British Journal of Haematology最新文献_第10页

Systemic capillary leak syndrome associated with smouldering multiple myeloma treated by bortezomib plus dexamethasone: A case report. 硼替佐米加地塞米松治疗阴燃性多发性骨髓瘤伴全身毛细血管渗漏综合征1例报告。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-04 DOI: 10.1111/bjh.20193

Qiling Bu, Huixing Zhou, Wen Gao, Wenming Chen

引用次数: 0

Higher risk of platelet engraftment failure and chronic graft-versus-host disease after allogeneic haematopoietic stem cell transplantation following chimeric antigen receptor T-cell therapy compared to chemotherapy: A propensity score-matched analysis 与化疗相比，嵌合抗原受体t细胞治疗后同种异体造血干细胞移植后血小板植入失败和慢性移植物抗宿主病的风险更高：倾向评分匹配分析

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-04 DOI: 10.1111/bjh.20180

Luxin Yang, Xiaoyu Lai, Lizhen Liu, Jimin Shi, Yanmin Zhao, Jian Yu, Yibo Wu, Huarui Fu, Yongxian Hu, Mingming Zhang, He Huang, Yi Luo

{"title":"Higher risk of platelet engraftment failure and chronic graft-versus-host disease after allogeneic haematopoietic stem cell transplantation following chimeric antigen receptor T-cell therapy compared to chemotherapy: A propensity score-matched analysis","authors":"Luxin Yang, Xiaoyu Lai, Lizhen Liu, Jimin Shi, Yanmin Zhao, Jian Yu, Yibo Wu, Huarui Fu, Yongxian Hu, Mingming Zhang, He Huang, Yi Luo","doi":"10.1111/bjh.20180","DOIUrl":"10.1111/bjh.20180","url":null,"abstract":"<div>\u0000 \u0000 Allogeneic haematopoietic stem cell transplantation (allo-HSCT) has been reported to further sustain long-term leukaemia-free survival following chimeric antigen receptor T-cell (CAR-T) therapy. It remains unclear whether bridging CAR-T to allo-HSCT results in higher treatment-related toxicity and mortality. We conducted a retrospective study to compare outcomes between allo-HSCT after CAR-T or conventional chemotherapy. After propensity score matching, 62 patients with prior CAR-T therapy and 124 patients with chemotherapy were ultimately included. Patients in the CAR-T cohort had a longer duration time from diagnosis to transplant (p < 0.001) and more advanced disease status before HSCT (p < 0.001) than that of the chemotherapy cohort. Patients with prior CAR-T cell therapy had a lower 28-day platelet engraftment rates [Hazard Rate (HR) = 1.38, 95% Confidence Interval (CI), 1.02–1.87, p = 0.037]. Multivariate analysis revealed that CAR-T therapy increased the risk of moderate to severe chronic graft-versus-host disease (cGVHD) (HR = 2.5, 95% CI, 1.01–6.19, p = 0.048). Compared with patients in the chemotherapy cohort, those in the CAR-T cell cohort experienced a higher incidence of transplantation-associated thrombotic microangiopathy (6.5% vs. 0.8%, p = 0.03) and probable/possible invasive fungal disease (10.0% vs. 3.3%, p = 0.08). The relapse rate, non-relapse mortality, and survival were comparable between cohorts. Caution should be exercised in allo-HSCT following CAR-T therapy because of the higher risk of platelet engraftment failure and cGVHD compared to chemotherapy.\u0000 </div>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":"207 1","pages":"162-170"},"PeriodicalIF":5.1,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144214463","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

Real-world clinical experience with NGS-based chimerism analyses in haematopoietic stem cell transplant patients. 基于ngs的嵌合分析在造血干细胞移植患者中的实际临床经验。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-04 DOI: 10.1111/bjh.20191

Jin Ju Kim, Soon Sung Kwon, Yu Jeong Choi, Yehyun Kang, Yu Jin Park, Saeam Shin, Seung-Tae Lee, Jong Rak Choi

