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British Journal of Haematology

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https://www.readbyqxmd.com/read/28905372/therapeutic-outcomes-using-subcutaneous-low-dose-alemtuzumab-for-acquired-bone-marrow-failure-conditions
#1
LETTER
Swapna Thota, Bhumika J Patel, Meena Sadaps, Suresh Balasubramanian, Srinivasa Sanikommu, Cassandra Hirsch, Serena Marotta, Mikkael A Sekeres, Antonio M Risitano, Jaroslaw P Maciejewski
No abstract text is available yet for this article.
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28905368/interim-fdg-pet-has-no-value-in-selecting-patients-who-require-treatment-modification-in-both-early-and-advanced-stage-hodgkin-lymphoma
#2
LETTER
Hugo J A Adams, Thomas C Kwee
No abstract text is available yet for this article.
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28905365/iron-storage-in-liver-bone-marrow-and-splenic-gaucheroma-reflects-residual-disease-in-type-1-gaucher-disease-patients-on-treatment
#3
Martine Regenboog, Anneloes E Bohte, Erik M Akkerman, Jaap Stoker, Carla E M Hollak
Gaucher disease (GD) is a lysosomal storage disorder characterized by the storage of glycosphingolipids in macrophages. Despite effective therapy, residual disease is present in varying degrees and may be associated with late complications, such as persistent bone or liver disease and increased cancer risk. Gaucher macrophages are capable of storing iron and locations of residual disease may thus be detectable with iron imaging. Forty type 1 GD (GD1) patients and 40 matched healthy controls were examined using a whole-body magnetic resonance imaging protocol consisting of standard sequences, allowing analysis of iron content per organ, expressed as R2* (Hz)...
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28905364/a-clinical-risk-score-for-pulmonary-artery-thrombosis-during-acute-chest-syndrome-in-adult-patients-with-sickle-cell-disease
#4
Anaïs Winchenne, Jérôme Cecchini, Jean-François Deux, Nicolas De Prost, Keyvan Razazi, Guillaume Carteaux, Frederic Galacteros, Anoosha Habibi, Pablo Bartolucci, Giovanna Melica, Mehdi Khellaf, Marc Michel, Bernard Maitre, Armand Mekontso Dessap
Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score...
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28891211/a-clinical-trial-combining-megakaryocytes-and-haematopoietic-stem-cells-to-promote-engraftment-after-autologous-transplantation
#5
LETTER
Helene Trebeden-Negre, Sylvain Choquet, Marie-Laure Tanguy, Michelle Rozenzwajg, Nabih Azar, Francois Lefrère, Farhad Heshmati, Ramdane Belhocine, Vincent Vieillard, Francoise Norol
No abstract text is available yet for this article.
September 10, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28891074/nelarabine-toxicity-in-children-and-adolescents-with-relapsed-refractory-t-all-t-lbl-can-we-avoid-throwing-the-baby-out-with-the-bathwater
#6
EDITORIAL
Andrea Malone, Owen P Smith
No abstract text is available yet for this article.
September 10, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28880377/detection-of-clinically-relevant-early-genomic-lesions-in-b-cell-malignancies-from-circulating-tumour-dna-using-a-single-hybridisation-based-next-generation-sequencing-assay
#7
LETTER
Piers A Blombery, Georgina L Ryland, John Markham, Jerick Guinto, Meaghan Wall, Michelle McBean, Kate Jones, Ella R Thompson, Daniel L Cameron, Anthony T Papenfuss, Miles H Prince, Michael Dickinson, David Alan Westerman
No abstract text is available yet for this article.
September 7, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28880374/skeletal-and-myocardial-microvascular-blood-flow-in-hydroxycarbamide-treated-patients-with-sickle-cell-disease
#8
Vandana Sachdev, Stanislav Sidenko, Melinda D Wu, Caterina P Minniti, Hwaida Hannoush, Cynthia L Brenneman, Myron A Waclawiw, Andrew E Arai, Alan N Schechter, Gregory J Kato, Jonathan R Lindner
In sickle cell disease (SCD), abnormal microvascular function combined with chronic anaemia predisposes patients to perfusion-demand mismatch. We hypothesized that skeletal muscle and myocardial perfusion, normalized to the degree of anaemia, is reduced at basal-state compared to controls, and that this defect is ameliorated by hydroxycarbamide (HC; also termed hydroxyurea) therapy. Twenty-one SCD patients, of whom 15 were treated with HC, and 27 controls underwent contrast-enhanced ultrasound (CEU) perfusion imaging of the forearm as well as the myocardium...
September 7, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28880367/cytokeratin-positive-primary-effusion-lymphoma-a-diagnostic-challenge
#9
Feisal Yamani, Weina Chen
No abstract text is available yet for this article.
September 7, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28875547/immunohistochemical-double-hit-score-enhances-nccn-ipi-and-is-associated-with-detrimental-outcomes-in-refractory-or-relapsing-patients-with-diffuse-large-b-cell-lymphoma
#10
LETTER
Katharina T Prochazka, Florian Posch, Alexander Deutsch, Christine Beham-Schmid, Herbert Stöger, Leke Abdyli, Hildegard Greinix, Martin Pichler, Peter Neumeister
No abstract text is available yet for this article.
September 6, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28875503/congenital-neutropenia-in-the-era-of-genomics-classification-diagnosis-and-natural-history
#11
REVIEW
Jean Donadieu, Blandine Beaupain, Odile Fenneteau, Christine Bellanné-Chantelot
This review focuses on the classification, diagnosis and natural history of congenital neutropenia (CN). CN encompasses a number of genetic disorders with chronic neutropenia and, for some, affecting other organ systems, such as the pancreas, central nervous system, heart, bone and skin. To date, 24 distinct genes have been associated with CN. The number of genes involved makes gene screening difficult. This can be solved by next-generation sequencing (NGS) of targeted gene panels. One of the major complications of CN is spontaneous leukaemia, which is preceded by clonal somatic evolution, and can be screened by a targeted NGS panel focused on somatic events...
