abstract：:Classical hairy cell leukemia (cHCL) is characterized by a near 100% frequency of the BRAFV600E mutation, whereas ∼30% of variant HCLs (vHCLs) have MAP2K1 mutations. However, recurrent genetic alterations cooperating with BRAFV600E or MAP2K1 mutations in HCL, as well as those in MAP2K1 wild-type vHCL, are not well def...

journal_title：Blood

authors： Durham BH,Getta B,Dietrich S,Taylor J,Won H,Bogenberger JM,Scott S,Kim E,Chung YR,Chung SS,Hüllein J,Walther T,Wang L,Lu SX,Oakes CC,Tibes R,Haferlach T,Taylor BS,Tallman MS,Berger MF,Park JH,Zenz T,Abdel-Waha

更新日期：2017-10-05 00:00:00

abstract：:Recent studies suggest that the evolutionary history of a cancer is important in forecasting clinical outlook. To gain insight into the clonal dynamics of multiple myeloma (MM) and its possible influence on patient outcomes, we analyzed whole exome sequencing tumor data for 333 patients from Myeloma XI, a UK phase 3 t...

journal_title：Blood

authors： Johnson DC,Lenive O,Mitchell J,Jackson G,Owen R,Drayson M,Cook G,Jones JR,Pawlyn C,Davies FE,Walker BA,Wardell C,Gregory WM,Cairns D,Morgan GJ,Houlston RS,Kaiser MF

更新日期：2017-10-05 00:00:00

abstract：:Integrin αIIbβ3 is a highly abundant heterodimeric platelet receptor that can transmit information bidirectionally across the plasma membrane, and plays a critical role in hemostasis and thrombosis. Upon platelet activation, inside-out signaling pathways increase the affinity of αIIbβ3 for fibrinogen and other ligands...

journal_title：Blood

authors： Durrant TN,van den Bosch MT,Hers I

更新日期：2017-10-05 00:00:00

abstract：:Hypomethylating agents (HMAs) improve survival in patients with higher-risk myelodysplastic syndromes (MDS) but are less well-studied in lower-risk disease. We compared the safety and efficacy of low-dose decitabine vs low-dose azacitidine in this group of patients. Adults with low- or intermediate 1-risk MDS or MDS/m...

journal_title：Blood

authors： Jabbour E,Short NJ,Montalban-Bravo G,Huang X,Bueso-Ramos C,Qiao W,Yang H,Zhao C,Kadia T,Borthakur G,Pemmaraju N,Sasaki K,Estrov Z,Cortes J,Ravandi F,Alvarado Y,Komrokji R,Sekeres MA,Steensma DP,DeZern A,Roboz G,

更新日期：2017-09-28 00:00:00

abstract：:We investigated the effect of platelets on ovarian cancer and the role of adenosine diphosphate (ADP) receptors (P2Y12 and P2Y1) on platelets in the growth of primary ovarian cancer tumors. We showed that in murine models of ovarian cancer, a P2Y12 inhibitor (ticagrelor) reduced tumor growth by 60% compared with aspir...

journal_title：Blood

authors： Cho MS,Noh K,Haemmerle M,Li D,Park H,Hu Q,Hisamatsu T,Mitamura T,Mak SLC,Kunapuli S,Ma Q,Sood AK,Afshar-Kharghan V

更新日期：2017-09-07 00:00:00

abstract：:Selinexor is the first oral selective inhibitor of nuclear export compound tested for cancer treatment. Selinexor has demonstrated a safety therapy profile with broad antitumor activity against solid and hematological malignancies in phases 2 and 3 clinical trials (#NCT03071276, #NCT02343042, #NCT02227251, #NCT0311056...

journal_title：Blood

authors： Machlus KR,Wu SK,Vijey P,Soussou TS,Liu ZJ,Shacham E,Unger TJ,Kashyap T,Klebanov B,Sola-Visner M,Crochiere M,Italiano JE Jr,Landesman Y

更新日期：2017-08-31 00:00:00

abstract：:The B-cell leukemia/lymphoma-2 (BCL-2) family of proteins governs the intrinsic pathway of mitochondrial apoptosis. Dysregulation of BCL-2 has long been known to be a crucial part of the pathophysiology of B-cell lymphomas; however, several early attempts to target this pathway therapeutically were unsuccessful becaus...

