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British Journal of Haematology

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https://www.readbyqxmd.com/read/29345306/hypoalbuminaemia-segregates-different-prognostic-subgroups-within-the-refined-standard-risk-acute-graft-versus-host-disease-score
#1
Mohamed A Kharfan-Dabaja, Kyle Sheets, Ambuj Kumar, Hemant S Murthy, Taiga Nishihori, Athanasios Tsalatsanis, Alain Mina, John Mathews, Ernesto Ayala, Julio Chavez, Lia E Perez, Brian C Betts, Claudio Anasetti, Joseph Pidala
Hypoalbuminaemia has been previously described to predict worse non-relapse mortality (NRM) and inferior overall survival (OS) in allogeneic haematopoietic cell transplant (allo-HCT) recipients. Here, we evaluate the role of hypoalbuminaemia (<35 g/l) at time of onset of acute graft-versus-host disease (aGVHD) when incorporated into the refined aGVHD score. The study population consisted of 522 patients, median age 53 (18-75) years, who underwent an allo-HCT mostly for haematological malignancies. Standard risk (SR) aGVHD comprised 467 patients (89%) and the number of high risk (HR) cases was 55 (11%)...
January 18, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29327472/mesenchymal-stem-cell-deficiency-influences-megakaryocytopoiesis-through-the-tnfaip3-nf-%C3%AE%C2%BAb-smad-pathway-in-patients-with-immune-thrombocytopenia
#2
Yun He, Lin-Lin Xu, Fei-Er Feng, Qian-Ming Wang, Xiao-Lu Zhu, Chen-Cong Wang, Jia-Min Zhang, Hai-Xia Fu, Lan-Ping Xu, Kai-Yan Liu, Xiao-Jun Huang, Xiao-Hui Zhang
Immune thrombocytopenia (ITP) is an autoimmune disease. Mesenchymal stem cells (MSCs) play important roles in the physiology and homeostasis of the haematopoietic system, including supporting megakaryocytic differentiation from CD34+ haematopoietic progenitor cells. Tumour necrosis factor alpha-induced protein 3 (TNFAIP3, also termed A20) plays a key role in terminating NF-κB signalling. Human genetic studies showed that the polymorphisms of the TNFAIP3 gene may contribute to ITP susceptibility. In this study, we showed a significant decrease in TNFAIP3 and increase in NF-κB/SMAD7 in ITP-MSCs...
January 12, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29318593/optimising-outcomes-for-patients-with-chronic-lymphocytic-leukaemia-on-ibrutinib-therapy-european-recommendations-for-clinical-practice
#3
John G Gribben, Francesc Bosch, Florence Cymbalista, Christian H Geisler, Paolo Ghia, Peter Hillmen, Carol Moreno, Stephan Stilgenbauer
Ibrutinib is indicated in Europe for the treatment of several B-cell malignancies, including chronic lymphocytic leukaemia (CLL). However, despite the high efficacy and favourable toxicity profile of ibrutinib, recent data suggest that it is not always administered optimally in clinical practice, with an increased tendency for dose reduction and a higher frequency of discontinuation. An expert panel of European haematologists was convened to identify practical issues pertinent to physicians involved in the therapeutic management of ibrutinib-treated CLL patients and here we outline the findings...
January 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29318591/immune-checkpoint-molecules-in-acute-myeloid-leukaemia-managing-the-double-edged-sword
#4
REVIEW
Willemijn Hobo, Tim J A Hutten, Nicolaas P M Schaap, Harry Dolstra
New immunotherapeutic interventions have revolutionized cancer treatment. The immune responsiveness of acute myeloid leukaemia (AML) was first demonstrated by allogeneic stem cell transplantation. In addition, milder immunotherapeutic approaches are exploited. However, the long-term efficacy of these therapies is hampered by various immune resistance and editing mechanisms. In this regard, co-inhibitory signalling pathways have been shown to play a crucial role. Via up-regulation of inhibitory checkpoints, tumour-reactive T cell and Natural Killer cell responses can be strongly impeded...
January 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29318588/microarray-based-comparative-genomic-hybridisation-reveals-additional-recurrent-aberrations-in-adult-patients-evaluated-for-myelodysplastic-syndrome-with-normal-karyotype
#5
LETTER
Ines Ouahchi, Luduo Zhang, Ramon Benitez Brito, Rudolf Benz, Rouven Müller, Nicolas Bonadies, Joëlle Tchinda
No abstract text is available yet for this article.
January 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29318584/treatment-of-relapsed-refractory-acute-myeloid-leukaemia-in-adults
#6
REVIEW
Armin Rashidi, Daniel J Weisdorf, Nelli Bejanyan
The prognosis of relapsed acute myeloid leukaemia (AML) is poor and treatment is challenging. While the most potent treatment modality for patients who achieve a complete remission after relapse is still allogeneic haematopoietic cell transplantation (allo-HCT), both transplant-related mortality and relapse rates are high and many patients are not candidates for this approach. After a few decades of relative stasis in this field, a large number of novel approaches have become available to tackle this highly fatal disease...
