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Haematologica

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https://www.readbyqxmd.com/read/29146710/jak2-calr-mpl-and-asxl1-mutational-status-correlates-with-distinct-histologic-features-in-philadelphia-chromosome-negative-myeloproliferative-neoplasms
#1
Waihay J Wong, Robert P Hasserjian, Geraldine S Pinkus, Lawrence J Breyfogle, Ann Mullaly, Olga Pozdnyakova
No abstract text is available yet for this article.
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29146709/alternatively-spliced-fibronectin-extra-domain-a-is-required-for-hemangiogenic-recovery-upon-bone-marrow-chemotherapy
#2
Alessandro Malara, Cristian Gruppi, Giuseppe Celesti, Vittorio Abbonante, Gianluca Viarengo, Luigi Laghi, Luigi De Marco, Andrés F Muro, Alessandra Balduini
No abstract text is available yet for this article.
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29146708/inflammatory-molecule-reduction-with-hydroxyurea-therapy-in-children-with-sickle-cell-anemia
#3
Rhiannon R Penkert, Julia L Hurwitz, Paul Thomas, Jason Rosch, Jola Dowdy, Yilun Sun, Li Tang, Jane S Hankins
No abstract text is available yet for this article.
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29146707/outcomes-after-allogeneic-hematopoietic-stem-cell-transplantation-in-patients-with-acute-myeloid-leukemia-harboring-t-7-11-p15-p15
#4
Kaito Harada, Noriko Doki, Jun Aoki, Jinichi Mori, Shinichiro Machida, Masayoshi Masuko, Naoyuki Uchida, Yuho Najima, Takahiro Fukuda, Heiwa Kanamori, Hiroyasu Ogawa, Shuichi Ota, Kazuei Ogawa, Satoshi Takahashi, Masanobu Kasai, Akio Maeda, Koji Nagafuji, Toshiro Kawakita, Tatsuo Ichinohe, Yoshiko Atsuta
No abstract text is available yet for this article.
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29146706/the-clinical-and-laboratory-evaluation-of-familial-hemophagocytic-lymphohistiocytosis-and-the-importance-of-hepatic-and-spinal-cord-involvement-a-single-center-experience
#5
Burcin Beken, Selin Aytac, Gunay Balta, Baris Kuskonmaz, Duygu Uckan, Sule Unal, Mualla Cetin, Fatma Gumruk
Familial hemophagocytic lymphohistiocytosis is an autosomal recessive, life-threatening condition characterized by defective immune response. A retrospective analysis was performed on 57 patients diagnosed with familial hemophagocytic lymphohistiocytosis at Hacettepe University Pediatric Hematology Department. Mutation analysis was performed on 37 patients and of these; 11 had UNC13D, 10 had PRF1 and 3 had STX11 gene mutation. Of these patients 44% were found to have central nervous system involvement on admission and spinal cord involvement was also seen in 5 patients...
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29146705/complement-c3-is-a-novel-modulator-of-the-anti-fviii-immune-response
#6
Julie Rayes, Mathieu Ing, Sandrine Delignat, Ivan Peyron, Laurent Gilardin, Carl-Wilhelm Vogel, David C Fritzinger, Véronique Frémeaux-Bacchi, Srinivas V Kaveri, Lubka T Roumenina, Sébastien Lacroix-Desmazes
Development of neutralizing antibodies against therapeutic factor VIII (FVIII) is the most serious complication of the treatment of hemophilia A. Increasing evidence shows the multifactorial origin of the anti-FVIII immune response, combining both genetic and environmental factors. While a role for the complement system on innate as well as adaptive immunity has been documented, the implication of complement activation on the onset of the anti-FVIII immune response is unknown. Here, using in vitro assays for FVIII endocytosis by human monocyte-derived dendritic cells and presentation to T cells, as well as animal models of in vivo complement depletion, we show a novel role for complement C3 in enhancing the immune response against therapeutic FVIII...
