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Haematologica

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https://www.readbyqxmd.com/read/28798071/bortezomib-as-a-new-therapeutic-approach-for-blastic-plasmacytoid-dendritic-cell-neoplasm
#1
Laure Philippe, Adam Ceroi, Elodie Bôle-Richard, Alizée Jenvrin, Sabeha Biichle, Sophie Perrin, Samuel Limat, Francis Bonnefoy, Eric Deconinck, Philippe Saas, Francine Garnache-Ottou, Fanny Angelot-Delettre
Blastic plasmacytoid dendritic cell neoplasm is an aggressive hematological malignancy with a poor prognosis. No consensus for optimal treatment modalities is available today. Targeting the NF-κB pathway is considered as a promising approach since blastic plasmacytoid dendritic cell neoplasm have been reported to exhibit a constitutive activation of the NF-κB pathway. Moreover, NF-κB inhibition in blastic plasmacytoid dendritic cell neoplasm cell lines using either an experimental specific inhibitor JSH23 or the clinical drug bortezomib interferes in vitro with leukemic cell proliferation and survival...
August 10, 2017: Haematologica
https://www.readbyqxmd.com/read/28798070/cxcl13-levels-are-elevated-in-patients-with-waldenstrom-s-macroglobulinemia-and-are-predictive-of-major-response-to-ibrutinib
#2
Josephine M Vos, Nickolas Tsakmaklis, Christopher J Patterson, Kirsten Meid, Jorge J Castillo, Philip Brodsky, Tomas Ganz, Steven T Pals, Marie José Kersten, Lian Xu, Guang Yang, Steven P Treon, Zachary R Hunter
No abstract text is available yet for this article.
August 10, 2017: Haematologica
https://www.readbyqxmd.com/read/28798069/mll-tet1-fusion-protein-promotes-immortalization-of-myeloid-progenitor-cells-and-leukemia-development
#3
Hyeng-Soo Kim, Seung Hwan Oh, Ju-Heon Kim, Jae-Young Kim, Do-Hyung Kim, Soo-Jin Lee, Sang-Un Choi, Kwon Moo Park, Zae Young Ryoo, Tae Sung Park, Sanggyu Lee
No abstract text is available yet for this article.
August 10, 2017: Haematologica
https://www.readbyqxmd.com/read/28798068/consolidation-treatment-with-lenalidomide-following-front-line-or-salvage-chemoimmunotherapy-in-chronic-lymphocytic-leukemia
#4
Paolo Strati, Michael J Keating, Jan A Burger, Susan M O'Brien, William G Wierda, Zeev Estrov, Gracy Zacharian, Alessandra Ferrajoli
No abstract text is available yet for this article.
August 10, 2017: Haematologica
https://www.readbyqxmd.com/read/28775123/tlr9-stimulation-can-induce-ikappabzeta-expression-and-igm-secretion-in-chronic-lymphocytic-leukemia-cells
#5
Eleonora Fonte, Maria Giovanna Vilia, Daniele Reverberi, Ilenia Sana, Lydia Scarfò, Pamela Ranghetti, Ugo Orfanelli, Simone Cenci, Giovanna Cutrona, Paolo Ghia, Marta Muzio
Chronic lymphocytic leukemia cells strongly depend on external stimuli for their survival. Both antigen receptor and co-stimulatory receptors including Toll-Like Receptors can modulate viability and proliferation of leukemic cells. Toll-Like Receptors ligands, and particularly the TLR9 ligand CpG, mediate heterogeneous responses in patient samples reflecting their clinical course. However, the molecular framework of the key signaling events underlying such heterogeneity is undefined. We focused our studies on a subset of chronic lymphocytic leukemia cases characterized by expression of CD38 and unmutated immunoglobulin genes, that respond to CpG with enhanced metabolic cell activity...
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28775122/circulating-microrna-expressions-can-predict-the-outcome-of-lenalidomide-plus-low-dose-dexamethasone-treatment-in-patients-with-refractory-relapsed-multiple-myeloma
#6
Seung-Hyun Jung, Sung-Eun Lee, Minho Lee, So-Hee Kim, Seon-Hee Yim, Tae Woo Kim, Chang-Ki Min, Yeun-Jun Chung
No abstract text is available yet for this article.
