keyword
https://read.qxmd.com/read/38485153/understanding-complex-disease-related-mechanisms-rational-therapies-for-diamond-blackfan-anaemia
#21
JOURNAL ARTICLE
Jeffrey M Lipton
The rich history surrounding Diamond-Blackfan anaemia (DBA), originally described in 1938 as congenital hypoplastic anaemia2 reflects the evolution of paediatric haematology. In their paper, the authors1 present the results of a clinical trial using the thrombopoietin-mimetic agent eltrombopag to treat red cell failure in DBA. A low response rate belies the importance of this work. Commentary on: Duncan et al. Treatment of refractory/relapsed Diamond-Blackfan anaemia with eltrombopag. Br J Haematol 2024 (Online ahead of print)...
March 14, 2024: British Journal of Haematology
https://read.qxmd.com/read/38485116/infection-risk-and-antimicrobial-prophylaxis-in-bendamustine-treated-patients-with-indolent-non-hodgkin-lymphoma-an-australasian-lymphoma-alliance-study
#22
JOURNAL ARTICLE
Kate Manos, Leonid Churilov, Andrew Grigg, Pietro Di Ciaccio, Jonathan Wong, Usha Chandra Sekaran, Joel Wight, Zhong Goh, Hayden Jina, Llewyn Butler, Costas K Yannakou, Nada Hamad, Gareth P Gregory, Shane Gangatharan, Tara Cochrane, Eliza A Hawkes, Masa Lasica
Infection and lymphopenia are established bendamustine-related complications. The relationship between lymphopenia severity and infection risk, and the role of antimicrobial prophylaxis, is not well described. This multicentre retrospective study analysed infection characteristics and antimicrobial prophylaxis in 302 bendamustine-treated indolent non-Hodgkin lymphoma patients. Lymphopenia (<1 × 109 /L) was near universal and time to lymphocyte recovery correlated with cumulative bendamustine dose...
March 14, 2024: British Journal of Haematology
https://read.qxmd.com/read/38479427/refining-risk-stratification-in-paediatric-b-acute-lymphoblastic-leukaemia-combining-ikzf1-plus-and-day-15-mrd-positivity
#23
JOURNAL ARTICLE
Hsi-Che Liu, Ying-Jung Huang, Tang-Her Jaing, Kang-Hsi Wu, Shih-Hsiang Chen, Shih-Chung Wang, Ting-Chi Yeh, Chih-Cheng Hsiao, Te-Kau Chang, Hsiu-Ju Yen, Fang-Liang Huang, Pei-Chin Lin, Jen-Yin Hou, Jiunn-Ming Sheen, Yu-Mei Liao, Tsung-Yen Chang, Yu-Chieh Chen, Shyh-Shin Chiou, Chao-Ping Yang, Ching-Hon Pui, Der-Cherng Liang, Lee-Yung Shih
This study investigates the potential utility of IKZF1 deletion as an additional high-risk marker for paediatric acute lymphoblastic leukaemia (ALL). The prognostic impact of IKZF1 status, in conjunction with minimal/measurable residual disease (MRD), was evaluated within the MRD-guided TPOG-ALL-2013 protocol using 412 newly diagnosed B-ALL patients aged 1-18. IKZF1 status was determined using multiplex ligation-dependent probe amplification. IKZF1 deletions, when co-occurring with CDKN2A, CDKN2B, PAX5 or PAR1 region deletions in the absence of ERG deletions, were termed IKZF1plus ...
March 13, 2024: British Journal of Haematology
https://read.qxmd.com/read/38471752/splinter-haemorrhages-in-a-patient-with-systemic-mastocytosis-and-hypereosinophilia
#24
JOURNAL ARTICLE
Mar Rodríguez-Troncoso, José María Llamas-Molina, Alberto Benavente-Fernández, María Narváez-Simón, Ricardo Ruiz-Villaverde
No abstract text is available yet for this article.