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29122993/the-microenvironmental-stromal-cells-abrogate-nf-%C3%AE%C2%BAb-inhibitor-induced-apoptosis-in-chronic-lymphocytic-leukemia
#7
Carl Philipp Simon-Gabriel, Katharina Foerster, Shifa Saleem, Dorothee Bleckmann, Marco Benkisser-Petersen, Nicolas Thornton, Kazuo Umezawa, Sarah Decker, Meike Burger, Hendrik Veelken, Rainer Claus, Christine Dierks, Justus Duyster, Katja Zirlik
NF-κB is known to play an important role in the pathogenesis of chronic lymphocytic leukemia. Several NF-κB inhibitors have been shown to successfully induce apoptosis of chronic lymphocytic leukemia cells in vitro. Since the microenvironment is known to be crucial for the survival of chronic lymphocytic leukemia cells, we tested here whether NF-κB inhibition may still induce apoptosis in these leukemic cells in the presence of protective stromal interaction. We used the specific NF-κB inhibitor Dehydroxymethylepoxyquinomicin...
November 9, 2017: Haematologica
https://www.readbyqxmd.com/read/29122992/labile-plasma-iron-levels-predict-survival-in-patients-with-lower-risk-myelodysplastic-syndromes
#8
Louise de Swart, Chloé Reiniers, Timothy Bagguley, Corine van Marrewijk, David Bowen, Eva Hellström-Lindberg, Aurelia Tatic, Argiris Symeonidis, Gerwin Huls, Jaroslav Cermak, Arjan A van de Loosdrecht, Hege Garelius, Dominic Culligan, Mac Macheta, Michail Spanoudakis, Panagiotis Panagiotidis, Marta Krejci, Nicole Blijlevens, Saskia Langemeijer, Jacqueline Droste, Dorine W Swinkels, Alexandra Smith, Theo de Witte
Red blood cell transfusions remain one of the cornerstones in supportive care of lower-risk patients with myelodysplastic syndromes. We hypothesized that patients develop oxidant mediated tissue injury through the formation of toxic iron species, caused either by red blood cell transfusions or by ineffective erythropoiesis. We analyzed serum samples from 100 lower-risk patients with myelodysplastic syndromes at six-month intervals for transferrin saturation, hepcidin-25, growth differentiation factor 15, soluble transferrin receptor, non-transferrin bound iron and labile plasma iron in order to evaluate temporal changes in iron metabolism and presence of potentially toxic iron species and their impact on survival...
November 9, 2017: Haematologica
https://www.readbyqxmd.com/read/29122991/maternal-embryonic-leucine-zipper-kinase-is-a-novel-target-for-proliferation-associated-high-risk-myeloma
#9
Arnold Bolomsky, Roy Heusschen, Karin Schlangen, Kathrin Stangelberger, Joséphine Muller, Wolfgang Schreiner, Niklas Zojer, Jo Caers, Heinz Ludwig
Treatment of high-risk patients is a major challenge in multiple myeloma. This is especially true for patients assigned to the gene-expression-profiling defined proliferation subgroup. Although recent efforts have identified some key players of proliferative myeloma, genetic interactions and players that can be targeted with clinically effective drugs have to be identified to overcome the poor prognosis of these patients. We therefore examined maternal embryonic leucine zipper kinase (MELK) for its implications in hyper-proliferative myeloma and analysed the activity of the MELK inhibitor OTSSP167 in vitro and in vivo...
November 9, 2017: Haematologica
https://www.readbyqxmd.com/read/29122990/expression-of-cobll1-encoding-novel-ror1-binding-partner-is-robust-predictor-of-survival-in-chronic-lymphocytic-leukemia
#10
Hana Plešingerová, Pavlína Janovská, Archana Mishra, Lucie Smyčková, Lucie Poppová, Antonín Libra, Karla Plevová, Petra Ovesná, Lenka Radová, Michael Doubek, Šárka Pavlová, Šárka Pospíšilová, Vítězslav Bryja
Chronic lymphocytic leukemia is a disease with upregulated expression of the transmembrane tyrosine-protein kinase ROR1, a member of the Wnt/planar cell polarity pathway. In this study, we identified COBLL1 as a novel interaction partner of ROR1. COBLL1 shows clear bimodal expression with high levels in chronic lymphocytic leukemia patients with mutated IGHV and approximately 30% of chronic lymphocytic leukemia patients with unmutated IGHV. In the remaining 70% of chronic lymphocytic leukemia patients with unmutated IGHV, COBLL1 expression is low...