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28775121/italian-real-life-experience-with-brentuximab-vedotin-results-of-a-large-observational-study-on-40-relapsed-refractory-systemic-anaplastic-large-cell-lymphoma
#7
Alessandro Broccoli, Cinzia Pellegrini, Alice Di Rocco, Benedetta Puccini, Caterina Patti, Guido Gini, Donato Mannina, Monica Tani, Chiara Rusconi, Alessandra Romano, Anna Vanazzi, Barbara Botto, Carmelo Carlo-Stella, Stefan Hohaus, Pellegrino Musto, Patrizio Mazza, Stefano Molica, Paolo Corradini, Angelo Fama, Francesco Gaudio, Michele Merli, Angela Gravetti, Giuseppe Gritti, Annalisa Arcari, Patrizia Tosi, Anna Marina Liberati, Antonello Pinto, Vincenzo Pavone, Filippo Gherlinzoni, Virginia Naso, Stefano Volpetti, Livio Trentin, Maria Cecilia Goldaniga, Maurizio Bonfichi, Amalia De Renzo, Corrado Schiavotto, Michele Spina, Sergio Storti, Angelo Michele Carella, Vittorio Stefoni, Lisa Argnani, Pier Luigi Zinzani
From November 2012 to July 2014, brentuximab vedotin was available in Italy for patients with relapsed systemic anaplastic large cell lymphoma outside a clinical trial context according to the national law 648/96. A large Italian observational retrospective study was conducted on the use of brentuximab vedotin in the everyday clinical practice to check if clinical trial results are confirmed even in a real life context. Primary endpoint was the best response; secondary endpoints were the overall response rate at the end of the treatment, duration of response, survival and the safety profile...
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28775120/pseudo-monoclonal-gammopathy-a-report-of-four-cases
#8
Majd D Jawad, Ronald S Go, Thomas E Witzig, Joseph R Mikhael, Aishwarya Ravindran, David L Murray
No abstract text is available yet for this article.
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28775119/incidence-and-management-of-toxicity-associated-with-ibrutinib-and-idelalisib-a-practical-approach
#9
Iris de Weerdt, Suzanne M Koopmans, Arnon P Kater, Michel van Gelder
The use of novel B cell receptor signaling inhibitors results in high response rates and long progression-free survival in patients with indolent B cell malignancies, such as chronic lymphocytic leukemia, follicular lymphoma, mantle cell lymphoma and Waldenstroms macroglobulinemia. Ibrutinib, the first-in-class inhibitor or Bruton's tyrosine kinase, and idelalisib, the first-in-class inhibitor of phosphatidylinositol 3-kinase δ have recently been approved for several indolent B cell malignancies. These drugs especially foresee in previously unmet needs for patients with relapsed or refractory disease, high-risk cytogenetic or molecular abnormalities or with comorbidities...
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28775118/chronic-lymphocytic-leukemia-cells-are-active-participants-in-microenvironmental-cross-talk
#10
Martijn Ha van Attekum, Eric Eldering, Arnon P Kater
The importance of the tumor microenvironment in chronic lymphocytic leukemia is widely accepted. Yet, the understanding of the complex interplay between the various types of bystander cells and chronic lymphocytic leukemia cells is incomplete. Whereas numerous studies have indicated that bystander cells provide chronic lymphocytic leukemia-supportive functions, it has also become clear that chronic lymphocytic leukemia cells actively engage in the formation of a supportive tumor microenvironment by engaging in several cross-talk mechanisms...
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28775117/endotoxemia-shifts-neutrophils-with-timp-free-gelatinase-b-mmp-9-from-bone-marrow-to-periphery-and-induces-systematic-upregulation-of-timp-1
#11
Jennifer Vandooren, Wannes Swinnen, Estefania Ugarte-Berzal, Lise Boon, Daphne Dorst, Erik Martens, Ghislain Opdenakker
Lipopolysaccharides or endotoxins elicit an excessive host inflammatory response and lead to life-threatening conditions such as endotoxemia and septic shock. Lipopolysaccharides trigger mobilization and stimulation of leukocytes and exaggerated production of pro-inflammatory molecules including cytokines and proteolytic enzymes. Matrix metalloproteinase-9 (MMP-9) or gelatinase B, a protease stored in the tertiary granules of polymorphonuclear leukocytes, has been implicated in such inflammatory reactions. Moreover, several studies even pinpointed MMP-9 as a potential target molecule to counter excessive inflammation in endotoxemia...
