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British Journal of Haematology

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https://www.readbyqxmd.com/read/30010189/reduced-regulatory-t-cells-treg-in-bone-marrow-preferentially-associate-with-the-expansion-of-cytotoxic-t-lymphocytes-in-low-risk-mds-patients
#1
LETTER
Angela Giovazzino, Stefania Leone, Valentina Rubino, Anna Teresa Palatucci, Giuseppe Cerciello, Fiorella Alfinito, Fabrizio Pane, Giuseppina Ruggiero, Giuseppe Terrazzano
No abstract text is available yet for this article.
July 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30010187/ruxolitinib-in-elderly-patients-with-myelofibrosis-impact-of-age-and-genotype-a-multicentre-study-on-291-elderly-patients
#2
Francesca Palandri, Lucia Catani, Massimiliano Bonifacio, Giulia Benevolo, Florian Heidel, Giuseppe A Palumbo, Monica Crugnola, Elisabetta Abruzzese, Daniela Bartoletti, Nicola Polverelli, Micaela Bergamaschi, Mario Tiribelli, Alessandra Iurlo, Massimo Breccia, Francesco Cavazzini, Alessia Tieghi, Gianni Binotto, Alessandro Isidori, Bruno Martino, Mariella D'Adda, Costanza Bosi, Elena Sabattini, Umberto Vitolo, Franco Aversa, Adalberto Ibatici, Roberto M Lemoli, Nicola Sgherza, Antonio Cuneo, Giovanni Martinelli, Giampietro Semenzato, Michele Cavo, Nicola Vianelli, Maria R Sapienza, Roberto Latagliata
Ruxolitinib is a JAK1/2 inhibitor that may control myelofibrosis (MF)-related splenomegaly and symptoms and can be prescribed regardless of age. While aging is known to correlate with worse prognosis, no specific analysis is available to confirm that ruxolitinib is suitable for use in older populations. A clinical database was created in 23 European Haematology Centres and retrospective data on 291 MF patients treated with ruxolitinib when aged ≥65 years were analysed in order to assess the impact of age and molecular genotype on responses, toxicities and survival...
July 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30010186/inherited-thpo-mutation-with-morphology-resembling-chronic-myeloid-leukaemia
#3
James W Murray, Indu Thakur, Philip P Connor
No abstract text is available yet for this article.
July 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30009455/guideline-for-the-treatment-of-chronic-lymphocytic-leukaemia-a-british-society-for-haematology-guideline
#4
LETTER
Anna H Schuh, Nilima Parry-Jones, Niamh Appleby, Adrian Bloor, Claire E Dearden, Christopher Fegan, George Follows, Christopher P Fox, Sunil Iyengar, Ben Kennedy, Helen McCarthy, Helen M Parry, Piers Patten, Andrew R Pettitt, Ingo Ringshausen, Renata Walewska, Peter Hillmen
No abstract text is available yet for this article.
July 15, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30004112/microdeletion-of-7p12-1p13-including-ikzf1-causes-intellectual-impairment-overgrowth-and-susceptibility-to-leukaemia
#5
LETTER
Tekla Järviaho, Vasilios Zachariadis, Bianca Tesi, Samuel Chiang, Yenan T Bryceson, Merja Möttönen, Riitta Niinimäki, Benedicte Bang, Elisa Rahikkala, Fulya Taylan, Johanna Uusimaa, Arja Harila-Saari, Ann Nordgren
No abstract text is available yet for this article.
July 13, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30004111/recipient-single-nucleotide-polymorphisms-in-paneth-cell-antimicrobial-peptide-genes-and-acute-graft-versus-host-disease-analysis-of-bmt-ctn-0201-and-0901-samples
#6
Armin Rashidi, Ryan Shanley, Sophia L Yohe, Bharat Thyagarajan, Julie Curtsinger, Claudio Anasetti, Edmund K Waller, Bart L Scott, Bruce R Blazar, Daniel J Weisdorf
Host genetics shape the gut microbiota, and gut dysbiosis increases the risk of acute graft-versus-host disease (aGVHD). Paneth cells and microbiota have interactions that contribute to immune regulation. α-defensin-5 (HD5) and regenerating islet-derived protein 3 alpha (Reg3A) are the most abundant Paneth cell antimicrobial peptides (AMPs). We hypothesized that single nucleotide polymorphisms (SNPs) in the genes for HD5 (DEFA5) and Reg3A (REG3A) predict aGVHD risk. We analysed pre-transplant recipient peripheral blood mononuclear cell samples from randomized Blood and Marrow Transplant Clinical Trials Network (BMT CTN) studies 0201 (94 patients with bone marrow and 93 with peripheral blood grafts) and 0901 (86 patients with myeloablative and 77 with reduced-intensity conditioning; all using peripheral blood grafts)...