journal_title：Blood

authors： Davids MS

更新日期：2017-08-31 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：:Age-associated clonal hematopoiesis caused by acquired mutations in myeloid cancer-associated genes is highly prevalent in the normal population. Its etiology, biological impact on hematopoiesis, and oncogenic risk is poorly defined at this time. To gain insight into this phenomenon, we analyzed a cohort of 2530 relat...

journal_title：Blood

authors： Buscarlet M,Provost S,Zada YF,Barhdadi A,Bourgoin V,Lépine G,Mollica L,Szuber N,Dubé MP,Busque L

更新日期：2017-08-10 00:00:00

abstract：:Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm associated with somatic mutations in the genes involved in the RAF/MEK/extracellular signal-regulated kinase (ERK) signaling pathway. Recently, oncogenic mutations in NRAS/KRAS, upstream regulators of the RAF/MEK/ERK pathway, have been reported in pulm...

journal_title：Blood

authors： Kamata T,Giblett S,Pritchard C

更新日期：2017-07-27 00:00:00

abstract：:As our technological capacities improve, genomic testing is increasingly integrating into patient care. The field of clinical hematology is no exception. Genomic testing carries great promise, but several ethical issues must be considered whenever such testing is performed. This review addresses these ethical consider...

journal_title：Blood

authors： Marron JM,Joffe S

更新日期：2017-07-27 00:00:00

abstract：:RUNX1 is crucial for the regulation of megakaryocyte specification, maturation, and thrombopoiesis. Runx1 possesses 2 promoters: the distal P1 and proximal P2 promoters. The major protein isoforms generated by P1 and P2 are RUNX1C and RUNX1B, respectively, which differ solely in their N-terminal amino acid sequences. ...

journal_title：Blood

authors： Draper JE,Sroczynska P,Leong HS,Fadlullah MZH,Miller C,Kouskoff V,Lacaud G

更新日期：2017-07-20 00:00:00

abstract：:Pediatric-type follicular lymphoma (PTFL) is a B-cell lymphoma with distinctive clinicopathological features. Recently, recurrent genetic alterations of potential importance for its pathogenesis that disrupt pathways associated with the germinal center reaction (TNFRSF14, IRF8), immune escape (TNFRSF14), and anti-apop...

journal_title：Blood

authors： Schmidt J,Ramis-Zaldivar JE,Nadeu F,Gonzalez-Farre B,Navarro A,Egan C,Montes-Mojarro IA,Marafioti T,Cabeçadas J,van der Walt J,Dojcinov S,Rosenwald A,Ott G,Bonzheim I,Fend F,Campo E,Jaffe ES,Salaverria I,Quintanilla-M

更新日期：2017-07-20 00:00:00

abstract：:Transformation to aggressive lymphoma is a critical event in the clinical course of follicular lymphoma (FL) patients. Yet, it is a challenge to reliably predict transformation at the time of diagnosis. Understanding the risk of transformation would be useful for guiding and monitoring patients, as well as for evaluat...

journal_title：Blood

authors： Kridel R,Sehn LH,Gascoyne RD

更新日期：2017-07-20 00:00:00

abstract：:Since its approval in 2011, the Janus kinase 1/2 (JAK1/2) inhibitor ruxolitinib has evolved to become the centerpiece of therapy for myelofibrosis (MF), and its use in patients with hydroxyurea resistant or intolerant polycythemia vera (PV) is steadily increasing. Several other JAK2 inhibitors have entered clinical te...

journal_title：Blood

authors： Bose P,Verstovsek S

更新日期：2017-07-13 00:00:00

abstract：:Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are rare histiocytic disorders induced by somatic mutation of MAPK pathway genes. BRAFV600E mutation is the most common mutation in both conditions and also occurs in the hematopoietic neoplasm hairy cell leukemia (HCL). It is not known if adult LCH...

journal_title：Blood

authors： Milne P,Bigley V,Bacon CM,Néel A,McGovern N,Bomken S,Haniffa M,Diamond EL,Durham BH,Visser J,Hunt D,Gunawardena H,Macheta M,McClain KL,Allen C,Abdel-Wahab O,Collin M

更新日期：2017-07-13 00:00:00

abstract：:Deep venous thrombosis (DVT) remains a common and serious cardiovascular problem with both fatal and long-term consequences. The consequences of DVT include the development of postthrombotic syndrome in 25% to 60% of DVT patients. Despite the clinical importance of venous thrombus resolution, the cellular and molecula...