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28751567/the-adamts13-1239-1253-peptide-is-a-dominant-hla-dr1-restricted-cd4-t-cell-epitope
#12
Laurent Gilardin, Sandrine Delignat, Ivan Peyron, Mathieu Ing, Yu-Chun Lone, Bagirath Gangadharan, Baptiste Michard, Yousra Kherabi, Meenu Sharma, Anastas Pashov, Jean-Baptiste Latouche, Mohamad Hamieh, Olivier Toutirais, Pascale Loiseau, Lionel Galicier, Agnès Veyradier, Srini Kaveri, Bernard Maillère, Paul Coppo, Sébastien Lacroix-Desmazes
Acquired thrombotic thrombocytopenic purpura is a rare and severe disease characterized by auto-antibodies directed against "A Disintegrin And Metalloproteinase with Thrombospondin type 1 repeats, 13th member" (ADAMTS13), a plasma protein involved in hemostasis. The implication of CD4+ T cells in the pathogenesis of the disease is suggested by the IgG isotype of the antibodies. However, the nature of the CD4+ T-cell epitopes remains poorly characterized. Here, we determined the HLA-DR-restricted CD4+ T-cell epitopes of ADAMTS13...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751566/prolonged-versus-standard-native-e-coli-asparaginase-therapy-in-childhood-acute-lymphoblastic-leukemia-and-non-hodgkin-lymphoma-final-results-of-the-eortc-clg-randomized-phase-iii-trial-58951
#13
Veerle Mondelaers, Stefan Suciu, Barbara De Moerloose, Alina Ferster, Françoise Mazingue, Geneviève Plat, Karima Yakouben, Anne Uyttebroeck, Patrick Lutz, Vitor Costa, Nicolas Sirvent, Emmanuel Plouvier, Martine Munzer, Maryline Poirée, Odile Minckes, Frédéric Millot, Dominique Plantaz, Philip Maes, Claire Hoyoux, Hélène Cave', Pierre Rohrlich, Yves Bertrand, Yves Benoit
Asparaginase is an essential component of combination chemotherapy for childhood acute lymphoblastic leukemia and non-Hodgkin's lymphoma. The value of asparaginase was further addressed in a group of non-very high risk patients by comparing a prolonged (long-asparaginase) versus standard (short-asparaginase) native E.coli asparaginase treatment through a randomized question as part of the phase III 58951 trial of the European Organisation for Research and Treatment of Cancer Children's Leukemia Group. The main endpoint was disease-free survival...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751565/incidence-and-outcome-of-acquired-aplastic-anemia-real-world-data-from-patients-diagnosed-in-sweden-from-2000-2011
#14
Krista Vaht, Magnus Göransson, Kristina Carlson, Cecilia Isaksson, Stig Lenhoff, Anna Sandstedt, Bertil Uggla, Jacek Winiarski, Per Ljungman, Mats Brune, Per-Ola Andersson
Aplastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70-80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000-2011...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751564/increase-of-von-willebrand-factor-with-ageing-in-type-1-von-willebrand-disease-fact-or-fiction
#15
Mariachiara Borghi, Giuseppe Guglielmini, Anna Maria Mezzasoma, Emanuela Falcinelli, Loredana Bury, Marco Malvestiti, Paolo Gresele
No abstract text is available yet for this article.
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751563/calreticulin-as-a-novel-b-cell-receptor-antigen-in-chronic-lymphocytic-leukemia
#16
Elisa Ten Hacken, Maria Gounari, Jaap Willem Back, Ekaterina Shimanovskaya, Lydia Scarfo, Ekaterina Kim, Jared Burks, Maurilio Ponzoni, Giuseppe Alvise Ramirez, William G Wierda, Zeev Estrov, Michael J Keating, Alessandra Ferrajoli, Kostas Stamatopoulos, Paolo Ghia, Jan A Burger
No abstract text is available yet for this article.