March 12, 2024: British Journal of Haematology
https://read.qxmd.com/read/38471714/mixed-chimerism-post-allogeneic-stem-cell-transplant-for-chediak-higashi-syndrome-clues-from-morphology-and-blood%C3%A2-banking
#25
JOURNAL ARTICLE
Sean McKeague, Kacey O'Rourke, Rebecca Adams, Yasmin Harvey, Tee Beng Keng, Glen Kennedy
No abstract text is available yet for this article.
March 12, 2024: British Journal of Haematology
https://read.qxmd.com/read/38471666/left-ventricular-global-longitudinal-strain-and-acute-myocardial-injury-in-patients-with-sickle-cell-disease-admitted-to-the-intensive-care-unit-for-vaso-occlusive-crisis
#26
JOURNAL ARTICLE
Guillaume Roger, Pierre Denormandie, Thibaut Gobe, Danila Azzolina, Tài Pham, Christelle Chantalat, Daphnée Cuveillier, Amir Bouchachi, Patrick Jourdain, Christopher Lai, Arthur Pavot, Nicolas Fage, Paul Domnariu, Jean-Louis Teboul, Xavier Monnet
In patients with sickle cell disease (SCD), SCD-related cardiomyopathy may be partly due to repeated ischaemic events related to sickling during vaso-occlusive crises, but few clinical studies support this hypothesis. We evaluated the incidence of acute myocardial ischaemia during vaso-occlusive crises as assessed by the left ventricular global longitudinal strain (LVGLS) and high-sensitive cardiac troponin T (hs-cTnT). We included adult patients with SCD admitted to the intensive care unit (ICU) for vaso-occlusive crisis...
March 12, 2024: British Journal of Haematology
https://read.qxmd.com/read/38471663/successful-treatment-of-scleromyxoedema-with-daratumumab
#27
JOURNAL ARTICLE
Katharina Epp, Raphael Teipel, Ulrike Reuner, Martin Bornhäuser, Claudia Günther, Karolin Trautmann-Grill
No abstract text is available yet for this article.
March 12, 2024: British Journal of Haematology
https://read.qxmd.com/read/38471658/intravascular-large-b-cell-lymphoma-presenting-with-haemophagocytic-syndrome
#28
JOURNAL ARTICLE
Pedro Baptista, Eliana Aguiar, Elsa Fonseca, Ricardo Pinto, Fernanda Trigo
No abstract text is available yet for this article.
March 12, 2024: British Journal of Haematology
https://read.qxmd.com/read/38471524/dna-methylation-profiling-of-myelodysplastic-syndromes-and%C3%A2-clinical-response-to-azacitidine-a-multicentre-retrospective-study
#29
JOURNAL ARTICLE
Aleix Noguera-Castells, Ignacio Campillo-Marcos, Veronica Davalos, Carlos A García-Prieto, David Valcárcel, Antonieta Molero, Laura Palomo, Norbert Gattermann, Michael Wulfert, Lorea Chaparro-González, Francesc Solé, Marta Cabezón, María J Jiménez-Lorenzo, Blanca Xicoy, Lurdes Zamora, Alessia De Stefano, Irene Casalin, Carlo Finelli, Matilde Y Follo, Manel Esteller
Real-world data have revealed that a substantial portion of patients with myelodysplastic syndromes (MDS) does not respond to epigenetic therapy with hypomethylating agents (HMAs). The cellular and molecular reasons for this resistance to the demethylating agent and biomarkers that would be able to predict the treatment refractoriness are largely unknown. In this study, we shed light on this enigma by characterizing the epigenomic profiles of patients with MDS treated with azacitidine. Our approach provides a comprehensive view of the evolving DNA methylation architecture of the disease and holds great potential for advancing our understanding of MDS treatment responses to HMAs...