July 13, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30004110/myelodysplastic-syndrome-mds-with-isolated-trisomy-8-a-type-of-mds-frequently-associated-with-myeloproliferative-features-a-report-by-the-groupe-francophone-des-my%C3%A3-lodysplasies
#7
Louis Drevon, Alice Marceau, Odile Maarek, Wendy Cuccuini, Emmanuelle Clappier, Virginie Eclache, Thomas Cluzeau, Valentine Richez, Inès Berkaoui, Sophie Dimicoli-Salazar, Audrey Bidet, Jean-Philippe Vial, Sophie Park, Christina Vieira Dos Santos, Eléonore Kaphan, Céline Berthon, Aspasia Stamatoullas, François Delhommeau, Nassera Abermil, Thorsten Braun, Rosa Sapena, Daniel Lusina, Aline Renneville, Lionel Adès, Sophie Raynaud, Pierre Fenaux
Isolated trisomy 8 (+8) is a frequent cytogenetic abnormality in the myelodysplastic syndromes (MDS), but its characteristics are poorly reported. We performed a retrospective study of 138 MDS patients with isolated +8, classified or reclassified as MDS (excluding MDS/myeloproliferative neoplasm). Myeloproliferative (MP) features were defined by the repeated presence of one of the following: white blood cell count >10 × 109 /l, myelemia (presence of circulating immature granulocytes with a predominance of more mature forms) >2%, palpable splenomegaly...
July 13, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984833/a-new-prognostic-model-for-myeloma-patients-relapsing-from-upfront-autologous-transplantation-based-on-iss-and-pfs1
#8
LETTER
Selina J Chavda, Paul M Maciocia, Pavlina Mesiri, Nicholas Counsell, Jaimal Kothari, Antonia Bird, Ciaran Mariner, Anastasia Chew, Katherine Clesham, Sally Moore, Laura Percy, Ali Rismani, Shirley D'Sa, Neil Rabin, Rakesh Popat, Kwee Yong
No abstract text is available yet for this article.
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984830/quality-of-life-during-and-following-sequential-treatment-of-previously-untreated-patients-with-multiple-myeloma-findings-of-the-medical-research-council-myeloma-ix-randomised-study
#9
Kara-Louise Royle, Walter M Gregory, David A Cairns, Sue E Bell, Gordon Cook, Roger G Owen, Mark T Drayson, Faith E Davies, Graham H Jackson, Gareth J Morgan, J Anthony Child
In the Medical Research Council (MRC) Myeloma IX trial (ISRCTN684564111) patients were randomised to sodium clodronate or zoledronic acid and induction treatment: cyclophosphamide, vincristine, doxorubicin and dexamethasone (CVAD) or cyclophosphamide, thalidomide and dexamethasone (CTD) followed by autologous stem cell transplant (ASCT) in the intensive pathway; attenuated CTD or melphalan and prednisolone (MP) in the non-intensive pathway. Subsequent randomisation allocated patients to either thalidomide or observation...
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984829/impairment-of-bone-marrow-endothelial-progenitor-cells-in-acute-graft-versus-host-disease-patients-after-allotransplant
#10
Xie-Na Cao, Yuan Kong, Yang Song, Min-Min Shi, Hong-Yan Zhao, Qi Wen, Zhong-Shi Lyu, Cai-Wen Duan, Yu Wang, Lan-Ping Xu, Xiao-Hui Zhang, Xiao-Jun Huang
Graft-versus-host disease (GVHD) is a major complication after allogeneic haematopoietic stem cell transplantation (allo-HSCT) that is frequently associated with bone marrow (BM) suppression, and clinical management is challenging. BM endothelial progenitor cells (EPCs) play crucial roles in the regulation of haematopoiesis and thrombopoiesis. However, little is known regarding the functional roles of BM EPCs in acute GVHD (aGVHD) patients. In the current prospective case-control study, reduced and dysfunctional BM EPCs, characterized by decreased migration and angiogenesis capacities and increased levels of reactive oxygen species (ROS) and apoptosis, were found in aGVHD patients compared with those without aGVHD...