January 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29318576/comparing-transfusion-reaction-risks-for-various-plasma-products-an-analysis-of-7%C3%A2-years-of-istare-haemovigilance-data
#7
Nicholas H Saadah, Johanna G van der Bom, Johanna C Wiersum-Osselton, Clive Richardson, Rutger A Middelburg, Constantina Politis, Philippe Renaudier, Pierre Robillard, Martin R Schipperus
Plasma transfusions may result in transfusion reactions. We used the International Surveillance of Transfusion-Associated Reactions and Events (ISTARE) database, containing yearly reported national annual aggregate data on transfusion reactions from participating countries, to investigate risks of plasma transfusion reactions and compare transfusion reaction risks for different plasma types. We calculated risks for plasma transfusion reactions and compared transfusion reaction risks between plasma types using random effects regression on repeated measures...
January 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29318575/post-partum-haemorrhage-and-tranexamic-acid-a-global-issue
#8
REVIEW
Stephen P Hibbs, Ian Roberts, Haleema Shakur-Still, Beverley J Hunt
Post-partum haemorrhage (PPH) remains the major cause of maternal death worldwide, with the overwhelming majority of bleeding deaths occurring in low income countries. These bleeding deaths occur due to a complex network of biological and socioeconomic factors, including changes to haemostasis and fibrinolysis during pregnancy. Tranexamic acid (TxA) has been shown to reduce death in bleeding trauma patients safely and is effective in reducing bleeding in surgical patients, however its role in PPH has been less well established...
January 9, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29315496/tumour-genesis-syndrome-in-relapsed-b-lymphoblastic-leukaemia
#9
Caroline Shrubsole, Matthew Collin
No abstract text is available yet for this article.
January 7, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29315493/idealisib-rapidly-improves-platelet-function-tests-in-patients-with-chronic-lymphocytic-leukaemia
#10
LETTER
Gianluigi Reda, Ramona Cassin, Andrea Artoni, Bruno Fattizzo, Anna Lecchi, Silvia La Marca, Paolo Bucciarelli, Giorgia V Levati, Flora Peyvandi, Agostino Cortelezzi
No abstract text is available yet for this article.
January 7, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29315483/prothrombin-times-in-the-presence-of-edoxaban-in-vivo-experience-from-king-s-college-hospital
#11
LETTER
Jignesh P Patel, Paradzai B Chitongo, Preetam Dighe, Lara N Roberts, Bipin Vadher, Raj K Patel, Roopen Arya
No abstract text is available yet for this article.
January 7, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29315478/prognostic-indicators-in-primary-plasma-cell-leukaemia-a-multicentre-retrospective-study-of-117-patients
#12
Artur Jurczyszyn, Jakub Radocha, Julio Davila, Mark A Fiala, Alessandro Gozzetti, Norbert Grząśko, Paweł Robak, Iwona Hus, Anna Waszczuk-Gajda, Renata Guzicka-Kazimierczak, Erden Atilla, Giuseppe Mele, Waldemar Sawicki, David S Jayabalan, Grzegorz Charliński, Agoston G Szabo, Roman Hajek, Michel Delforge, Agnieszka Kopacz, Dorotea Fantl, Anders Waage, Irit Avivi, Marek Rodzaj, Xavier Leleu, Valentine Richez, Wanda Knopińska-Posłuszny, Anna Masternak, Andrew J Yee, Agnieszka Barchnicka, Agnieszka Druzd-Sitek, Thomas Guerrero-Garcia, Jieqi Liu, David H Vesole, Jorge J Castillo
We report a multicentre retrospective study that analysed clinical characteristics and outcomes in 117 patients with primary plasma cell leukaemia (pPCL) treated at the participating institutions between January 2006 and December 2016. The median age at the time of pPCL diagnosis was 61 years. Ninety-eight patients were treated with novel agents, with an overall response rate of 78%. Fifty-five patients (64%) patients underwent upfront autologous stem cell transplantation (ASCT). The median follow-up time was 50 months (95% confidence interval [CI] 33; 76), with a median overall survival (OS) for the entire group of 23 months (95% CI 15; 34)...
January 7, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29285764/paediatric-amendment-to-adult-bsh-guidelines-for-aplastic-anaemia
#13
LETTER
Sujith Samarasinghe, Paul Veys, Ajay Vora, Rob Wynn
No abstract text is available yet for this article.
December 28, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29270992/how-i-manage-patients-with-inherited-haemophilia-a-and-b-and-factor-inhibitors
#14
REVIEW
Rolf C R Ljung
Development of inhibitors to coagulation factor VIII or IX is still the most challenging complication in haemophilia care. 'Bypassing agents' may be used to treat a bleed but the eradication of the inhibitor by immune tolerance induction (ITI) is the main objective in the treatment of a patient with haemophilia who has developed neutralizing antibodies. Several options exist for ITI and the patient may be at 'good' or 'bad risk' for successful outcome with different regimens. This paper offers a review of current regimens to be considered in the treatment of a bleed in a patient with an inhibitor but the main focus is the aspects of different choices in the management of the child or the adult with severe or mild forms of haemophilia A or B, who has developed an inhibitor...