March 12, 2024: British Journal of Haematology
https://read.qxmd.com/read/38462984/capture-based-targeted-sequencing-using-a-t-cell-control-in-myeloid-malignancies-and-idiopathic-cytopenias
#30
JOURNAL ARTICLE
Grzegorz Pietka, Corinne De Lord, Gwynn Matthias, Betty Cheung, Sangeeta Atwal, Michelle Furtado, Jonathan Cullis, Liz Grey-Davies, Srinivasan Narayanan, Andrew McGregor, Mari Kilner, Jenny Bosworth, Mary Frances McMullin, Thomas Coats, Agapi Parcharidou, Jamie Cavenagh, Jenny Byrne, Sunil Iyengar, Kabir Mohammed, Nicholas Cross, Mike Hubank, Sara Ribeiro, Jamshid Khorashad, Dorte Wren, Simon O'Connor, David Taussig
We report on a study of next-generation sequencing in 257 patients undergoing investigations for cytopenias. We sequenced bone marrow aspirates using a target enrichment panel comprising 82 genes and used T cells from paired blood as a control. One hundred and sixty patients had idiopathic cytopenias, 81 had myeloid malignancies and 16 had lymphoid malignancies or other diagnoses. Forty-seven of the 160 patients with idiopathic cytopenias had evidence of somatic pathogenic variants consistent with clonal cytopenias...
March 11, 2024: British Journal of Haematology
https://read.qxmd.com/read/38462771/identification-of-coq2-as-a-regulator-of-proliferation-and-lipid-peroxidation-through-genome-scale-crispr-cas9-screening-in-myeloma-cells
#31
JOURNAL ARTICLE
Miao Li, Chang-Lin Zhang, Di-Sheng Zhou, Sze-Hoi Chan, Xue-Qi Liu, Shu-Na Chen, Zi-Yi Yang, Fei-Er Ju, Xiao-Yan Sang, Zi-Xuan Liu, Qiao-Xia Zhang, Yu-Ming Pan, Si-Si Deng, Xiao-Mei Wang, Li Zhong, Xing-Ding Zhang, Xin Du
Multiple myeloma (MM) is the second most common malignant haematological disease with a poor prognosis. The limit therapeutic progress has been made in MM patients with cancer relapse, necessitating deeper research into the molecular mechanisms underlying its occurrence and development. A genome-wide CRISPR-Cas9 loss-of-function screening was utilized to identify potential therapeutic targets in our research. We revealed that COQ2 plays a crucial role in regulating MM cell proliferation and lipid peroxidation (LPO)...
March 10, 2024: British Journal of Haematology
https://read.qxmd.com/read/38462764/treatment-of-refractory-relapsed-diamond-blackfan-anaemia-with-eltrombopag
#32
JOURNAL ARTICLE
Brynn B Duncan, Jennifer L Lotter, Jeanine Superata, Ma Evette Barranta, Tania Machado, Ivana Darden, Sanjay Venugopal, Colin O Wu, Janis L Abkowitz, Cynthia E Dunbar, David J Young
Diamond-Blackfan anaemia (DBA) is a rare, inherited bone marrow failure syndrome with a ribosomal defect causing slowed globin chain production with normal haem synthesis, causing an overabundance of reactive iron/haem and erythroid-specific cellular toxicity. Eltrombopag, a non-peptide thrombopoietin receptor agonist, is a potent intracellular iron chelator and induced a robust durable response in an RPS19-mutated DBA patient on another trial. We hypothesized eltrombopag would improve RBC production in DBA patients...
March 10, 2024: British Journal of Haematology
https://read.qxmd.com/read/38448009/impact-of-revised-international-staging-system-2-risk-stratification-on-outcomes-of-patients-with-multiple-myeloma-receiving-autologous-haematopoietic-stem-cell-transplantation
#33
JOURNAL ARTICLE
Kamal Alzahrani, Oren Pasvolsky, Zhongya Wang, Denái R Milton, Mark R Tanner, Qaiser Bashir, Samer Srour, Neeraj Saini, Paul Lin, Jeremy Ramdial, Yago Nieto, Hans C Lee, Krina K Patel, Elisabet E Manasanch, Partow Kebriaei, Sheeba K Thomas, Donna M Weber, Robert Z Orlowski, Elizabeth J Shpall, Richard Champlin, Muzaffar H Qazilbash
The second revision of the International Staging System (R2-ISS) is a simple tool to risk-stratify newly diagnosed multiple myeloma (NDMM) patients. Here, we completed a retrospective analysis to evaluate the utility of R2-ISS in NDMM patients who underwent up-front autologous haematopoietic stem cell transplantation (auto-HCT). A total of 1291 patients were included, with a median age of 62 years (range 29-83). The distribution of R2-ISS stages was: 123 (10%) stage I, 471 (36%) stage II, 566 (44%) stage III and 131 (10%) stage IV...
