British Journal of Haematology最新文献_第3页

Understanding erythroid physiology and pathology in humanized mice: A closer look. 了解人源化小鼠的红细胞生理学和病理学：近距离观察

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-25 DOI: 10.1111/bjh.20023

Lu Lu, Chenfei Liu, Lezong Chen, Xumiao Zhang, Yinglin Su, Zhenzhen Chou, Yang Liang, Yuanbin Song

引用次数: 0

Acute myeloid leukaemia with SRSF2 and BRAF mutations preceded by histiocytic proliferation in the bone marrow.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-25 DOI: 10.1111/bjh.20028

Minako Mori, Tomomi Sakai, Tomomi Oka, Maki Sakurada, Makoto Iwasaki, June Takeda, Junya Kanda, Yasuhito Nannya, Seishi Ogawa, Akifumi Takaori-Kondo, Koki Moriyoshi, Hiroshi Kawabata

引用次数: 0

Outcomes and prognostic factors associated with relapse after haploidentical stem cell transplantation for paediatric T-cell acute lymphoblastic leukaemia.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-24 DOI: 10.1111/bjh.20007

Chen Zhao, Meng-Yu Xiao, Feng Zhang, Lu Bai, Guan-Hua Hu, Pan Suo, Feng-Rong Wang, Zhi-Dong Wang, Xiao-Dong Mo, Yu Wang, Yuan-Yuan Zhang, Lan-Ping Xu, Xiao-Jun Huang, Yi-Fei Cheng, Xiao-Hui Zhang

{"title":"Outcomes and prognostic factors associated with relapse after haploidentical stem cell transplantation for paediatric T-cell acute lymphoblastic leukaemia.","authors":"Chen Zhao, Meng-Yu Xiao, Feng Zhang, Lu Bai, Guan-Hua Hu, Pan Suo, Feng-Rong Wang, Zhi-Dong Wang, Xiao-Dong Mo, Yu Wang, Yuan-Yuan Zhang, Lan-Ping Xu, Xiao-Jun Huang, Yi-Fei Cheng, Xiao-Hui Zhang","doi":"10.1111/bjh.20007","DOIUrl":"https://doi.org/10.1111/bjh.20007","url":null,"abstract":"The outcomes are poor for paediatric patients with T-cell acute lymphoblastic leukaemia (T-ALL) who relapse after haematopoietic stem cell transplantation (HSCT). However, studies focusing on paediatric patients with T-ALL following haploidentical HSCT (haplo-HSCT) are limited. We retrospectively identified a consecutive cohort comprising of 128 paediatric T-ALL after haplo-HSCT from 2642 consecutive ALL patients between January 2010 and June 2022. The 2-year overall survival and leukaemia-free survival were 67.77% ± 4.21% and 66.34% ± 3.82%, respectively, and the cumulative incidence of relapse (CIR) and non-relapse mortality were 33.82% ± 0.70% and 12.65% ± 0.46% respectively. According to the multivariate Cox regression analysis, CD34 cells, minimal residual disease (MRD) ≥0.01% before HSCT, chronic graft-versus-host disease (cGvHD) and cytomegalovirus were associated with relapse (p < 0.05). To develop a scoring system for stratifying patients, we combined the variables and stratified them into low (0-2 points) and high (3, 4) groups. Consequently, the 2-year CIR in low and high groups were 23.76% ± 1.83% and 48.22% ± 2.42% (p = 0.009), respectively. Children with T-ALL have poor long-term survival, and haplo-HSCT is a potent and safe treatment; however, the incidence of relapse is high. Eliminating pre-HSCT MRD, guaranteeing sufficient CD34 cells infusion and the occurrence of cGvHD and cytomegalovirus reactivation may benefit from relapse.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143490223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Identification of new candidate drugs in myelodysplastic syndromes with splicing factor mutations by transcriptional profiling and connectivity map analysis. 通过转录谱分析和连接图分析，确定剪接因子突变骨髓增生异常综合征的新候选药物。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-23 DOI: 10.1111/bjh.20026

Tianyu Sun, Shalini Singh, Hayson Chenyu Wang, Juseong Lee, Hamid Dolatshad, Pak Leng Cheong, Douglas R Higgs, Jacqueline Boultwood, Andrea Pellagatti

引用次数: 0

How I treat iron-refractory iron deficiency anaemia-An expert opinion-based treatment guidance for children and adults.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-22 DOI: 10.1111/bjh.20030

V Hoving, A E Donker, S E M Schols, D W Swinkels

引用次数: 0

Real-world evidence of pegcetacoplan in patients with paroxysmal nocturnal haemoglobinuria: A nationwide Italian study.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-22 DOI: 10.1111/bjh.20025

Elisabetta Metafuni, Filippo Achille Brioschi, Andrea Patriarca, Claudia Leoni, Giorgia Battipaglia, Daniela Carlino, Annalisa Condorelli, Valeria Di Giacomo, Sarah Marktel, Maria Antonietta Marzilli, Maurizio Miglino, Esther Natalie Oliva, Antonella Sau, Alessandra Ricco, Grazia Sanpaolo, Annarita Trolese, Anna Paola Iori, Simona Sica, Wilma Barcellini, Bruno Fattizzo

