abstract：:Pediatric thromboembolism (≤18 years) is very rare (0.07-0.14/10,000/year) but may be more prevalent in children with severe thrombophilia (protein C, protein S or antithrombin deficiency). The aim of this study was to define the prevalence and clinical characteristics of pediatric thrombosis in subjects with inherite...

journal_title：Haematologica

authors： de la Morena-Barrio B,Orlando C,de la Morena-Barrio ME,Vicente V,Jochmans K,Corral J

更新日期：2019-12-01 00:00:00

abstract：:Children with immune thrombocytopenia for ≥6 months completing a romiplostim study received weekly subcutaneous romiplostim (1-10 μg/kg targeting platelet counts of 50-200×109/L) in this extension to examine romiplostim's long-term safety and efficacy. Sixty-five children received romiplostim for a median of 2.6 years...

journal_title：Haematologica

authors： Tarantino MD,Bussel JB,Blanchette VS,Beam D,Roy J,Despotovic J,Raj A,Carpenter N,Mehta B,Eisen M

更新日期：2019-11-01 00:00:00

abstract：:It has been postulated that monitoring measurable residual disease (MRD) could be used as a surrogate marker of progression-free survival (PFS) in chronic lymphocytic leukemia (CLL) patients after treatment with immunochemotherapy regimens. In this study, we analyzed the outcome of 84 patients at 3 years of follow-up ...

journal_title：Haematologica

authors： García-Marco JA,Jiménez JL,Recasens V,Zarzoso MF,González-Barca E,De Marcos NS,Ramírez MJ,Parraga FJP,Yañez L,De La Serna Torroba J,Malo MDG,Ariznavarreta GD,Persona EP,Guinaldo MAR,De Paz Arias R,Llanos EB,Jarque I,Val

更新日期：2019-11-01 00:00:00

abstract：:Hemophagocytic lymphohistiocytosis is a life-threatening systemic hyperinflammatory disorder with primary and secondary forms. Primary hemophagocytic lymphohistiocytosis is associated with inherited defects in various genes that affect the immunological cytolytic pathway. Secondary hemophagocytic lymphohistiocytosis i...

journal_title：Haematologica

authors： Tsuboi I,Harada T,Hirabayashi Y,Aizawa S

更新日期：2019-10-01 00:00:00

abstract：:Normal hematopoiesis can be disrupted by the leukemic bone marrow microenvironment, which leads to cytopenia-associated symptoms including anemia, hemorrhage and infection. Thrombocytopenia is a major and sometimes fatal complication in patients with acute leukemia. However, the mechanisms underlying defective thrombo...

journal_title：Haematologica

authors： Gao A,Gong Y,Zhu C,Yang W,Li Q,Zhao M,Ma S,Li J,Hao S,Cheng H,Cheng T

更新日期：2019-10-01 00:00:00

abstract：:The regulation of platelets by oxidants is critical for vascular health and may explain thrombotic complications in diseases such as diabetes and dementia, but remains poorly understood. Here, we describe a novel technique combining electron paramagnetic resonance spectroscopy and turbidimetry, which has been utilized...

journal_title：Haematologica

authors： Vara D,Cifuentes-Pagano E,Pagano PJ,Pula G

更新日期：2019-09-01 00:00:00

abstract：:The balance between self-renewal and differentiation is crucial to ensure the homeostasis of the hematopoietic system, and is a hallmark of hematopoietic stem cells. However, the underlying molecular pathways, including the role of micro-RNA, are not completely understood. To assess the contribution of micro-RNA, we p...

journal_title：Haematologica

authors： Crisafulli L,Muggeo S,Uva P,Wang Y,Iwasaki M,Locatelli S,Anselmo A,Colombo FS,Carlo-Stella C,Cleary ML,Villa A,Gentner B,Ficara F

更新日期：2019-09-01 00:00:00

abstract：:New drugs are needed for the treatment of relapsed acute lymphoblastic leukemia and preclinical evaluation of the MEK inhibitor, selumetinib, has shown that this drug has excellent activity in those leukemias with RAS pathway mutations. The proapoptotic protein, BIM is pivotal in the induction of cell death by both se...

journal_title：Haematologica

authors： Matheson EC,Thomas H,Case M,Blair H,Jackson RK,Masic D,Veal G,Halsey C,Newell DR,Vormoor J,Irving JAE

更新日期：2019-09-01 00:00:00

abstract：:Twice-weekly carfilzomib is approved at 27 and 56 mg/m2 to treat relapsed multiple myeloma patients. In the phase III study ARROW, once-weekly 70 mg/m 2 carfilzomib prolonged the median progression-free survival of relapsed multiple myeloma patients in comparison with twice-weekly 27 mg/m2 carfilzomib, without adding ...