March 6, 2024: British Journal of Haematology
https://read.qxmd.com/read/38447995/epidemiological-features-and-prognosis-for-primary-gastrointestinal-follicular-lymphoma
#34
JOURNAL ARTICLE
Xuelei Li, Fenghua Gao, Xiangrui Meng, Xiaoyan Zhang, Cong Sun, Hengqi Liu, Jingwei Yu, Xia Liu, Xue Han, Lanfang Li, Lihua Qiu, Zhengzi Qian, Shiyong Zhou, Wenchen Gong, Zahra Golchehre, Zahra Chavoshzadeh, Xiubao Ren, Xianhuo Wang, Huilai Zhang
Primary gastrointestinal follicular lymphoma (PGI-FL) is a rare extra-nodal lymphoma. Its epidemiology and prognosis remain unclear. We performed a retrospective analysis of eligible patients with 1648 PGI-FL and 34 892 nodal FL (N-FL) in the Surveillance, Epidemiology and End Results (SEER) database. The age-adjusted average annual incidence of PGI-FL was 0.111/100000. The median overall survival (OS) for PGI-FL and N-FL patients was 207 and 165 months respectively. The 5-year diffuse large B-cell lymphoma (DLBCL) transformation rates were 2...
March 6, 2024: British Journal of Haematology
https://read.qxmd.com/read/38444113/relevance-of-cd20-antigen-expression-among-paediatric-patients-with-b-lineage-acute-lymphoblastic-leukaemia
#35
JOURNAL ARTICLE
Karthik Bommannan, Jhansi Rani Arumugam, Venkatraman Radhakrishnan, Shirley Sundersingh
Literature regarding prognostic relevance of CD20 antigen expression among paediatric B-lineage acute lymphoblastic leukaemia (B-ALL) patients is sparse and contradictory. We analysed clinical laboratory parameters and survival characteristics pertinent to CD20 expression among 224 treatment-naïve paediatric B-ALL patients. 50% patients had CD20 expression (CD20+ B-ALL). There was no difference in the clinical & laboratory presentation and end of induction measurable residual disease (EOI-MRD) status according to CD20 expression...
March 5, 2024: British Journal of Haematology
https://read.qxmd.com/read/38442908/cxcr4-whim-syndrome-is-a-cancer-predisposition-condition-for-virus-induced-malignancies
#36
JOURNAL ARTICLE
Clémentine Moulin, Blandine Beaupain, Felipe Suarez, Yves Bertrand, Sarah Cohen Beaussant, Alain Fischer, Julie Durin, Dana Ranta, Marion Espéli, Françoise Bachelerie, Christine Bellanné-Chantelot, Thierry Molina, Jean François Emile, Karl Balabanian, Claire Deback, Jean Donadieu
Warts, hypogammaglobulinaemia, infections and myelokathexis syndrome (WHIMS) is a rare combined primary immunodeficiency caused by the gain of function of the CXCR4 chemokine receptor. We present the prevalence of cancer in WHIMS patients based on data from the French Severe Chronic Neutropenia Registry and an exhaustive literature review. The median follow-up of the 14 WHIMS 'patients was 28.5 years. A central review and viral evaluation of pathological samples were organized, and we conducted a thorough literature review to identify all reports of WHIMS cases...
