron absorption from the duodenum and iron release from macrophages. Mutations affecting hepcidin regulators or the hepcidin gene itself cause hemochromatosis, a common genetic disorder. Hepcidin is produced mainly b...

journal_title：Blood

authors： Zumerle S,Mathieu JR,Delga S,Heinis M,Viatte L,Vaulont S,Peyssonnaux C

更新日期：2014-06-05 00:00:00

abstract：:Activation of sialic-acid-binding immunoglobulin-like lectin-G (Siglec-G) by noninfectious damage-associated molecular patterns controls innate immune responses. However, whether it also regulates T-cell-mediated adaptive immune responses is not known. Graft-versus-host reaction is a robust adaptive immune response ca...

journal_title：Blood

authors： Toubai T,Hou G,Mathewson N,Liu C,Wang Y,Oravecz-Wilson K,Cummings E,Rossi C,Evers R,Sun Y,Wu J,Choi SW,Fang D,Zheng P,Liu Y,Reddy P

更新日期：2014-05-29 00:00:00

abstract：:In systemic light-chain amyloidosis, λ light chains produced by clonal plasma cells cause organ damage and early death. In pursuit of novel therapy, we developed 1 pool of short interfering RNA (siRNA) targeting the constant region of λ light chains that substantially and promptly reduces λ-light-chain production and ...

journal_title：Blood

authors： Zhou P,Ma X,Iyer L,Chaulagain C,Comenzo RL

更新日期：2014-05-29 00:00:00

abstract：:C-abl oncogene 1, nonreceptor tyrosine kinase (ABL1) kinase inhibitors such as imatinib mesylate (imatinib) are effective in managing chronic myeloid leukemia (CML) but incapable of eliminating leukemia stem cells (LSCs), suggesting that kinase-independent pathways support LSC survival. Given that the bone marrow (BM)...

journal_title：Blood

authors： Ng KP,Manjeri A,Lee KL,Huang W,Tan SY,Chuah CT,Poellinger L,Ong ST

更新日期：2014-05-22 00:00:00

abstract：:In this issue of Blood, Kasirer-Friede and colleagues show that the adhesion and degranulation promoter protein (ADAP) promotes αIIbβ3 activation by presenting the cytoplasmic proteins talin and kindlin to the β3 cytoplasmic tail. ...

journal_title：Blood

authors： Bennett JS

更新日期：2014-05-15 00:00:00

abstract：:The corepressor Rcor1 has been linked biochemically to hematopoiesis, but its function in vivo remains unknown. We show that mice deleted for Rcor1 are profoundly anemic and die in late gestation. Definitive erythroid cells from mutant mice arrest at the transition from proerythroblast to basophilic erythroblast. Rema...

journal_title：Blood

authors： Yao H,Goldman DC,Nechiporuk T,Kawane S,McWeeney SK,Tyner JW,Fan G,Kerenyi MA,Orkin SH,Fleming WH,Mandel G

更新日期：2014-05-15 00:00:00

abstract：:Serum response factor (SRF) is a ubiquitously expressed transcription factor and master regulator of the actin cytoskeleton. We have previously shown that SRF is essential for megakaryocyte maturation and platelet formation and function. Here we elucidate the role of SRF in neutrophils, the primary defense against inf...

journal_title：Blood

authors： Taylor A,Tang W,Bruscia EM,Zhang PX,Lin A,Gaines P,Wu D,Halene S

更新日期：2014-05-08 00:00:00

abstract：:Richter syndrome (RS) is associated with poor outcome. The prognosis of patients with histologically aggressive chronic lymphocytic leukemia (CLL), or HAC, has not been studied. We aimed to correlate 2-deoxy-2-[(18)F]fluoroglucose/positron emission tomography (FDG/PET) data, histological diagnosis, clinical characteri...

journal_title：Blood

authors： Falchi L,Keating MJ,Marom EM,Truong MT,Schlette EJ,Sargent RL,Trinh L,Wang X,Smith SC,Jain N,Estrov Z,O'Brien S,Wierda WG,Lerner S,Ferrajoli A

更新日期：2014-05-01 00:00:00

abstract：:The interaction between platelet receptor glycoprotein Ibα and the A1 domain of von Willebrand factor (VWF) mediates tethering/translocation of platelets to sites of vascular injury. Unexpectedly, we observed platelets translocating over A1A2A3 domains protein slower than on A1 domain at high shear stress. This observ...

journal_title：Blood

authors： Da Q,Behymer M,Correa JI,Vijayan KV,Cruz MA

更新日期：2014-04-24 00:00:00

abstract：:There is emerging evidence that neutrophil extracellular traps (NETs) play important roles in inflammatory processes. Here we report that neutrophils have to be simultaneously activated by integrin-mediated outside-in- and G-protein-coupled receptor (GPCR) signaling to induce NET formation in acute lung injury (ALI), ...

journal_title：Blood

authors： Rossaint J,Herter JM,Van Aken H,Napirei M,Döring Y,Weber C,Soehnlein O,Zarbock A

更新日期：2014-04-17 00:00:00

abstract：:Monoclonal gammopathy of undetermined significance (MGUS) is present in ∼2% of individuals age >50 years. The increased risk of multiple myeloma (MM) in relatives of individuals with MGUS is consistent with MGUS being a marker of inherited genetic susceptibility to MM. Common single-nucleotide polymorphisms (SNPs) at ...

journal_title：Blood

authors： Weinhold N,Johnson DC,Rawstron AC,Försti A,Doughty C,Vijayakrishnan J,Broderick P,Dahir NB,Begum DB,Hosking FJ,Yong K,Walker BA,Hoffmann P,Mühleisen TW,Langer C,Dörner E,Jöckel KH,Eisele L,Nöthen MM,Hose D,Davies

更新日期：2014-04-17 00:00:00

abstract：:Nucleophosmin-mutated acute myeloid leukemia (NPM1mut-AML) patients have a high rate of complete remission (CR) to induction chemotherapy. However, the mechanisms responsible for such effects are unknown. Because miR-10 family members are expressed at high levels in NPM1mut-AML, we evaluated whether these microRNAs co...

journal_title：Blood

authors： Havelange V,Ranganathan P,Geyer S,Nicolet D,Huang X,Yu X,Volinia S,Kornblau SM,Andreeff M,Croce CM,Marcucci G,Bloomfield CD,Garzon R

更新日期：2014-04-10 00:00:00

abstract：:Myeloproliferative neoplasms (MPNs) are a group of clonal disorders characterized by aberrant hematopoietic proliferation and an increased tendency toward leukemic transformation. We used targeted next-generation sequencing (NGS) of 104 genes to detect somatic mutations in a cohort of 197 MPN patients and followed clo...

journal_title：Blood

authors： Lundberg P,Karow A,Nienhold R,Looser R,Hao-Shen H,Nissen I,Girsberger S,Lehmann T,Passweg J,Stern M,Beisel C,Kralovics R,Skoda RC

更新日期：2014-04-03 00:00:00