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British Journal of Haematology

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https://www.readbyqxmd.com/read/28643460/periodic-limb-movement-in-sleep-and-sickle-cell-disease-a-neglected-association
#1
Ana C Cabañas-Pedro, Suely Roizenblatt, Altay A L de Souza, Sérgio Tufik, Maria S Figueiredo
High frequency of periodic limb movements in sleep (PLMS) has been described among children with sickle cell disease (SCD), but there is little information about PLMS among adults with SCD. We aim to determine the frequency of PLMS among adults with SCD and to identify possible associations with iron status and haemolytic parameters. We analysed polysomnography on 99 adults: 74 with sickle cell anaemia (HbSS), 19 with HbSC (double heterozygosis HbS and HbC) and 6 with HbS-beta thalassaemia. Laboratory data were collected close to the time of the polysomnography examination...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643426/cell-of-origin-classification-by-gene-expression-and-myc-rearrangements-in-diffuse-large-b-cell-lymphoma-of-children-and-adolescents
#2
Monika Szczepanowski, Jonas Lange, Christian W Kohler, Neus Masque-Soler, Martin Zimmermann, Sietse M Aukema, Michael Altenbuchinger, Thorsten Rehberg, Friederike Mahn, Reiner Siebert, Rainer Spang, Birgit Burkhardt, Wolfram Klapper
We present the largest series of diffuse large B-cell lymphoma (DLBCL) in patients younger than 18 years analysed to date by gene expression profiling using Nanostring technology to identify molecular subtypes and fluorescent in situ hybridization for translocations of MYC. We show that the activated B cell-like subtype of DLBCL is exceedingly rare in children and - in contrast to adults- not associated with outcome. Furthermore, we review the current literature and demonstrate that MYC translocations are not more frequent in paediatric compared to adult DLBCL...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643376/effect-of-n-acetylcysteine-on-pain-in-daily-life-in-patients-with-sickle-cell-disease-a-randomised-clinical-trial
#3
LETTER
Joep W R Sins, Karin Fijnvandraat, Anita W Rijneveld, Martine B Boom, Jean-Louis H Kerkhoffs, Alfred H van Meurs, Marco R de Groot, Harriët Heijboer, Marie-Françoise Dresse, Phu Quoc Lê, Philippe Hermans, Anna Vanderfaeillie, Eric W Van Den Neste, Fleur S Benghiat, Rachel Kesse-Adu, André Delannoy, André Efira, Marie-Agnès Azerad, Corianne A de Borgie, Bart J Biemond
No abstract text is available yet for this article.
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643365/a-multi-centre-phase-i-trial-of-the-parp-inhibitor-olaparib-in-patients-with-relapsed-chronic-lymphocytic-leukaemia-t-prolymphocytic-leukaemia-or-mantle-cell-lymphoma
#4
LETTER
Guy Pratt, Christina Yap, Ceri Oldreive, Daniel Slade, Rebecca Bishop, Mike Griffiths, Martin J S Dyer, Chris Fegan, David Oscier, Andrew Pettitt, Estella Matutes, Stephen Devereux, David Allsup, Adrian Bloor, Peter Hillmen, George Follows, Simon Rule, Paul Moss, Tatjana Stankovic
No abstract text is available yet for this article.
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643364/development-of-clinical-paroxysmal-nocturnal-haemoglobinuria-in-children-with-aplastic-anaemia
#5
Atsushi Narita, Hideki Muramatsu, Yusuke Okuno, Yuko Sekiya, Kyogo Suzuki, Motoharu Hamada, Shinsuke Kataoka, Daisuke Ichikawa, Rieko Taniguchi, Norihiro Murakami, Daiei Kojima, Eri Nishikawa, Nozomu Kawashima, Nobuhiro Nishio, Asahito Hama, Yoshiyuki Takahashi, Seiji Kojima
The clinical significance of paroxysmal nocturnal haemoglobinuria (PNH) in children with aplastic anaemia (AA) remains unclear. We retrospectively studied 57 children with AA between 1992 and 2010. During the follow-up, five patients developed clinical PNH, in whom somatic PIGA mutations were detected by targeted sequencing. The 10-year probability of clinical PNH development was 10·2% (95% confidence interval, 3·6-20·7%). Furthermore, the detection of minor PNH clones by flow cytometry at AA diagnosis was a risk factor for the subsequent development of clinical PNH...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643346/red-blood-cells-free-%C3%AE-haemoglobin-pool-a-biomarker-to-monitor-the-%C3%AE-thalassemia-intermedia-variability-the-alphapool-study
#6
Corinne Vasseur, Elisa Domingues-Hamdi, Katia Ledudal, Philippe Le Corvoisier, Caroline Barau, Bijan Ghaleh, Amandine Rialland, Serge Pissard, Frédéric Galactéros, Véronique Baudin-Creuza
The severity of β-thalassaemia (β-thal) intermedia is mainly correlated to the degree of imbalanced α/non α-globin chain synthesis. The phenotypic diversity of β-thal depends on this imbalance and reflects all possible combinations of α- and β-globin genotypes, levels of fetal haemoglobin (HbF) and co-inheritance of other modulating factors. This study aimed to demonstrate the validity of a new surrogate of α/non α-globin biosynthetic ratio by measuring the soluble α-Hb pool in lysed red blood cells...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643341/in-vivo-attraction-of-myeloma-cells-by-renal-cell-carcinoma
#7
Tsuyoshi Muta, Kenichi Aoki, Ryosuke Ogawa, Takakazu Sasaguri
No abstract text is available yet for this article.