journal_title：Blood

authors： Mukhopadhyay S,Antalis TM,Nguyen KP,Hoofnagle MH,Sarkar R

更新日期：2017-06-15 00:00:00

abstract：:FLT3-ITD+ acute myeloid leukemia (AML) accounts for ∼25% of all AML cases and is a subtype that carries a poor prognosis. microRNA-155 (miR-155) is specifically overexpressed in FLT3-ITD+ AML compared with FLT3 wild-type (FLT3-WT) AML and is critical for the growth of FLT3-ITD+ AML cells in vitro. However, miR-155's r...

journal_title：Blood

authors： Wallace JA,Kagele DA,Eiring AM,Kim CN,Hu R,Runtsch MC,Alexander M,Huffaker TB,Lee SH,Patel AB,Mosbruger TL,Voth WP,Rao DS,Miles RR,Round JL,Deininger MW,O'Connell RM

更新日期：2017-06-08 00:00:00

abstract：:Healthy blood neutrophils are functionally quiescent in the bloodstream, have a short lifespan, and exit the circulation to carry out innate immune functions, or undergo rapid apoptosis and macrophage-mediated clearance to mitigate host tissue damage. Limitation of unnecessary intravascular neutrophil activation is al...

journal_title：Blood

authors： Lizcano A,Secundino I,Döhrmann S,Corriden R,Rohena C,Diaz S,Ghosh P,Deng L,Nizet V,Varki A

更新日期：2017-06-08 00:00:00

abstract：:Polymorphonuclear neutrophils (PMNs) have previously been reported to mediate phagocytosis of anti-CD20-opsonized B cells from patients with chronic lymphocytic leukemia (CLL). However, recent data have suggested that PMNs, like macrophages, can also mediate trogocytosis. We have performed experiments to more precisel...

journal_title：Blood

authors： Valgardsdottir R,Cattaneo I,Klein C,Introna M,Figliuzzi M,Golay J

更新日期：2017-05-11 00:00:00

abstract：:Mitochondrial DNA (mtDNA) biosynthesis requires replication factors and adequate nucleotide pools from the mitochondria and cytoplasm. We performed gene expression profiling analysis of 542 human acute myeloid leukemia (AML) samples and identified 55% with upregulated mtDNA biosynthesis pathway expression compared wit...

journal_title：Blood

authors： Liyanage SU,Hurren R,Voisin V,Bridon G,Wang X,Xu C,MacLean N,Siriwardena TP,Gronda M,Yehudai D,Sriskanthadevan S,Avizonis D,Shamas-Din A,Minden MD,Bader GD,Laposa R,Schimmer AD

更新日期：2017-05-11 00:00:00

abstract：:B12 deficiency is the leading cause of megaloblastic anemia, and although more common in the elderly, can occur at any age. Clinical disease caused by B12 deficiency usually connotes severe deficiency, resulting from a failure of the gastric or ileal phase of physiological B12 absorption, best exemplified by the autoi...

journal_title：Blood

authors： Green R

更新日期：2017-05-11 00:00:00

abstract：:Hematopoietic stem and progenitor cells (HSPCs) are vulnerable to endogenous damage and defects in DNA repair can limit their function. The 2 single-stranded DNA (ssDNA) binding proteins SSB1 and SSB2 are crucial regulators of the DNA damage response; however, their overlapping roles during normal physiology are incom...

journal_title：Blood

authors： Shi W,Vu T,Boucher D,Biernacka A,Nde J,Pandita RK,Straube J,Boyle GM,Al-Ejeh F,Nag P,Jeffery J,Harris JL,Bain AL,Grzelak M,Skrzypczak M,Mitra A,Dojer N,Crosetto N,Cloonan N,Becherel OJ,Finnie J,Skaar JR,Walkle

更新日期：2017-05-04 00:00:00

abstract：:Transfusion-related acute lung injury (TRALI) is the leading cause of transfusion-related fatalities and is characterized by acute respiratory distress following blood transfusion. Donor antibodies are frequently involved; however, the pathogenesis and protective mechanisms in the recipient are poorly understood, and ...

journal_title：Blood

authors： Kapur R,Kim M,Aslam R,McVey MJ,Tabuchi A,Luo A,Liu J,Li Y,Shanmugabhavananthan S,Speck ER,Zufferey A,Yousef G,Zhang H,Rondina MT,Weyrich AS,Porcelijn L,Kuebler WM,Slutsky AS,Semple JW