引用次数: 0

Immune-deficient MISTRG mice support expansion of leukaemia-initiating cells in xenograft models of paediatric acute myeloid leukaemia.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-21 DOI: 10.1111/bjh.20029

Patrick Connerty, Jinhan Xie, Fatima El-Najjar, Toby N Trahair, Nisitha Jayatilleke, Chelsea Mayoh, Richard B Lock

引用次数: 0

Maternal and perinatal outcomes of sickle cell disease in pregnancy: A nationwide study in France.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-21 DOI: 10.1111/bjh.20009

Alice Corsia, Laure Joseph, Nathanael Beeker, Sandra Manceau, Marine Driessen, Benoit Meunier, Marina Cavazzana, Mathis Collier, Jean-Marc Treluyer

{"title":"Maternal and perinatal outcomes of sickle cell disease in pregnancy: A nationwide study in France.","authors":"Alice Corsia, Laure Joseph, Nathanael Beeker, Sandra Manceau, Marine Driessen, Benoit Meunier, Marina Cavazzana, Mathis Collier, Jean-Marc Treluyer","doi":"10.1111/bjh.20009","DOIUrl":"https://doi.org/10.1111/bjh.20009","url":null,"abstract":"This nationwide cohort study provides a comprehensive overview of maternal and perinatal outcomes associated with sickle cell disease (SCD) during pregnancy. Using the French national health database, all singleton pregnancy-related hospital discharges from 2013 to 2020 in women aged 15-55 (n = 5 752 080) were selected. Of these, 1022 births were to women with SCD, 308 of whom were on long-term treatment, that is, hydroxyurea (HU) and/or transfusion programme. Pregnancies with SCD were more likely to involve pre-eclampsia (9.6% vs. 1.7%; p < 0.001), pulmonary embolism (0.70% vs. 0.02%; p < 0.001), caesarean sections (52.8% vs. 18.2%; p < 0.001) and postpartum haemorrhage (8.3% vs. 4.1%; p < 0.001) compared to pregnancies without SCD. Preterm birth (<37 weeks) was much more common in women with SCD (28.5% vs. 5.6%). Infants born to women with SCD faced greater adverse neonatal outcomes (22.4% vs. 8.0%; p < 0.001). Although untreated SCD was linked to fewer complications than long-term treated SCD, both conditions presented greater risks compared with pregnancies without SCD. Unexpectedly, babies born to women with SCD had a higher incidence of congenital abnormalities (6.3% vs. 3.4%; p < 0.001), not attributed to HU use. Overall, despite advances in SCD management, pregnancy in SCD remains a high-risk condition, for both mothers and babies.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143472001","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Outcomes of daratumumab-bortezomib-thalidomide-dexamethasone in treatment-naive systemic AL amyloidosis.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-21 DOI: 10.1111/bjh.20021

Jahanzaib Khwaja, Sriram Ravichandran, Oliver Cohen, Darren Foard, May Low, Ana Martinez-Naharro, Lucia Venneri, Marianna Fontana, Philip N Hawkins, Julian Gillmore, Helen J Lachmann, Carol Whelan, Shameem Mahmood, Ashutosh Wechalekar

{"title":"Outcomes of daratumumab-bortezomib-thalidomide-dexamethasone in treatment-naive systemic AL amyloidosis.","authors":"Jahanzaib Khwaja, Sriram Ravichandran, Oliver Cohen, Darren Foard, May Low, Ana Martinez-Naharro, Lucia Venneri, Marianna Fontana, Philip N Hawkins, Julian Gillmore, Helen J Lachmann, Carol Whelan, Shameem Mahmood, Ashutosh Wechalekar","doi":"10.1111/bjh.20021","DOIUrl":"https://doi.org/10.1111/bjh.20021","url":null,"abstract":"Systemic light chain (AL) amyloidosis is an incurable disorder caused by extra-cellular deposition of light-chain aggregates in critical organs. An immunomodulatory agent-based quadruplet including anti-CD38 therapy has not been investigated as a first-line treatment in AL amyloidosis. We report the UK experience of daratumumab-bortezomib-thalidomide-dexamethasone for the first-line treatment of AL amyloidosis. Consecutive patients with a new diagnosis of systemic AL between 2021 and 2023 were retrospectively reviewed from the UK National Amyloidosis Centre database. One hundred and two patients were included; median age was 61 years, involved free light-chain concentration 234 mg/L, 65% had cardiac and 63% renal involvement with modified Mayo stage I/II/IIIa/IIIb in 24%/36%/21% and 19% respectively. A median of 6 cycles was delivered. By intention-to-treat analysis, best haematological overall response rate was 97%: complete response at 65%, very good partial response at 22%, partial response at 11% and no response at 3%. At 6- and 12-month time points from treatment initiation, best cardiac response rates were 39% and 45%, respectively, for evaluable patients. At a median duration of 18 months follow-up, the estimated 1-year overall survival was 89% (95% confidence interval [CI] 81-94) and treatment-free survival/death was 82% (95% CI 73-89). We demonstrate efficacy in this real-world population with comparable results to the gold standard, daratumumab-bortezomib-cyclophosphamide-dexamethasone.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143466687","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The potential role of the αVβ3 integrin receptor in placental biology and normal and complicated pregnancies.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-20 DOI: 10.1111/bjh.20019

Amihai Rottenstreich, Barry S Coller

引用次数: 0