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984828/overall-survival-of-children-and-adolescents-with-mature-b-cell-non-hodgkin-lymphoma-who-had-refractory-or-relapsed-disease-during-or-after-treatment-with-fab-lmb-96-a-report-from-the-fab-lmb-96-study-group
#11
Mitchell Cairo, Anne Auperin, Sherrie L Perkins, Ross Pinkerton, Lauren Harrison, Stanton Goldman, Catherine Patte
We determined the risk factors associated with poor survival in children and adolescents with de novo mature B cell non-Hodgkin lymphoma (B-NHL) who had refractory or relapsed disease during or after the French-American-British mature lymphoma B (FAB/LMB) 96 multi-agent chemotherapy. Among the 1 111 registered on study, 104 patients (9·4%) had refractory disease or disease relapse after first complete remission. Among these 104 patients, 28 (27%) patients had refractory disease and 76 (73%) had relapsed disease...
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984826/the-use-of-erythropoiesis-stimulating-agents-is-safe-and-effective-in-the-management-of-anaemia-in-myelofibrosis-patients-treated-with-ruxolitinib
#12
Elena Crisà, Daniela Cilloni, Elena M Elli, Vincenzo Martinelli, Giuseppe A Palumbo, Novella Pugliese, Eloise Beggiato, Chiara Frairia, Marco Cerrano, Giuseppe Lanzarone, Monia Marchetti, Mauro Mezzabotta, Mario Boccadoro, Dario Ferrero
Erythropoiesis-stimulating agents (ESAs) were combined with ruxolitinib in 59 anaemic myelofibrosis patients (93% with Dynamic International Prognostic Scoring System [DIPSS] intermediate-2/high risk; 52·5% transfusion-dependent). Anaemia response (AR) rate was 54% and 76% of patients responded at 5 years. A further 15% displayed minor improvement in anaemia and 78% of patients reduced spleen size. Endogenous erythropoietin levels <125 u/l correlated with a higher AR rate (63% vs. 20%, P = 0·008). No thrombotic events or other toxicities occurred...
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984825/high-dose-therapy-and-autologous-stem-cell-transplantation-in-marginal-zone-lymphomas-a-retrospective-study-by-the-ebmt-lymphoma-working-party-and-fil-gitmo
#13
Irit Avivi, Luca Arcaini, Virginia V Ferretti, Ariane Boumendil, Hervé Finel, Giuseppe Milone, Francesco Zaja, Devizzi Liliana, Maurizio Musso, Blaise Didier, Emmanuel Bachy, Mohammed Wattad, Emmanuelle Nicolas-Virelizier, Martin Gramatzki, Jean-Henri Bourhis, Denis Caillot, Anette Haenel, Gerhard Held, Catherine Thieblemont, Pavel Jindra, David Pohlreich, François Guilhot, Frank Kroschinsky, Björn Wahlin, Christof Scheid, Norbert Ifrah, Christian Berthou, Peter Dreger, Silvia Montoto, Annarita Conconi
The role of autologous stem cell transplantation (ASCT) in patients with marginal zone lymphoma (MZL) is debatable. This study investigated the outcome and prognostic factors affecting the outcome of patients undergoing ASCT for MZL. Eligible patients had non-transformed nodal, extra-nodal (MALT) or splenic MZL (SMZL), aged ≥18 years, who underwent a first ASCT between1994 and 2013 and were reported to the European Society for Blood and Marrow Transplantation, Fondazione Italiana Linfomi or Gruppo Italiano Trapianto Di Midollo Osseo registries...
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984823/outcome-of-haematopoietic-stem-cell-transplantation-in-dyskeratosis-congenita
#14
Francesca Fioredda, Simona Iacobelli, Elisabeth T Korthof, Cora Knol, Anja van Biezen, Dorine Bresters, Paul Veys, Ayami Yoshimi, Franca Fagioli, Brune Mats, Marco Zecca, Maura Faraci, Maurizio Miano, Luca Arcuri, Michael Maschan, Tracey O'Brien, Miguel A Diaz, Julian Sevilla, Owen Smith, Regis Peffault de Latour, Josue de la Fuente, Reuven Or, Maria T Van Lint, Jakub Tolar, Mahmoud Aljurf, Alain Fisher, Elena V Skorobogatova, Cristina Diaz de Heredia, Antonio Risitano, Jean-Hugues Dalle, Petr Sedláček, Ardeshir Ghavamzadeh, Carlo Dufour
Dyskeratosis congenita (DC) is a genetic multisystem disorder with frequent involvement of the bone marrow. Haematopoietic stem cell transplantation (HSCT) is the only definitive cure to restore haematopoiesis, even though it cannot correct other organ dysfunctions. We collected data on the outcome of HSCT in the largest cohort of DC (n = 94) patients ever studied. Overall survival (OS) and event-free survival (EFS) at 3 years after HSCT were 66% and 62%, respectively. Multivariate analysis showed better outcomes in patients aged less than 20 years and in patients transplanted from a matched, rather than a mismatched, donor...