更新日期：2017-05-04 00:00:00

abstract：:Understanding leukemia heterogeneity is critical for the development of curative treatments as the failure to eliminate therapy-persistent leukemic stem cells (LSCs) may result in disease relapse. Here we have combined high-throughput immunophenotypic screens with large-scale single-cell gene expression analysis to de...

journal_title：Blood

authors： Warfvinge R,Geironson L,Sommarin MNE,Lang S,Karlsson C,Roschupkina T,Stenke L,Stentoft J,Olsson-Strömberg U,Hjorth-Hansen H,Mustjoki S,Soneji S,Richter J,Karlsson G

更新日期：2017-04-27 00:00:00

abstract：:Recently, we have identified scavenger receptor class A member I (SR-AI) as a receptor for coagulation factor X (FX), mediating the formation of an FX reservoir at the macrophage surface. Here, we demonstrate that the FX/SR-AI-complex comprises a third protein, pentraxin-2 (PTX2). The presence of PTX2 is essential to ...

journal_title：Blood

authors： Muczynski V,Aymé G,Regnault V,Vasse M,Borgel D,Legendre P,Bazaa A,Harel A,Loubière C,Lenting PJ,Denis CV,Christophe OD

更新日期：2017-04-27 00:00:00

abstract：:In the phase 3 Study of Eliglustat Tartrate (Genz-112638) in Patients With Gaucher Disease Who Have Reached Therapeutic Goals With Enzyme Replacement Therapy (ENCORE), at 1 year, eliglustat was noninferior to imiglucerase enzyme therapy in maintaining stable platelet counts, hemoglobin concentrations, and spleen and l...

journal_title：Blood

authors： Cox TM,Drelichman G,Cravo R,Balwani M,Burrow TA,Martins AM,Lukina E,Rosenbloom B,Goker-Alpan O,Watman N,El-Beshlawy A,Kishnani PS,Pedroso ML,Gaemers SJM,Tayag R,Peterschmitt MJ

更新日期：2017-04-27 00:00:00

abstract：:The treatment of multiple myeloma is considered a continuously evolving paradigm as a result of the growing availability of new and highly effective drugs, including first- and second-generation proteasome inhibitors, immunomodulatory agents, and monoclonal antibodies. Clinical trials advocate long-term rather than sh...

journal_title：Blood

authors： Delforge M,Ludwig H

更新日期：2017-04-27 00:00:00

abstract：:Chronic myelomonocytic leukemia (CMML) is a hematological malignancy characterized by uncontrolled proliferation of dysplastic myelomonocytes and frequent progression to acute myeloid leukemia (AML). We identified mutations in the Cbl gene, which encodes a negative regulator of cytokine signaling, in a subset of CMML ...

journal_title：Blood

authors： Nakata Y,Ueda T,Nagamachi A,Yamasaki N,Ikeda KI,Sera Y,Takubo K,Kanai A,Oda H,Sanada M,Ogawa S,Tsuji K,Ebihara Y,Wolff L,Honda ZI,Suda T,Inaba T,Honda H

更新日期：2017-04-13 00:00:00

abstract：:GATA family proteins play essential roles in development of many cell types, including hematopoietic, cardiac, and endodermal lineages. The first three factors, GATAs 1, 2, and 3, are essential for normal hematopoiesis, and their mutations are responsible for a variety of blood disorders. Acquired and inherited GATA1 ...

journal_title：Blood

authors： Crispino JD,Horwitz MS

更新日期：2017-04-13 00:00:00

abstract：:CD99 is a crucial regulator of the transmigration (diapedesis) of leukocytes through the blood vessel wall. Here, we report that CD99 acts at 2 different steps in the extravasation process. In agreement with previous antibody-blocking experiments, we found that CD99 gene inactivation caused neutrophil accumulation bet...

journal_title：Blood

authors： Goswami D,März S,Li YT,Artz A,Schäfer K,Seelige R,Pacheco-Blanco M,Jing D,Bixel MG,Araki M,Araki K,Yamamura KI,Vestweber D

更新日期：2017-03-30 00:00:00

abstract：:Human herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accou...