journal_title：Haematologica

authors： Bringhen S,Mina R,Petrucci MT,Gaidano G,Ballanti S,Musto P,Offidani M,Spada S,Benevolo G,Ponticelli E,Galieni P,Cavo M,Di Toritto TC,Di Raimondo F,Montefusco V,Palumbo A,Boccadoro M,Larocca A

更新日期：2019-08-01 00:00:00

abstract：:Proteolysis targeting chimeric molecule ARV 825 causes ubiquitination of bromodomains resulting in their efficient degradation by proteasome activity. Bromodomain degradation down-regulates MYC transcription contributing to growth inhibition of various human cancers. We examined the therapeutic potential of ARV 825 ag...

journal_title：Haematologica

authors： Lim SL,Damnernsawad A,Shyamsunder P,Chng WJ,Han BC,Xu L,Pan J,Pravin DP,Alkan S,Tyner JW,Koeffler HP

更新日期：2019-06-01 00:00:00

abstract：:Germline RUNX1 mutations lead to thrombocytopenia and platelet dysfunction in familial platelet disorder with predisposition to acute myelogenous leukemia (AML). Multiple aspects of platelet function are impaired in these patients, associated with altered expression of genes regulated by RUNX1 We aimed to identify RUN...

journal_title：Haematologica

authors： Glembotsky AC,Sliwa D,Bluteau D,Balayn N,Marin Oyarzún CP,Raimbault A,Bordas M,Droin N,Pirozhkova I,Washington V,Goette NP,Marta RF,Favier R,Raslova H,Heller PG

更新日期：2019-06-01 00:00:00

abstract：:Here we critically evaluate the role of elevated hematocrit as the principal determinant of thrombotic risk in polycythemia and erythrocytosis, defined by an expansion of red cell mass. Since red cell volume determination is no longer readily available, in clinical practice, polycythemia and erythrocytosis are defined...

journal_title：Haematologica

authors： Gordeuk VR,Key NS,Prchal JT

更新日期：2019-04-01 00:00:00

abstract：:Thromboembolism is a serious complication of induction therapy for childhood acute lymphoblastic leukemia. We prospectively compared the efficacy and safety of antithrombotic interventions in the consecutive leukemia trials ALL-BFM 2000 and AIEOP-BFM ALL 2009. Patients with newly diagnosed acute lymphoblastic leukemia...

journal_title：Haematologica

authors： Greiner J,Schrappe M,Claviez A,Zimmermann M,Niemeyer C,Kolb R,Eberl W,Berthold F,Bergsträsser E,Gnekow A,Lassay E,Vorwerk P,Lauten M,Sauerbrey A,Rischewski J,Beilken A,Henze G,Korte W,Möricke A,THROMBOTECT Study Inv

更新日期：2019-04-01 00:00:00

abstract：:Platelets are critical to arterial thrombosis, which underlies myocardial infarction and stroke. Activated platelets, regardless of the nature of their stimulus, initiate energy-intensive processes that sustain thrombus, while adapting to potential adversities of hypoxia and nutrient deprivation within the densely pac...

journal_title：Haematologica

authors： Kulkarni PP,Tiwari A,Singh N,Gautam D,Sonkar VK,Agarwal V,Dash D

更新日期：2019-04-01 00:00:00

abstract：:Hypertension is a major, independent risk factor for atherosclerotic cardiovascular disease. However, this pathology can arise through multiple pathways, which could influence vascular disease through distinct mechanisms. An overactive sympathetic nervous system is a dominant pathway that can precipitate in elevated b...

journal_title：Haematologica

authors： Al-Sharea A,Lee MKS,Whillas A,Michell DL,Shihata WA,Nicholls AJ,Cooney OD,Kraakman MJ,Veiga CB,Jefferis AM,Jackson K,Nagareddy PR,Lambert G,Wong CHY,Andrews KL,Head GA,Chin-Dusting J,Murphy AJ

更新日期：2019-03-01 00:00:00

abstract：:Asparaginase-associated pancreatitis is a life-threatening toxicity to childhood acute lymphoblastic leukemia treatment. To elucidate genetic predisposition and asparaginase-associated pancreatitis pathogenesis, ten trial groups contributed remission samples from patients aged 1.0-17.9 years treated for acute lymphobl...

journal_title：Haematologica

authors： Wolthers BO,Frandsen TL,Patel CJ,Abaji R,Attarbaschi A,Barzilai S,Colombini A,Escherich G,Grosjean M,Krajinovic M,Larsen E,Liang DC,Möricke A,Rasmussen KK,Samarasinghe S,Silverman LB,van der Sluis IM,Stanulla M,Tulstr