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643330/muc1-c-is-a-target-in-lenalidomide-resistant-multiple-myeloma
#8
Li Yin, Ashujit Tagde, Reddy Gali, Yu-Tzu Tai, Teru Hideshima, Kenneth Anderson, David Avigan, Donald Kufe
Lenalidomide (LEN) acts directly on multiple myeloma (MM) cells by inducing cereblon-mediated degradation of interferon regulatory factor 4, Ikaros (IKZF)1 and IKZF3, transcription factors that are essential for MM cell survival. The mucin 1 (MUC1) C-terminal transmembrane subunit (MUC1-C) oncoprotein is aberrantly expressed by MM cells and protects against reactive oxygen species (ROS)-mediated MM cell death. The present studies demonstrate that targeting MUC1-C with GO-203, a cell-penetrating peptide inhibitor of MUC1-C homodimerization, is more than additive with LEN in downregulating the WNT/β-catenin pathway, suppressing MYC, and inducing late apoptosis/necrosis...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643326/pseudo-grey-platelet-syndrome-in-a-pregnant-patient
#9
Kiwa Tanaka, Mika Moriya, Tsuyoshi Hasegawa, Shuhei Okuyama, Tetsuya Ueki, Katsushi Tajima
No abstract text is available yet for this article.
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643324/factors-predicting-the-identification-of-new-organisms-in-follow-up-blood-cultures-drawn-during-episodes-of-neutropenic-sepsis
#10
LETTER
George S Heriot, Constantine Tam, Mary-Jo Waters
No abstract text is available yet for this article.
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643323/favourable-outcomes-of-poor-prognosis-diffuse-large-b-cell-lymphoma-patients-treated-with-dose-dense-rituximab-high-dose-methotrexate-and-six-cycles-of-chop-14-compared-to-first-line-autologous-transplantation
#11
Tim Strüßmann, Kristina Fritsch, Axel Baumgarten, Thomas Fietz, Monika Engelhardt, Roland Mertelsmann, Gabriele Ihorst, Justus Duyster, Jürgen Finke, Reinhard Marks
The optimal therapeutic approach for young diffuse large B-cell lymphoma (DLBCL) patients with high-intermediate and high-risk age-adjusted international prognostic index (aaIPI) remains unknown. Hereby we report a 10-year single-centre study of 63 consecutively treated patients. To optimize outcome, two approaches were carried out: Cohort 1 patients received four cycles R-CHOP-21 (rituximab, cyclophosphamide, daunorubicin, vincristine, prednisolone over 21 days) followed by first-line high-dose chemotherapy with autologous stem-cell support (HDCT-ASCT), resulting in 2-year progression-free (PFS) and overall survival (OS) of 60·6% and 67·9%...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28616874/romiplostim-monotherapy-in-thrombocytopenic-patients-with-myelodysplastic-syndromes-long-term-safety-and-efficacy
#12
Pierre Fenaux, Petra Muus, Hagop Kantarjian, Roger M Lyons, Richard A Larson, Mikkael A Sekeres, Pamela S Becker, Amelia Orejudos, Janet Franklin
Romiplostim can improve platelet counts in about 50% of patients with low- or intermediate 1-risk (lower risk) myelodysplastic syndromes (MDS) and thrombocytopenia, but its long-term toxicity and efficacy are not known. This open-label extension study evaluated the long-term safety and efficacy of romiplostim in 60 patients with lower risk MDS and platelet counts ≤50 × 10(9) /l. The primary endpoint was adverse event (AE) incidence. Secondary endpoints were efficacy parameters, including bleeding events and platelet response...
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28616864/a-phase-i-study-of-moxetumomab-pasudotox-in-adults-with-relapsed-or-refractory-b-cell-acute-lymphoblastic-leukaemia
#13
LETTER
Nicholas J Short, Hagop Kantarjian, Elias Jabbour, Jorge E Cortes, Deborah A Thomas, Michael E Rytting, Naval Daver, Yesid Alvarado, Marina Konopleva, Partow Kebriaei, William G Wierda, Courtney D DiNardo, Carol Bivins, Deborah McCue, Mary A Richie, Farhad Ravandi
No abstract text is available yet for this article.