{"title":"Real-world clinical experience with NGS-based chimerism analyses in haematopoietic stem cell transplant patients.","authors":"Jin Ju Kim, Soon Sung Kwon, Yu Jeong Choi, Yehyun Kang, Yu Jin Park, Saeam Shin, Seung-Tae Lee, Jong Rak Choi","doi":"10.1111/bjh.20191","DOIUrl":"https://doi.org/10.1111/bjh.20191","url":null,"abstract":"Next-generation sequencing (NGS) has improved the sensitivity of chimerism assays beyond the limitations of conventional short tandem repeat (STR) methods, enabling the detection of minimal recipient haematopoiesis after haematopoietic stem cell transplantation (HSCT). We evaluated the clinical utility of CASAL, an NGS-based chimerism assay, in routine practice. We retrospectively analysed 310 patients who underwent STR or CASAL chimerism testing between April 2021 and September 2023. CASAL provided significantly more informative markers than STR (median 18 vs. 6; p < 0.001). Among 260 CASAL samples with paired molecular minimal residual disease (MRD) data, concordance at the 10-4 threshold was ~84%. Low-level mixed chimerism (2%-5%) detected beyond 1 month post-HSCT was associated with impending relapse. In survival analyses, patients with both MC and MRD positivity (MC/MRD+) had the highest relapse risk across both platforms. Multivariable Cox regression confirmed MC/MRD+ as an independent predictor of relapse (hazard ratio 5.87, 95% CI: 1.17-29.57). CASAL enables sensitive chimerism monitoring and shows a strong correlation with molecular MRD and clinical outcomes. These findings support its clinical utility for individualized post-HSCT surveillance, especially in patients lacking leukaemia-specific molecular targets.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144214464","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

Assessment of neurocognitive functioning in sickle cell disease and thalassaemia and the association with silent cerebral infarcts, cerebral haemodynamics and oxygen metabolism. 镰状细胞病和地中海贫血的神经认知功能评估及其与无症状性脑梗死、脑血流动力学和氧代谢的关系

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-04 DOI: 10.1111/bjh.20181

Liza Afzali-Hashemi, Melanie Franse, Koen P A Baas, Anouk Schrantee, Erfan Nur, John C Wood, Aart J Nederveen, Bart J Biemond, Sharon H O'Neil

{"title":"Assessment of neurocognitive functioning in sickle cell disease and thalassaemia and the association with silent cerebral infarcts, cerebral haemodynamics and oxygen metabolism.","authors":"Liza Afzali-Hashemi, Melanie Franse, Koen P A Baas, Anouk Schrantee, Erfan Nur, John C Wood, Aart J Nederveen, Bart J Biemond, Sharon H O'Neil","doi":"10.1111/bjh.20181","DOIUrl":"https://doi.org/10.1111/bjh.20181","url":null,"abstract":"Neurocognitive impairment is a key concern in sickle cell disease (SCD) patients, affecting several factors including academic performance, employment and quality of life. While the exact causes remain unclear, correlations with silent cerebral infarcts (SCIs) and abnormal perfusion and oxygenation have been reported in paediatric SCD populations. This study aimed to examine these associations in adults with SCD, including 70 severe SCD patients (HbSS & HbSβ0-thal), 22 mild SCD patients (HbSC & HbSβ+-thal), 16 thalassaemia patients and 29 healthy controls. We assessed seven neurocognitive domains: attention, working memory, processing speed, non-verbal IQ, verbal learning, visual memory and fine motor dexterity. Additionally, we analysed relationships between neurocognitive performance, SCIs, haemodynamics and oxygen metabolism parameters, including cerebral blood flow (CBF), oxygen extraction fraction (OEF) and cerebral metabolic rate of oxygen (CMRO2). Our findings reveal that only processing speed was affected in SCD patients relative to norms, thalassaemia patients and controls. Fine motor dexterity was lower across all groups, possibly due to normative data not reflecting the transition from handwriting to typing. Notably, we found no correlation between neurocognitive performance, SCIs and perfusion and oxygenation parameters, suggesting that reduced processing speed may stem from other SCD-related factors, highlighting the need for further research.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144223810","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

Risk factors for immediate postpartum sickle cell disease-specific maternal morbidity 产后立即镰状细胞病特异性产妇发病率的危险因素。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-03 DOI: 10.1111/bjh.20178

Joanna Loh, Kevin H. M. Kuo, Ilinca Georgescu, Stella Wang, Ella Huszti, Nadine Shehata, A. Kinga Malinowski