September 6, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857136/results-of-a-multicentre-uk-wide-retrospective-study-evaluating-the-efficacy-of-brentuximab-vedotin-in-relapsed-refractory-classical-hodgkin-lymphoma-in-the-transplant-naive-setting
#12
Toby A Eyre, Elizabeth H Phillips, Kim M Linton, Shireen Kassam, Adam Gibb, Suzanne Allibone, John Radford, Karl Peggs, Cathy Burton, Gillian Stewart, Rifca LeDieu, Catherine Booth, Wendy L Osborne, Fiona Miall, David W Eyre, Kirit M Ardeshna, Graham P Collins
Relapsed or refractory classical Hodgkin lymphoma (cHL) is associated with a poor outcome when standard chemotherapy fails. Brentuximab vedotin (BV) is an anti-CD30 monoclonal antibody-drug conjugate licensed for use at relapse after autologous stem cell transplant (ASCT) or following two prior therapies in those unsuitable for ASCT. There are limited data assessing the ability of BV to enable curative SCT. We performed a UK-wide retrospective study of 99 SCT-naïve relapsed/refractory cHL. All had received 2 prior lines and were deemed fit for transplant but had an insufficient remission to proceed...
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857132/inflammatory-mediators-in-sickle-cell-anaemia-highlight-the-difference-between-steady-state-and-crisis-in-paediatric-patients
#13
LETTER
Magda O S Carvalho, Théo Araujo-Santos, João H O Reis, Larissa C Rocha, Bruno A V Cerqueira, Nívea F Luz, Isa M Lyra, Valma M Lopes, Cynara G Barbosa, Luciana M Fiuza, Rayra P Santiago, Camylla V B Figueiredo, Caroline C da Guarda, Manoel Barral Neto, Valéria M Borges, Marilda S Gonçalves
No abstract text is available yet for this article.
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857128/lessons-learned-from-a-review-of-paroxysmal-nocturnal-haemoglobinuria-pnh-requests-a-report-from-the-uk-pnh-network
#14
LETTER
Morag Griffin, Ian Neilly, Manos Nikolousis, Srinivasan Narayanan, Katharine Lowndes, Mickey Koh, Jose Ros, Wendy Ingram, Steven Couzens, Richard Karim, Lowri Morgan, Mary McMullin, Narind Sharma, Lindsay Mitchell, Mark Layton, Patrick Medd, Shikha Chattree, Anita Hill
No abstract text is available yet for this article.
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857127/empty-marrow-following-previous-irradiation-in-a-patient-with-therapy-related-myelodysplastic-syndrome
#15
Chun-Hui Lee, Tsai-Yun Chen
No abstract text is available yet for this article.
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857126/effective-treatment-of-chemotherapy-induced-atypical-haemolytic-uraemic-syndrome-a-case-series-of-7-treated-patients
#16
LETTER
Ilene C Weitz, Thomas Deloughery
No abstract text is available yet for this article.
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857122/course-of-anti-alk-antibody-titres-during-chemotherapy-in-children-with-anaplastic-large-cell-lymphoma
#17
LETTER
Lara Mussolin, Marta Pillon, Martin Zimmermann, Elisa Carraro, Giuseppe Basso, Fabian Knoerr, Wilhelm Woessmann, Christine Damm-Welk
No abstract text is available yet for this article.
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857120/impact-of-genetic-variants-on-haematopoiesis-in-patients-with-thrombocytopenia-absent-radii-tar-syndrome
#18
Georgi Manukjan, Hendrik Bösing, Markus Schmugge, Gabriele Strauß, Harald Schulze
Thrombocytopenia absent radii (TAR) syndrome is clearly defined by the combination of radial aplasia and reduced platelet counts. The genetics of TAR syndrome has recently been resolved and comprises a microdeletion on Chromosome 1 including the RBM8A gene and a single nucleotide polymorphism (SNP) either at the 5' untranslated region (5'UTR) or within the first intron of RBM8A. Although phenotypically readily diagnosed after birth, the genetic determination of particular SNPs in TAR syndrome harbours valuable information to evaluate disease severity and treatment decisions...
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28857117/failure-to-achieve-early-disease-response-is-associated-with-inferior-survival-in-patients-with-newly-diagnosed-multiple-myeloma
#19
LETTER
Adrian Chee, Michael S Low, Shahla Vilcassim, Gareth P Gregory, Michael Gilbertson, Sumita Ratnasingam, George Grigoriadis, Pasquale L Fedele
No abstract text is available yet for this article.
August 31, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28850697/a-longitudinal-evaluation-of-health-related-quality-of-life-in-patients-with-al-amyloidosis-associations-with-health-outcomes-over-time
#20
Vaishali Sanchorawala, Kristen L McCausland, Michelle K White, Martha S Bayliss, Spencer D Guthrie, Stephen Lo, Martha Skinner
Light chain (AL) amyloidosis is a rare disease associated with significant, irreversible organ dysfunction and high case fatality. An observational study was conducted to assess health-related quality of life (HRQoL) in patients treated for AL amyloidosis between 1994 and 2014 with both high dose melphalan and stem cell transplantation (HDM/SCT) or non-SCT chemotherapy regimens. The SF-36v1(®) Health Survey (SF-36) was administered to assess HRQoL during clinic visits. Analysis of variance was used to compare pre- and post-treatment HRQoL within each treatment group to an age- and gender-adjusted general population (GP) normative sample...
August 29, 2017: British Journal of Haematology
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