journal_title：Blood

authors： Fajgenbaum DC,Uldrick TS,Bagg A,Frank D,Wu D,Srkalovic G,Simpson D,Liu AY,Menke D,Chandrakasan S,Lechowicz MJ,Wong RS,Pierson S,Paessler M,Rossi JF,Ide M,Ruth J,Croglio M,Suarez A,Krymskaya V,Chadburn A,Colleoni

更新日期：2017-03-23 00:00:00

abstract：:Chronic myeloid leukemia (CML) is caused by the acquisition of the tyrosine kinase BCR-ABL1 in a hemopoietic stem cell, transforming it into a leukemic stem cell (LSC) that self-renews, proliferates, and differentiates to give rise to a myeloproliferative disease. Although tyrosine kinase inhibitors (TKIs) that target...

journal_title：Blood

authors： Holyoake TL,Vetrie D

更新日期：2017-03-23 00:00:00

abstract：:Over the past few years, substantial advances have been made in understanding the pathogenesis, evolution, and complexity of mast cell neoplasms. New diagnostic and prognostic parameters and novel therapeutic targets with demonstrable clinical impact have been identified. Several of these new markers, molecular target...

journal_title：Blood

authors： Valent P,Akin C,Metcalfe DD

更新日期：2017-03-16 00:00:00

abstract：:Several strategies can be used to manage fetal or neonatal alloimmune thrombocytopenia (FNAIT) in subsequent pregnancies. Serial fetal blood sampling (FBS) and intrauterine platelet transfusions (IUPT), as well as weekly maternal IV immunoglobulin infusion (IVIG), with or without additional corticosteroid therapy, are...

journal_title：Blood

authors： Winkelhorst D,Murphy MF,Greinacher A,Shehata N,Bakchoul T,Massey E,Baker J,Lieberman L,Tanael S,Hume H,Arnold DM,Baidya S,Bertrand G,Bussel J,Kjaer M,Kaplan C,Kjeldsen-Kragh J,Oepkes D,Ryan G

更新日期：2017-03-16 00:00:00

abstract：:The introduction of the Bruton tyrosine kinase (BTK) inhibitor ibrutinib has dramatically changed the management of chronic lymphocytic leukemia (CLL). Although responses have been durable in the majority of patients, relapses do occur, especially in the high-risk patient population. Most relapses occur as the result ...

journal_title：Blood

authors： Woyach JA

更新日期：2017-03-09 00:00:00

abstract：:Immunological control may contribute to achievement of deep molecular response in chronic myeloid leukemia (CML) patients on tyrosine kinase inhibitor (TKI) therapy and may promote treatment-free remission (TFR). We investigated effector and suppressor immune responses in CML patients at diagnosis (n = 21), on TKI (im...

journal_title：Blood

authors： Hughes A,Clarson J,Tang C,Vidovic L,White DL,Hughes TP,Yong AS

更新日期：2017-03-02 00:00:00

abstract：:Complement plays a key role in host defense, but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins, including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic...

journal_title：Blood

authors： Ueda Y,Mohammed I,Song D,Gullipalli D,Zhou L,Sato S,Wang Y,Gupta S,Cheng Z,Wang H,Bao J,Mao Y,Brass L,Zheng XL,Miwa T,Palmer M,Dunaief J,Song WC

更新日期：2017-03-02 00:00:00

abstract：:Platelets contain and release several matrix metalloproteinases (MMPs). Among these, active MMP-2 enhances platelet aggregation by favoring the activation of phosphatidylinositol 3- kinase (PI3K) and contributes to arterial thrombosis. The platelet surface target of MMP-2 and the mechanism through which it primes plat...

journal_title：Blood

authors： Sebastiano M,Momi S,Falcinelli E,Bury L,Hoylaerts MF,Gresele P

更新日期：2017-02-16 00:00:00

abstract：:Human herpesvirus 8 (HHV-8) is the causative agent of Kaposi sarcoma (KS) and multicentric Castleman disease (MCD), a life-threatening, virally induced B-cell lymphoproliferative disorder. HHV-8 is a B-lymphotropic γ-herpesvirus closely related to the Epstein-Barr virus (EBV). Invariant natural killer T (iNKT) cells a...

journal_title：Blood

authors： Sbihi Z,Dossier A,Boutboul D,Galicier L,Parizot C,Emarre A,Hoareau B,Dupin N,Marcelin AG,Oudin A,Fieschi C,Agbalika F,Autran B,Oksenhendler E,Carcelain G

更新日期：2017-02-16 00:00:00