{"title":"Risk factors for immediate postpartum sickle cell disease-specific maternal morbidity","authors":"Joanna Loh, Kevin H. M. Kuo, Ilinca Georgescu, Stella Wang, Ella Huszti, Nadine Shehata, A. Kinga Malinowski","doi":"10.1111/bjh.20178","DOIUrl":"10.1111/bjh.20178","url":null,"abstract":"Pregnant individuals with sickle cell disease (SCD) are more likely to have postpartum complications. In this retrospective single-centre study, 201 pregnancies from 145 individuals were assessed between 2000 and 2018 for the presence of a composite of immediate postpartum complications from admission during delivery to discharge, which included vaso-occlusive pain events, acute chest syndrome, infection (blood, urine, pulmonary) and/or new-onset hypoxia. Hypoxia was defined as SpO2 below 92%. An immediate postpartum complication occurred in 74 pregnancies (37%), of which 97% experienced a vaso-occlusive pain event. No immediate postpartum deaths were recorded. Pregnancies with HbSS/HbSβ0 as compared to HbSC/HbSβ+ had a higher incidence of the composite outcome (51% vs. 19%). On univariate analysis, HbSS/HbSβ0 genotype was associated with a higher likelihood of developing a postpartum complication (OR 4.49, 95%CI 2.35–8.57; p < 0.0001); however, this significance was reduced on multivariable analysis. The strongest risk factors associated with experiencing the composite outcome on multivariable analysis were hypoxia during pregnancy (OR 4.95, 95%CI 1.86–13.23; p = 0.001) and an unscheduled caesarean delivery (OR 2.67; 95%CI 1.26–5.64; p = 0.01). Future studies need to address whether correcting hypoxia and use of predictive scores to guide the need for a scheduled caesarean delivery can reduce immediate postpartum complication rates.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":"207 1","pages":"225-234"},"PeriodicalIF":5.1,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/bjh.20178","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144214465","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Final results of a multicentre pilot study evaluating brentuximab vedotin with cyclophosphamide, doxorubicin, etoposide and prednisone (BV-CHEP) for the treatment of aggressive adult T-cell leukaemia/lymphoma 一项评估brentuximab vedotin联合环磷酰胺、阿霉素、依托泊苷和强的松（BV-CHEP）治疗侵袭性成人t细胞白血病/淋巴瘤的多中心试点研究的最终结果。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-03 DOI: 10.1111/bjh.20177

Christopher Dittus, Matthew J. Weinstock, Jeffrey Barnes, Jose Sandoval-Sus, Natalie S. Grover, Anne Beaven, Kelly Hoye, Anthony Shelton, Xianming Tan, Maya D. Srinivasan, Areej El-Jawahri, Shayna Sarosiek, J. Mark Sloan

引用次数: 0

Impact of age and time since diagnosis on thrombosis risk in myeloproliferative neoplasm patients: A nationwide register-based matched cohort study 年龄和诊断后时间对骨髓增殖性肿瘤患者血栓形成风险的影响：一项基于全国登记的匹配队列研究。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-02 DOI: 10.1111/bjh.20141

Nurgul Batyrbekova, Malin Hultcrantz, Anna Ravn Landtblom, Robert Szulkin, Paul W. Dickman, Therese M.-L. Andersson, Elisavet Syriopoulou

{"title":"Impact of age and time since diagnosis on thrombosis risk in myeloproliferative neoplasm patients: A nationwide register-based matched cohort study","authors":"Nurgul Batyrbekova, Malin Hultcrantz, Anna Ravn Landtblom, Robert Szulkin, Paul W. Dickman, Therese M.-L. Andersson, Elisavet Syriopoulou","doi":"10.1111/bjh.20141","DOIUrl":"10.1111/bjh.20141","url":null,"abstract":"Thromboembolism is one of the major complications in myeloproliferative neoplasms (MPNs), especially in the initial years post-diagnosis and with increasing age. This nationwide matched cohort study used Swedish registers to assess whether attained age or time since diagnosis is more important for thrombosis risk. We identified 17 830 MPN patients diagnosed between 2001 and 2021, each matched with 10 population controls by sex, age and diagnosis year. Rates of first thrombosis post diagnosis and hazard ratios (HRs) were estimated using flexible parametric survival models over time since diagnosis and attained age. Thrombosis rates were significantly higher in MPN patients at all ages, especially in the first 2 years post-diagnosis, after which the rates remained higher than in age-matched controls. At 5 years, HR (95% confidence interval) for any thrombosis comparing patients to controls was 1.8 (1.7–1.9) at age 65, 1.6 (1.5–1.7) at age 70 and 1.5 (1.4–1.6) at age 75 and older. Attained age was more important than time since diagnosis, particularly in patients without prior thrombosis. Thrombosis rates were highest within the first 2 years post-diagnosis, then depended more on attained age than on time since diagnosis. These findings emphasize the importance of age and early management in MPN patients to optimize clinical care.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":"207 1","pages":"189-200"},"PeriodicalIF":5.1,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/bjh.20141","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144207256","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Low rates of endothelial cell dysfunction and transplant-related mortality in 537 children receiving fludarabine-treosulfan conditioning for all transplant indications: A retrospective multicentre study on behalf of the UK Paediatric BMT group. 537名接受氟达拉滨-曲硫丹调节治疗的儿童的内皮细胞功能障碍和移植相关死亡率低：代表英国儿科BMT组的一项回顾性多中心研究。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-06-01 DOI: 10.1111/bjh.20184