更新日期：2019-03-01 00:00:00

abstract：:Anemia is highly prevalent, especially in older individuals. In selected populations, anemia has been reported to be associated with impaired survival and health-related quality of life. However, data on this impact in the general population are rare. Furthermore, discussions on the optimal definition of anemia have n...

journal_title：Haematologica

authors： Wouters HJCM,van der Klauw MM,de Witte T,Stauder R,Swinkels DW,Wolffenbuttel BHR,Huls G

更新日期：2019-03-01 00:00:00

abstract：:Spontaneous Rh blood group changes are a striking sign, reported to occur mainly in patients with hematologic disorders. Upon routine blood grouping, 2 unrelated individuals showed unexplained mixed red cell phenotype regarding the highly immunogenic c antigen (RH4), clinically relevant for blood transfusion and fetom...

journal_title：Haematologica

authors： Dauber EM,Mayr WR,Hustinx H,Schönbacher M,Budde H,Legler TJ,König M,Haas OA,Fritsch G,Körmöczi GF

更新日期：2019-03-01 00:00:00

abstract：:We previously reported initial results in 102 multiple myeloma (MM) patients treated with sequential high-dose melphalan and autologous hematopoietic cell transplantation followed by 200 cGy total body irradiation with or without fludarabine 90 mg/m2 and allogeneic hematopoietic cell transplantation. Here we present l...

journal_title：Haematologica

authors： Maffini E,Storer BE,Sandmaier BM,Bruno B,Sahebi F,Shizuru JA,Chauncey TR,Hari P,Lange T,Pulsipher MA,McSweeney PA,Holmberg L,Becker PS,Green DJ,Mielcarek M,Maloney DG,Storb R

更新日期：2019-02-01 00:00:00

abstract：:Iron recycling by macrophages is essential for erythropoiesis, but may also be relevant for iron redistribution to neighboring cells at the local tissue level. Using mice with iron retention in macrophages due to targeted inactivation of the iron exporter ferroportin, we investigated the role of macrophage iron releas...

journal_title：Haematologica

authors： Recalcati S,Gammella E,Buratti P,Doni A,Anselmo A,Locati M,Cairo G

更新日期：2019-01-01 00:00:00

abstract：:Inherited bone marrow failure syndromes are experiments of nature characterized by impaired hematopoiesis with cancer and leukemia predisposition. The mutations associated with inherited bone marrow failure syndromes affect fundamental cellular pathways, such as DNA repair, telomere maintenance, or proteostasis. How t...

journal_title：Haematologica

authors： Oyarbide U,Topczewski J,Corey SJ

更新日期：2019-01-01 00:00:00

abstract：:Hematologic responses to hypomethylating agents are often delayed in patients with myelodysplastic syndrome or acute myeloid leukemia. Fetal hemoglobin is a potential novel bio-marker of response: recently, we demonstrated that a high fetal hemoglobin level prior to decitabine treatment was associated with superior ou...

journal_title：Haematologica

authors： Stomper J,Ihorst G,Suciu S,Sander PN,Becker H,Wijermans PW,Plass C,Weichenhan D,Bissé E,Claus R,Lübbert M

更新日期：2019-01-01 00:00:00

abstract：:The detection of iron deficiency anemia is challenged by the paucity of diagnostic tests demonstrating high sensitivity and specificity. Using two biomarkers, zinc-protoporphyrin/heme and hepcidin, we established the diagnostic cut-off values for iron deficiency anemia in preschool children and women. We randomly sele...

journal_title：Haematologica

authors： Kanuri G,Chichula D,Sawhney R,Kuriakose K,De'Souza S,Pais F,Arumugam K,Shet AS

更新日期：2018-12-01 00:00:00

abstract：:The bone marrow microenvironment regulates normal and malignant hematopoiesis, but the underlying molecular mechanisms remain poorly defined. Using a chimeric mice model, we demonstrate that Twist1 deletion in the bone marrow microenvironment results in alteration of multiple niche cells as well as downregulated expre...

journal_title：Haematologica

authors： Liu X,Ma Y,Li R,Guo D,Wang N,Zhao Y,Yin J,Ren Q,Lin Y,Ma X

更新日期：2018-12-01 00:00:00

abstract：:Pathological erythropoiesis with consequent anemia is a leading cause of symptomatic morbidity in internal medicine. The etiologies of anemia are complex and include reactive as well as neoplastic conditions. Clonal expansion of erythroid cells in the bone marrow may result in peripheral erythrocytosis and polycythemi...