December 22, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29270982/a-comparison-of-minimal-residual-disease-detection-in-autografts-among-aso-qpcr-droplet-digital-pcr-and-next-generation-sequencing-in-patients-with-multiple-myeloma-who-underwent-autologous-stem-cell-transplantation
#15
LETTER
Hiroyuki Takamatsu, Rachel K Wee, Yoshitaka Zaimoku, Ryoichi Murata, Jianbiao Zheng, Martin Moorhead, Victoria E H Carlton, Katherine A Kong, Naoki Takezako, Shigeki Ito, Toshihiro Miyamoto, Kenji Yokoyama, Kosei Matsue, Tsutomu Sato, Toshiro Kurokawa, Hideo Yagi, Yasushi Terasaki, Kinya Ohata, Morio Matsumoto, Takashi Yoshida, Malek Faham, Shinji Nakao
No abstract text is available yet for this article.
December 22, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29271126/persistent-polyclonal-b-cell-lymphocytosis-two-nuclei-and-sometimes-more
#16
Coralie Derrieux, Emilie Klein
No abstract text is available yet for this article.
December 21, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29271057/pre-treatment-neutrophil-lymphocyte-ratio-and-platelet-lymphocyte-ratio-are-prognostic-of-progression-in-early-stage-classical-hodgkin-lymphoma
#17
Jay P Reddy, Mike Hernandez, Jillian R Gunther, Bouthaina S Dabaja, Geoffrey V Martin, Wen Jiang, Mani Akhtari, Pamela K Allen, Bradley J Atkinson, Grace L Smith, Chelsea C Pinnix, Sarah A Milgrom, Zeinab Abou Yehia, Eleanor M Osborne, Yasuhiro Oki, Hun Lee, Fredrick Hagemeister, Michelle A Fanale
To determine whether pre-treatment neutrophil/lymphocyte (NLR) or platelet/lymphocyte ratios (PLR) are predictive for progression in early-stage classical Hodgkin lymphoma (cHL), we derived NLR and PLR values for 338 stage I/II cHL patients and appropriate cut-off point values to define progression. Two-year freedom from progression (FFP) for patients with NLR ≥6·4 was 82·2% vs. 95·7% with NLR <6·4 (P < 0·001). Similarly, 2-year FFP was 84·3% for patients with PLR ≥266·2 vs. 96·1% with PLR <266·2 (P = 0·003)...
December 21, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29270985/inverse-correlation-of-v%C3%AE-2-t-cell-recovery-with-ebv-reactivation-after-haematopoietic-stem-cell-transplantation
#18
Jiangying Liu, Zhilei Bian, Xiaoyu Wang, Lan-Ping Xu, Qiang Fu, Chenguang Wang, Ying-Jun Chang, Yu Wang, Xiao-Hui Zhang, Zhengfan Jiang, Xiao-Jun Huang
Epstein-Barr virus (EBV) reactivation remains a life-threatening complication in recipients of a haploidentical haematopoietic stem cell transplantation (haploHSCT). Reconstitution of adaptive T lymphocytes is generally compromised at the early stages following transplant, suggesting an important role of other effector cells in preventing EBV infection. Our previous studies demonstrated that recovery of CD4- CD8- T cells negatively correlated with EBV reactivation after haploHSCT. In this prospective study on 132 adult patients with haematopoietic malignancy, recovery of T-cell subpopulations was characterized post-haploHSCT...
December 21, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29270984/g-protein-coupled-receptor-gpr34l-mutation-affects-thrombocyte-function-in-zebrafish
#19
Seongcheol Kim, Abdullah Alsrhani, Lala Zafreen, Gauri Khandekar, Florence L Marlow, Elliott W Abrams, Mary C Mullins, Pudur Jagadeeswaran
Haemostasis is a defence mechanism that has evolved to protect organisms from losing their circulating fluid. We have previously introduced zebrafish as a model to study the genetics of haemostasis to identify novel genes that play a role in haemostasis. Here, we identify a zebrafish mutant that showed prolonged time to occlusion (TTO) in the laser injury venous thrombosis assay. By linkage analysis and fine mapping, we found a mutation in the orphan G protein-coupled receptor 34 like gene (gpr34l) causing a change of Val to Glu in the third external loop of Gpr34l...
December 21, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29270983/recent-advances-in-understanding-the-pathogenesis-and-management-of-reticular-dysgenesis
#20
REVIEW
Manfred Hoenig, Ulrich Pannicke, Hubert B Gaspar, Klaus Schwarz
Reticular Dysgenesis is a rare immunodeficiency which is clinically characterized by the combination of Severe Combined Immunodeficiency (SCID) with agranulocytosis and sensorineural deafness. Mutations in the gene encoding adenylate kinase 2 (AK2) were identified to cause this phenotype. In this review, we will demonstrate important clinical differences between reticular dysgenesis and other SCID entities and summarize recent concepts in the understanding of the pathophysiology of the disease and the management strategies for this difficult condition...
December 21, 2017: British Journal of Haematology
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