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29984820/central-nervous-system-plasmablastic-lymphoma-evolving-from-polyclonal-plasmacytosis
#15
Umair T Khan, Igor Racu-Amoasii, Arvind Arumainathan, Ushma Meswani, Andrew R Pettitt, Geetha Menon
No abstract text is available yet for this article.
July 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29978577/relative-potency-of-the-thrombopoietin-receptor-agonists-eltrombopag-avatrombopag-and-romiplostim-in-a-patient-with-chronic-immune-thrombocytopenia
#16
Hanny Al-Samkari, David J Kuter
No abstract text is available yet for this article.
July 5, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29978498/outcomes-of-patients-with-simultaneous-diagnosis-of-chronic-lymphocytic-leukaemia-small-lymphocytic-lymphoma-and-multiple-myeloma
#17
LETTER
Sikander Ailawadhi, Bhagirathbhai R Dholaria, Sharad Khurana, Taimur Sher, Victoria Alegria, Aneel Paulus, Meghna Ailawadhi, Aditya Mehta, Asher Chanan-Khan, Vivek Roy
No abstract text is available yet for this article.
July 5, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29978459/the-dual-inhibitor-of-the-phosphoinositol-3-and-pim-kinases-ibl-202-is-effective-against-chronic-lymphocytic-leukaemia-cells-under-conditions-that-mimic-the-hypoxic-tumour-microenvironment
#18
Kyle Crassini, Yandong Shen, Michael O'Dwyer, Michael O'Neill, Richard Christopherson, Stephen Mulligan, O Giles Best
Despite significant advances in treatment, chronic lymphocytic leukaemia (CLL) remains an incurable disease. Ibrutinib and idelalisib, which inhibit Bruton Tyrosine kinase (BTK) and phosphoinositol-3 (PI3) kinase-δ respectively, have become important treatment options for the disease and demonstrate the potential of targeting components of the B-cell receptor-signalling pathway. IBL-202 is a dual inhibitor of the PIM and PI3 kinases. Synergy between the pan-PIM inhibitor, pPIMi, and idelalisib against a range of haematological cell lines and primary CLL cells supports the rationale for preclinical studies of IBL-202 in CLL...
July 5, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29978458/clinical-outcomes-and-survival-of-patients-with-myeloma-and-lymphoma-enrolled-into-phase-i-clinical-trials
#19
LETTER
Dima El-Sharkawi, Chin-Hin Ng, Elspeth M Payne, Kwee L Yong, Kirit M Ardeshna, Asim Khwaja, William Townsend, Rakesh Popat
No abstract text is available yet for this article.
July 5, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29978456/paediatric-patients-with-acute-leukaemia-and-kmt2a-mll-rearrangement-show-a-distinctive-expression-pattern-of-histone-deacetylases
#20
Nerea Vega-García, Roberta Malatesta, Camino Estella, Sara Pérez-Jaume, Elena Esperanza-Cebollada, Montserrat Torrebadell, Albert Català, Susanna Gassiot, Rubén Berrueco, Anna Ruiz-Llobet, Anna Alonso-Saladrigues, Montserrat Mesegué, Sandra Pont-Martí, Susana Rives, Mireia Camós
Histone deacetylase inhibitors (HDACi) had emerged as promising drugs in leukaemia, but their toxicity due to lack of specificity limited their use. Therefore, there is a need to elucidate the role of HDACs in specific settings. The study of HDAC expression in childhood leukaemia could help to choose more specific HDACi for selected candidates in a personalized approach. We analysed HDAC1-11, SIRT1, SIRT7, MEF2C and MEF2D mRNA expression in 211 paediatric patients diagnosed with acute leukaemia. There was a global overexpression of HDACs, while specific HDACs correlated with clinical and biological features, and some even predicted outcome...
July 5, 2018: British Journal of Haematology
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