journal_title：Haematologica

authors： Valent P,Büsche G,Theurl I,Uras IZ,Germing U,Stauder R,Sotlar K,Füreder W,Bettelheim P,Pfeilstöcker M,Oberbauer R,Sperr WR,Geissler K,Schwaller J,Moriggl R,Béné MC,Jäger U,Horny HP,Hermine O

更新日期：2018-10-01 00:00:00

abstract：:The stimulation of erythrocyte formation increases the demand for iron by the bone marrow and this in turn may affect the levels of circulating diferric transferrin. As this molecule influences the production of the iron regulatory hormone hepcidin, we hypothesized that erythropoiesis-driven changes in diferric transf...

journal_title：Haematologica

authors： Mirciov CSG,Wilkins SJ,Hung GCC,Helman SL,Anderson GJ,Frazer DM

更新日期：2018-10-01 00:00:00

abstract：:The immunomodulatory drugs, lenalidomide and pomalidomide yield high response rates in multiple myeloma patients, but are associated with a high rate of thrombocytopenia and increased risk of secondary hematologic malignancies. Here, we demonstrate that the immunomodulatory drugs induce self-renewal of hematopoietic p...

journal_title：Haematologica

authors： Liu A,Li S,Donnenberg V,Fu J,Gollin SM,Ma H,Lu C,Stolz DB,Mapara MY,Monaghan SA,Lentzsch S

更新日期：2018-10-01 00:00:00

abstract：:The success of hematopoietic stem cell transplantation is determined by multiple factors. Additional complexity is conferred by covariables showing time-dependent effects. We evaluated the effect of predictors on competing-risk outcomes after hematopoietic stem cell transplantation in a time-dependent manner. We analy...

journal_title：Haematologica

authors： Fuerst D,Frank S,Mueller C,Beelen DW,Schetelig J,Niederwieser D,Finke J,Bunjes D,Kröger N,Neuchel C,Tsamadou C,Schrezenmeier H,Beyersmann J,Mytilineos J

更新日期：2018-09-01 00:00:00

abstract：:Diseases with clonal hematopoiesis such as myelodysplastic syndrome and acute myeloid leukemia have high rates of relapse. Only a small subset of acute myeloid leukemia patients are cured with chemotherapy alone. Relapse in these diseases occurs at least in part due to the failure to eradicate leukemic stem cells or h...

journal_title：Haematologica

authors： Mani R,Goswami S,Gopalakrishnan B,Ramaswamy R,Wasmuth R,Tran M,Mo X,Gordon A,Bucci D,Lucas DM,Mims A,Brooks C,Dorrance A,Walker A,Blum W,Byrd JC,Lozanski G,Vasu S,Muthusamy N

更新日期：2018-08-01 00:00:00

abstract：:Patients with advanced myeloma experience a high symptom burden particularly near the end of life, making timely hospice use crucial. Little is known about the quality and determinants of end-of-life care for this population, including whether potential increases in hospice use are also accompanied by "late" enrollmen...

journal_title：Haematologica

authors： Odejide OO,Li L,Cronin AM,Murillo A,Richardson PG,Anderson KC,Abel GA

更新日期：2018-08-01 00:00:00

abstract：:Mucosa-associated lymphoid tissue (MALT) lymphoma originates from a background of diverse chronic inflammatory disorders at various anatomic sites. The genetics underlying its development, particularly in those associated with autoimmune disorders, is poorly characterized. By whole exome sequencing of 21 cases of MALT...

journal_title：Haematologica

authors： Moody S,Thompson JS,Chuang SS,Liu H,Raderer M,Vassiliou G,Wlodarska I,Wu F,Cogliatti S,Robson A,Ashton-Key M,Bi Y,Goodlad J,Du MQ

更新日期：2018-08-01 00:00:00

abstract：:This analysis explored factors influencing survival of patients with primary refractory and relapsed peripheral T-cell lymphomas enrolled in the prospective International T-cell Project. We analyzed data from 1020 patients with newly diagnosed disease, enrolled between September 2006 and December 2015. Out of 937 pati...

journal_title：Haematologica

authors： Bellei M,Foss FM,Shustov AR,Horwitz SM,Marcheselli L,Kim WS,Cabrera ME,Dlouhy I,Nagler A,Advani RH,Pesce EA,Ko YH,Martinez V,Montoto S,Chiattone C,Moskowitz A,Spina M,Biasoli I,Manni M,Federico M,International T-c

更新日期：2018-07-01 00:00:00

abstract：:Tissue Factor is a cell-surface glycoprotein expressed in various cells of the vasculature and is the principal regulator of the blood coagulation cascade and hemostasis. Notably, aberrant expression of Tissue Factor is associated with cardiovascular pathologies such as atherosclerosis and thrombosis. Here, we sought ...