Thomas Altmann, Kanchan Rao, Ramya Hanasoge-Nataraj, Katharine Patrick, Ben Carpenter, Rachael Hough, Anna-Maria Ewins, Sanjay Tewari, Oana Mirci-Danicar, Mary Slatter, Robert Wynn, Su Han Lum

引用次数: 0

Pregnancy and delivery outcomes in individuals with RUNX1-Familial Platelet Disorder. runx1家族性血小板紊乱患者的妊娠和分娩结局

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-29 DOI: 10.1111/bjh.20194

Natalie T Deuitch, Lylach Haizler-Cohen, Amra Kajdic, Erica Bresciani, Kathleen Craft, Joie Davis, Shawn Chong, David J Young, P Paul Liu

引用次数: 0

Survival and thalassaemia-related complications in HbE/beta-thalassaemia and alpha-thalassaemia: A 10-year longitudinal study in Thailand. HbE/ β -地中海贫血和α -地中海贫血患者的生存和地中海贫血相关并发症：泰国一项为期10年的纵向研究

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-26 DOI: 10.1111/bjh.20179

Nattiya Teawtrakul, Arunee Jetsrisuparb, Saranya Pongudom, Kanchana Chansung, Chittima Sirijerachai, Supan Fucharoen

{"title":"Survival and thalassaemia-related complications in HbE/beta-thalassaemia and alpha-thalassaemia: A 10-year longitudinal study in Thailand.","authors":"Nattiya Teawtrakul, Arunee Jetsrisuparb, Saranya Pongudom, Kanchana Chansung, Chittima Sirijerachai, Supan Fucharoen","doi":"10.1111/bjh.20179","DOIUrl":"https://doi.org/10.1111/bjh.20179","url":null,"abstract":"Despite advancements in thalassaemia care, survival rates and complications vary significantly by genotype. This study assesses survival outcomes and associated complications in patients with thalassaemia in north-eastern Thailand. A longitudinal cohort study from October 2012 to September 2023 involved patients aged ≥10 years (median: 31 years, range: 20-74) attending Srinagarind and Udonthani Hospitals. Cox regression analyses identified predictors of survival and complications. Of 380 enrolled patients, 340 completed follow-up. Over 10 years, 39 patients (11.4%) died, primarily from cardiovascular complications (61.5%), at a median age of 45 years (range: 22-72). Patients were grouped as HbE/β-thalassaemia, HbH/HbHCS with HbE mutation and HbH/HbHCS. Survival at 60 years was lower in HbE/β-thalassaemia (55.9%) and HbH/HbHCS with HbE mutation (58.3%) compared to HbH/HbHCS (79.6%, p = 0.08). Significant predictors of mortality included cardiovascular complications (HR 2.4; 95% CI, 1.01-5.5; p = 0.04), recurrent bacterial infections (HR 7.6; 95% CI, 3.1-18.8; p < 0.001) and elevated ferritin (>2500 ng/mL; HR 2.0; 95% CI, 1.01-3.9; p = 0.04). Cardiovascular complications, recurrent bacterial infections and high ferritin levels significantly influence survival in thalassaemia patients. Deletional alpha-thalassaemia patients had superior survival, while HbE/β-thalassaemia and non-deletional alpha-thalassaemia or co-inherited HbE mutation had poorer outcomes, underscoring the need for genotype-specific management strategies.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144148975","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