November 9, 2017: Haematologica
https://www.readbyqxmd.com/read/29101207/the-ferroportin-disease-pathogenesis-diagnosis-and-treatment
#11
Antonello Pietrangelo
The Ferroportin Disease is an autosomal dominant hereditary iron loading disorder associated with heterozygote mutations of the ferroportin-1 (FPN) gene. It represents one of commonest cause of genetic hyperferritinemia, regardless of ethnicity. FPN1 transfers iron from the intestine, macrophages and placenta into the bloodstream. In the ferroportin disease, loss-of-function mutations of FPN1 limit but do not impair iron-export in enterocytes, but severely affects iron-transfer in macrophages. This leads to progressive and preferential iron trapping in tissue macrophages, reduced iron release to serum transferrin (i...
November 3, 2017: Haematologica
https://www.readbyqxmd.com/read/29101206/knockdown-of-tp53-in-asxl1-negative-background-rescues-apoptotic-phenotype-of-human-hematopoietic-stem-and-progenitor-cells-but-without-overt-malignant-transformation
#12
Susan Hilgendorf, Edo Vellenga
No abstract text is available yet for this article.
November 3, 2017: Haematologica
https://www.readbyqxmd.com/read/29101205/outcome-after-relapse-of-myelodysplastic-syndrome-and-secondary-acute-myeloid-leukemia-after-allogeneic-stem-cell-transplantation-a-retrospective-registry-analysis-on-698-patients-by-the-chronic-malignancies-working-party-of-european-society-of-blood-and-marrow
#13
Christoph Schmid, Liesbeth C de Wreede, Anja van Biezen, Jürgen Finke, Gerhard Ehninger, Arnold Ganser, Liisa Volin, Dietger Niederwieser, Dietrich Beelen, Paolo Alessandrino, Lothar Kanz, Michael Schleuning, Jakob Passweg, Hendrik Veelken, Johan Maertens, Jan J Cornelissen, Didier Blaise, Martin Gramatzki, Noel Milpied, Ibrahim Yakub-Agha, Ghulam Mufti, Montserrat Rovira, Renate Arnold, Theo De Witte, Marie Robin, Nikolaus Kröger
No standard exists for the treatment of myelodysplastic syndrome relapsing after allogeneic stem cell transplantation. We performed a retrospective registry analysis of outcome and risk factors in 698 patients, treated with different strategies. Median overall survival from relapse was 4.7 months (4.1-5.3), 2-year survival was 17.7% (14.8-21.2%). Shorter remission after transplantation (p<0.001), advanced disease (p=0.001), older age (p=0.007), unrelated donor (p=0.008) and acute Graft-versus-Host disease before relapse (p<0...
November 3, 2017: Haematologica
https://www.readbyqxmd.com/read/29097500/a-bio-clinical-prognostic-model-using-myc-and-bcl2-predicts-outcome-in-relapsed-refractory-diffuse-large-b-cell-lymphoma
#14
Mark Bosch, Ariz Akhter, Bingshu E Chen, Adnan Mansoor, David Lebrun, David Good, Michael Crump, Lois Shepherd, David W Scott, Douglas A Stewart
The objective of this study was to create a bio-clinical model, based on clinical and molecular predictors of event-free and overall survival for relapsed/refractory diffuse large B-cell lymphoma patients treated on the Canadian Cancer Trials Group LY12 prospective study. Sufficient histologic material was available for 91 cases to create tissue microarrays and perform immunohistochemistry staining for CD10, BCL6, MUM1/IRF4, FOXP1, LMO2, BCL2, MYC, P53 and pySTAT3 expression. 67 cases had material sufficient for fluorescent in-situ hybridization for MYC and BCL2...
November 2, 2017: Haematologica
https://www.readbyqxmd.com/read/29097499/randomized-study-of-continuous-high-dose-lenalidomide-sequential-azacitidine-and-lenalidomide-or-azacitidine-in-persons-%C3%A2-65-years-with-newly-diagnosed-acute-myeloid-leukemia
#15
Bruno C Medeiros, Kelly McCaul, Suman Kambhampati, Daniel A Pollyea, Rajat Kumar, Lewis R Silverman, Andrea Kew, Lalit Saini, C L Beach, Ravi Vij, Xiwei Wang, Jim Zhong, Robert Peter Gale
Therapy of acute myeloid leukemia in older persons is associated with poor outcomes because of intolerance to intensive therapy, resistant disease and co-morbidities. This multi-center, randomized, open-label, phase-2 trial compared safety and efficacy of three therapeutic strategies in persons ≥65 years with newly-diagnosed acute myeloid leukemia: (1) continuous high-dose lenalidomide (N=15); (2) sequential azacitidine and lenalidomide (N=39); and (3) azacitidine (N=34) only. The efficacy endpoint was 1-year survival...