journal_title：Haematologica

authors： Kurakula K,Koenis DS,Herzik MA Jr,Liu Y,Craft JW Jr,van Loenen PB,Vos M,Tran MK,Versteeg HH,Goumans MTH,Ruf W,de Vries CJM,Şen M

更新日期：2018-06-01 00:00:00

abstract：:Signaling through the αβT cell receptor (TCR) is a crucial determinant of T-cell fate and can induce two opposite outcomes during thymocyte development: cell death or survival and differentiation. To date, the role played by T-cell receptor in the oncogenic transformation of developing T cells remains unclear. Here we...

journal_title：Haematologica

authors： Gon S,Loosveld M,Crouzet T,Potier D,Bonnet M,Morin SO,Michel G,Vey N,Nunès JA,Malissen B,Roncagalli R,Nadel B,Payet-Bornet D

更新日期：2018-06-01 00:00:00

abstract：:In contrast to well-established hierarchical concepts of tumor stem cells, leukemia-initiating cells in B-cell precursor acute lymphoblastic leukemia have not yet been phenotypically identified. Different subpopulations, as defined by surface markers, have shown equal abilities to reconstitute leukemia upon transplant...

journal_title：Haematologica

authors： Trentin L,Queudeville M,Eckhoff SM,Hasan N,Münch V,Boldrin E,Seyfried F,Enzenmüller S,Debatin KM,Meyer LH

更新日期：2018-06-01 00:00:00

abstract：:Glycoprotein VI, a major platelet activation receptor for collagen and fibrin, is considered a particularly promising, safe antithrombotic target. In this study, we show that human glycoprotein VI signals upon platelet adhesion to fibrinogen. Full spreading of human platelets on fibrinogen was abolished in platelets f...

journal_title：Haematologica

authors： Mangin PH,Onselaer MB,Receveur N,Le Lay N,Hardy AT,Wilson C,Sanchez X,Loyau S,Dupuis A,Babar AK,Miller JL,Philippou H,Hughes CE,Herr AB,Ariëns RA,Mezzano D,Jandrot-Perrus M,Gachet C,Watson SP

更新日期：2018-05-01 00:00:00

abstract：:Previously, we found that LDL-receptor related protein-1 on macrophages mediated shear stress-dependent clearance of von Willebrand factor. In control experiments, however, we observed that von Willebrand factor also binds to macrophages independently of this receptor under static conditions, suggesting the existence ...

journal_title：Haematologica

authors： Wohner N,Muczynski V,Mohamadi A,Legendre P,Proulle V,Aymé G,Christophe OD,Lenting PJ,Denis CV,Casari C

更新日期：2018-04-01 00:00:00

abstract：:Tyrosine kinase inhibitors (TKIs) are highly effective for the treatment of chronic myeloid leukemia (CML), but very few patients are cured. The major drawbacks regarding TKIs are their low efficacy in eradicating the leukemic stem cells responsible for disease maintenance and relapse upon drug cessation. Herein, we p...

journal_title：Haematologica

authors： Landberg N,von Palffy S,Askmyr M,Lilljebjörn H,Sandén C,Rissler M,Mustjoki S,Hjorth-Hansen H,Richter J,Ågerstam H,Järås M,Fioretos T

更新日期：2018-03-01 00:00:00

abstract：:Patients with anaplastic lymphoma kinase-positive anaplastic large cell lymphoma often present with B-symptoms or hemophagocytosis and generate an anti-tumor immune response. Specific serum cytokine levels or profiles may reflect the tumor burden, non-specific immune stimulation by the tumor or differences in the stre...

journal_title：Haematologica

authors： Knörr F,Damm-Welk C,Ruf S,Singh VK,Zimmermann M,Reiter A,Woessmann W

更新日期：2018-03-01 00:00:00

abstract：:Survival of myeloma patients has greatly improved with the use of autologous stem cell transplantation and novel agents, such as proteasome inhibitors, immunomodulatory drugs and monoclonal antibodies. Compared to bortezomib- and lenalidomide-based regimens alone, the addition of high-dose melphalan followed by autolo...

journal_title：Haematologica

authors： Gay F,Engelhardt M,Terpos E,Wäsch R,Giaccone L,Auner HW,Caers J,Gramatzki M,van de Donk N,Oliva S,Zamagni E,Garderet L,Straka C,Hajek R,Ludwig H,Einsele H,Dimopoulos M,Boccadoro M,Kröger N,Cavo M,Goldschmidt H,B

更新日期：2018-02-01 00:00:00