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28612396/diagnosis-of-inherited-bleeding-disorders-in-the-genomic-era
#14
REVIEW
Suthesh Sivapalaratnam, Janine Collins, Keith Gomez
Inherited bleeding disorders affect between 1 in 1000 individuals for the most common disorder, von Willebrand Disease, to only 8 reported cases worldwide of alpha-2-antiplasmin deficiency. Those with an identifiable abnormality can be divided into disorders of coagulation factors (87%), platelet count and function (8%) and the fibrinolytic system (3%). Of the patients registered in the UK with a bleeding disorder, the remaining 2% are unclassifiable. In addition to bleeding symptoms, patients with an inherited bleeding disorder can manifest other abnormalities, making an accurate and complete diagnosis that reflects the underlying molecular pathology important...
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28612379/mccune-albright-syndrome-associated-bone-marrow-failure-and-extramedullary-haematopoeisis-secondary-to-fibrous-dysplasia
#15
Ali Jassem Mahdi, Phillip Connor, Indu Thakur
No abstract text is available yet for this article.
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28612370/platelet-transfusions-are-we-ready-to-chill-out
#16
EDITORIAL
Paul M Ness
No abstract text is available yet for this article.
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28597546/endothelin-1-receptor-blockade-as-new-possible-therapeutic-approach-in-multiple-myeloma
#17
Anna Russignan, Cecilia Spina, Nicola Tamassia, Adriana Cassaro, Antonella Rigo, Anna Bagnato, Laura Rosanò, Angela Bonalumi, Michele Gottardi, Lucia Zanatta, Alice Giacomazzi, Maria Teresa Scupoli, Martina Tinelli, Ugo Salvadori, Federico Mosna, Alberto Zamò, Marco A Cassatella, Fabrizio Vinante, Cristina Tecchio
New effective treatments are needed to improve outcomes for multiple myeloma (MM) patients. Receptors with restricted expression on plasma cells (PCs) represent attractive new therapeutic targets. The endothelin-1 (EDN1) axis, consisting of EDN1 acting through EDN-receptor A (EDNRA) and B (EDNRB), was previously shown to be overexpressed in several tumours, including MM. However, there is incomplete understanding of how EDN1 axis regulates MM growth and response to therapy. Besides EDNRA, the majority of MM cell lines and primary malignant PCs express high levels of EDNRB and release EDN1...
June 9, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28597542/the-efficacy-and-safety-of-gemcitabine-cisplatin-prednisone-thalidomide-versus-chop-in-patients-with-newly-diagnosed-peripheral-t-cell-lymphoma-with-analysis-of-biomarkers
#18
Ling Li, Wenjing Duan, Lei Zhang, Xin Li, Xiaorui Fu, Xinhua Wang, Jingjing Wu, Zhenchang Sun, Xudong Zhang, Yu Chang, Feifei Nan, Jiaqin Yan, Zhaoming Li, Ken H Young, Mingzhi Zhang
We compared the efficacy and safety of gemcitabine, cisplatin, prednisone and thalidomide (GDPT) with standard CHOP regimen (cyclophosphamide, doxorubicin, vincristine, prednisone) for patients with newly diagnosed peripheral T-cell lymphoma (PTCL) in a prospective randomized controlled and open-label clinical trial. Between July 2010 and June 2016, 103 patients were randomly allocated into two groups, of whom 52 were treated with GDPT therapy and 51 with CHOP therapy. The 2-year progression-free survival (PFS) and overall survival (OS) rates were better in the GDPT group than in the CHOP group (57% vs...
June 9, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28591463/immunosuppression-associated-kaposi-sarcoma-following-stem-cell-transplantation
#19
Andrew J Innes, Matthew Lee, Nicholas Francis, Eduardo Olavarria
No abstract text is available yet for this article.
June 7, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28591409/impact-of-concomitant-dexamethasone-dosing-schedule-on-bortezomib-induced-peripheral-neuropathy-in-multiple-myeloma
#20
Shaji K Kumar, Jacob P Laubach, Thomas J Giove, Maureen Quick, Rachel Neuwirth, Godwin Yung, S Vincent Rajkumar, Paul G Richardson
Peripheral neuropathy (PN) is the most troublesome adverse event associated with the proteasome inhibitor bortezomib. Studies suggest an inflammatory aetiology for bortezomib-induced PN (BiPN) and it has been hypothesized that reducing inflammation with concomitant dexamethasone may reduce BiPN incidence and/or severity. We retrospectively analysed PN rates from 32 studies (2697 patients with previously untreated multiple myeloma) incorporating bortezomib and differing dexamethasone schedules: partnered dosing (days of and after bortezomib), weekly dosing, and other dosing schedules (e...
June 7, 2017: British Journal of Haematology
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