March 5, 2024: British Journal of Haematology
https://read.qxmd.com/read/38442905/increased-donor-inhibitory-kir-are-associated-with-reduced-gvhd-and-improved-survival-following-hla-matched-unrelated-donor-hct-in-paediatric-acute-leukaemia
#37
JOURNAL ARTICLE
Elizabeth Krieger, Rehan Qayyum, Amir Toor
Killer immunoglobulin-like receptor (KIR) and KIR-ligand (KIRL) interactions play an important role in natural killer cell-mediated effects after haematopoietic stem cell transplantation (HCT). Previous work has shown that accounting for known KIR-KIRL interactions may identify donors with optimal NK cell-mediated alloreactivity in the adult transplant setting. Paediatric acute leukaemia patients were retrospectively analysed, and KIR-KIRL combinations and maximal inhibitory KIR ligand (IM-KIR) scores were determined...
March 5, 2024: British Journal of Haematology
https://read.qxmd.com/read/38442902/molecular-characterization-of-diffuse-large-b-cell-lymphomas-associated-with-hepatitis-c-virus-infection
#38
JOURNAL ARTICLE
Roberta Sciarra, Michele Merli, Caterina Cristinelli, Marco Lucioni, Silvia Zibellini, Roberta Riboni, Daniela Furlan, Silvia Uccella, Caterina Zerbi, Benedetta Bianchi, Manuel Gotti, Virginia Valeria Ferretti, Chiara Varraso, Sara Fraticelli, Tanja Lazic, Irene Defrancesco, Barbara Mora, Laura Libera, Alessandro Mazzacane, Federico Carpi, Martha Berliner, Giuseppe Neri, Ettore Rizzo, Federica De Paoli, Fausto Sessa, Francesco Passamonti, Marco Paulli, Luca Arcaini
Hepatitis C virus (HCV)-associated diffuse large B-cell lymphoma (DLBCL) displays peculiar clinicopathological characteristics, but its molecular landscape is not fully elucidated. In this study, we investigated the clinicopathological and molecular features of 54 patients with HCV-associated DLBCL. The median age was 71 years. An underlying marginal zone lymphoma component was detected in 14.8% of cases. FISH analysis showed rearrangements involving BCL6 in 50.9% of cases, MYC in 11.3% and BCL2 in 3...
March 5, 2024: British Journal of Haematology
https://read.qxmd.com/read/38438264/prolonged-coagulation-times-in-severe-fever-with-thrombocytopenia-syndrome-virus-infection-the-indicators-of-heparin-like-effect-and-increased-haemorrhagic-risk
#39
JOURNAL ARTICLE
Ning Tang, Peihong Yuan, Ming Luo, Dengju Li
Prolonged coagulation times, such as activated partial thromboplastin time (APTT) and thrombin time (TT), are common in patients infected with severe fever with thrombocytopenia syndrome virus (SFTSV) and have been confirmed to be related to patient's poor outcome by previous studies. To find out the reason for prolonged coagulation time in patients with SFTSV infection, and whether it predicts haemorrhagic risk or not. Seventy-eight consecutive patients with confirmed SFTSV infection were enrolled in this prospective, single-centre, observational study...
March 4, 2024: British Journal of Haematology
https://read.qxmd.com/read/38438140/longitudinal-evaluation-of-adverse-events-due-to-steroid-use-in-primary-immune-thrombocytopenia-a-population-based-study
#40
JOURNAL ARTICLE
Jiaan-Der Wang, Fan-Chen Ku, I-Ju Chiang, Meng-Yun Ku, Yi-Ming Chen, Li-Nien Chien
This study aimed to investigate the association between the steroid use patterns and the risk of AEs in patients with primary immune thrombocytopenia (ITP). A total of 2691 newly diagnosed adults with ITP between 2011 and 2018 were identified from the National Health Insurance Research Database in Taiwan, and the date of first steroid use was defined as the index date. Post-index steroid use was calculated on a 90-day basis as a time-dependent variable and categorized by the average prednisolone-equivalent daily dose (<10 mg vs...
March 4, 2024: British Journal of Haematology
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