November 2, 2017: Haematologica
https://www.readbyqxmd.com/read/29097498/short-term-administration-of-jak2-inhibitors-reduces-splenomegaly-in-mouse-models-of-%C3%A3-thalassemia-intermedia-and-major
#16
Carla Casu, Vania Lo Presti, Paraskevi Rea Oikonomidou, Luca Melchiori, Osheiza Abdulmalik, Pedro Ramos, Stefano Rivella
No abstract text is available yet for this article.
November 2, 2017: Haematologica
https://www.readbyqxmd.com/read/29097497/mds1-and-evi1-complex-locus-mecom-a-novel-candidate-gene-for-hereditary-hematological-malignancies
#17
Tim Ripperger, Winfried Hofmann, Jan C Koch, Katayoon Shirneshan, Detlef Haase, Gerald Wulf, Peter R Issing, Matthias Karnebogen, Gunnar Schmidt, Bernd Auber, Brigitte Schlegelberger, Thomas Illig, Birgit Zirn, Doris Steinemann
No abstract text is available yet for this article.
November 2, 2017: Haematologica
https://www.readbyqxmd.com/read/29097496/response-to-mek-inhibition-with-trametinib-and-tyrosine-kinase-inhibition-with-imatinib-in-multifocal-histiocytic-sarcoma
#18
Sophie Voruz, Anne Cairoli, Olaia Naveiras, Laurence de Leval, Edoardo Missiaglia, Krisztian Homicsko, Olivier Michielin, Sabine Blum
No abstract text is available yet for this article.
November 2, 2017: Haematologica
https://www.readbyqxmd.com/read/29097495/ebv-associated-primary-nodal-t-nk-cell-lymphoma-shows-distinct-molecular-signature-and-copy-number-changes
#19
Siok-Bian Ng, Tae-Hoon Chung, Seiichi Kato, Shigeo Nakamura, Emiko Takahashi, Young-Hyeh Ko, Joseph D Khoury, C Cameron Yin, Richie Soong, Anand D Jeyasekharan, Michal Marek Hoppe, Viknesvaran Selvarajan, Soo-Yong Tan, Soon-Thye Lim, Choon-Kiat Ong, Maarja-Liisa Nairismägi, Priyanka Maheshwari, Shoa-Nian Choo, Shuangyi Fan, Chi-Kuen Lee, Shih-Sung Chuang, Wee-Joo Chng
The molecular biology of primary nodal T- and NK-cell lymphoma and its relationsihp with extranodal NK/T-cell lymphoma, nasal type is poorly understood. In this study, we assessed the relationship between nodal and extranodal EBV-positive T/NK-cell lymphomas using gene expression profiling and copy number aberration analyses. We performed GEP and CNA analysis on 66 cases of EBV-associated T/NK-cell lymphoma from nodal and extranodal sites, and correlated the molecular signatures with clinicopathologic features...
November 2, 2017: Haematologica
https://www.readbyqxmd.com/read/29079600/treatment-of-essential-thrombocythemia-in-europe-a-prospective-long-term-observational-study-of-3649-high-risk-patients-in-the-exels-study
#20
Gunnar Birgegard, Carlos Besses, Martin Griesshammer, Luigi Gugliotta, Claire N Harrison, Mohamed Hamdani, Jackie Wu, Henri Achenbach, Jean-Jacques Kiladjian
Evaluation of Xagrid® Efficacy and Long-term Safety, a Phase IV, prospective, non interventional study performed in 13 European countries enrolled high risk essential thrombocythemia patients treated with cytoreductive therapy. Primary objectives were safety and pregnancy outcomes. Of 3721 registered patients, 3649 received cytoreductive therapy. At registration, 3611 were receiving: anagrelide (Xagrid®) (n=804), other cytoreductive therapy (n=2666), anagrelide + other cytoreductive therapy (n=141). Median age was 56 vs 70 years for anagrelide vs other cytoreductive therapy...
October 27, 2017: Haematologica
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