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751562/impact-of-prior-therapy-on-the-efficacy-and-safety-of-oral-ixazomib-lenalidomide-dexamethasone-vs-placebo-lenalidomide-dexamethasone-in-patients-with-relapsed-refractory-multiple-myeloma-in-tourmaline-mm1
#17
Maria-Victoria Mateos, Tamas Masszi, Norbert Grzasko, Markus Hansson, Irwindeep Sandhu, Ludek Pour, Luísa Viterbo, Sharon R Jackson, Anne-Marie Stoppa, Peter Gimsing, Mehdi Hamadani, Gabriela Borsaru, Deborah Berg, Jianchang Lin, Alessandra Di Bacco, Helgi van de Velde, Paul G Richardson, Philippe Moreau
Prior treatment exposure in patients with relapsed/refractory multiple myeloma may affect outcomes with subsequent therapies. We analyzed efficacy and safety according to prior treatment in the phase 3 TOURMALINE-MM1 study of ixazomib-lenalidomide-dexamethasone (ixazomib-Rd) versus placebo-Rd. Patients with relapsed/refractory multiple myeloma received ixazomib-Rd or placebo-Rd. Efficacy and safety were evaluated in subgroups defined according to type (proteasome inhibitor [PI] and immunomodulatory drug) and number (1 vs 2 or 3) of prior therapies received...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751561/exome-sequencing-identifies-recurrent-bcor-gene-alterations-and-the-absence-of-klf2-tnfaip3-and-myd88-mutations-in-splenic-diffuse-red-pulp-small-b-cell-lymphoma
#18
Laurent Jallades, Lucile Baseggio, Pierre Sujobert, Sarah Huet, Kaddour Chabane, Evelyne Callet-Bauchu, Aurélie Verney, Sandrine Hayette, Jean-Pierre Desvignes, David Salgado, Nicolas Levy, Christophe Béroud, Pascale Felman, Françoise Berger, Jean-Pierre Magaud, Laurent Genestier, Gilles Salles, Alexandra Traverse-Glehen
Splenic diffuse red pulp lymphoma is an indolent small B-cell lymphoma recognised as a provisional entity in the WHO 2008 classification. Its precise relationship with other related splenic B-cell lymphomas with frequent leukaemic involvement or other lymphoproliferative disorders remains undetermined. We performed whole-exome sequencing to explore the genetic landscape of 10 splenic diffuse red pulp lymphoma cases from paired tumour and normal samples. A selection of 109 somatic mutations was then evaluated in a cohort including 42 splenic diffuse red pulp lymphoma samples and compared to those identified in 46 splenic marginal zone lymphoma and 8 hairy-cell leukaemia samples...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751560/mutational-status-of-ighv-is-the-most-reliable-prognostic-marker-in-trisomy-12-chronic-lymphocytic-leukemia
#19
Pietro Bulian, Riccardo Bomben, Michele Dal Bo, Antonella Zucchetto, Francesca Maria Rossi, Massimo Degan, Federico Pozzo, Tamara Bittolo, Vanessa Bravin, Tiziana D'Agaro, Michaela Cerri, Annalisa Chiarenza, Kari G Chaffee, Adalgisa Condoluci, Giovanni D'Arena, Michele Spina, Francesco Zaja, Gabriele Pozzato, Francesco Di Raimondo, Davide Rossi, Giovanni Del Poeta, Gianluca Gaidano, Tait D Shanafelt, Valter Gattei
No abstract text is available yet for this article.
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28751559/loss-of-function-but-not-dominant-negative-intragenic-ikzf1-deletions-are-associated-with-an-adverse-prognosis-in-adult-bcr-abl-negative-acute-lymphoblastic-leukemia
#20
Benjamin Kobitzsch, Nicola Gökbuget, Stefan Schwartz, Richard Reinhardt, Monika Brüggemann, Andreas Viardot, Ralph Wäsch, Michael Starck, Eckhard Thiel, Dieter Hoelzer, Thomas Burmeister
Genetic alterations of the transcription factor IKZF1 ("IKAROS") are detected in around 15-30% of cases of BCR-ABL-negative B-cell precursor acute lymphoblastic leukemia (ALL). Different types of intragenic deletions have been observed, resulting in a functionally inactivated allele ("loss-of-function") or in "dominant-negative" isoforms. The prognostic impact of these alterations especially in adult acute lymphoblastic leukemia is not well defined. We analyzed 482 well-characterized cases of adult BCR-ABL-negative B-precursor acute lymphoblastic leukemia uniformly treated in the framework of the GMALL studies and detected IKZF1 alterations in 128 cases (27%)...
July 27, 2017: Haematologica
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