British Journal of Haematology最新文献_第9页

Trends in survival outcomes after allogeneic transplantation for MDS and MDS/MPN in a real-world experience: A 25-year nationwide study. 异基因移植治疗MDS和MDS/MPN后的生存趋势：一项为期25年的全国研究。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-05 DOI: 10.1111/bjh.70463

Hidehiro Itonaga, Yasushi Miyazaki, Noriaki Tachi, Shuhei Kurosawa, Atsushi Marumo, Takaaki Konuma, Toru Ebina, Noriko Doki, Naoyuki Uchida, Takahiro Fukuda, Tetsuya Nishida, Yuta Katayama, Noboru Asada, Masatoshi Sakurai, Takeshi Maeda, Tetsuya Eto, Masatsugu Tanaka, Hirohisa Nakamae, Makoto Onizuka, Koji Kawamura, Yoshinobu Kanda, Yoshiko Atsuta, Takayoshi Tachibana

{"title":"Trends in survival outcomes after allogeneic transplantation for MDS and MDS/MPN in a real-world experience: A 25-year nationwide study.","authors":"Hidehiro Itonaga, Yasushi Miyazaki, Noriaki Tachi, Shuhei Kurosawa, Atsushi Marumo, Takaaki Konuma, Toru Ebina, Noriko Doki, Naoyuki Uchida, Takahiro Fukuda, Tetsuya Nishida, Yuta Katayama, Noboru Asada, Masatoshi Sakurai, Takeshi Maeda, Tetsuya Eto, Masatsugu Tanaka, Hirohisa Nakamae, Makoto Onizuka, Koji Kawamura, Yoshinobu Kanda, Yoshiko Atsuta, Takayoshi Tachibana","doi":"10.1111/bjh.70463","DOIUrl":"https://doi.org/10.1111/bjh.70463","url":null,"abstract":"Allogeneic haematopoietic stem cell transplantation (allo-HSCT) offers a curative potential for myelodysplastic syndrome (MDS) and myelodysplastic/myeloproliferative neoplasm (MDS/MPN). We examined survival trends using a nationwide database of 7175 patients who underwent their first allo-HSCT between 1998 and 2022. Overall mortality decreased over time in patients with early MDS (hazard ratio [HR] 0.79, 95% confidence interval [CI] 0.65-0.97, p = 0.026 in 2013-2017; HR 0.54, 95% CI 0.42-0.70, p < 0.001 in 2018-2022), advanced MDS (HR 0.80, 95% CI 0.71-0.90, p < 0.001 in 2013-2017; HR 0.74, 95% CI 0.65-0.85, p < 0.001 in 2018-2022), chronic myelomonocytic leukaemia (HR 0.45, 95% CI 0.32-0.64, p < 0.001 in 2013-2017; HR 0.53, 95% CI 0.37-0.74, p < 0.001 in 2018-2022) and atypical chronic myeloid leukaemia (HR 0.32, 95% CI 0.13-0.80, p = 0.016 in 2013-2017; HR 0.26, 95% CI 0.10-0.68, p = 0.006 in 2018-2022). These decreases in overall mortality were mainly attributable to reductions in non-relapse mortality. Meanwhile, no significant difference in overall mortality was observed in patients with MDS/MPN-unclassified and therapy-related myeloid neoplasm. Across all disease subtypes, relapse incidence did not significantly decrease over time, highlighting persistent challenges in reducing the risk of post-transplant relapse.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147621337","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

Neurofilament as a biomarker of neurodegeneration in childhood Langerhans cell histiocytosis: A feasibility study on 273 patients. 神经丝作为儿童朗格汉斯细胞组织细胞增多症神经退行性变的生物标志物：273例患者的可行性研究

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-05 DOI: 10.1111/bjh.70466

Solenne Le Louet, Camille Brunaud, Mohamed Barkaoui, Aurore Chevallier, François Chalard, Ludovic Mansuy, Alexandra Salmon, Nimrod Buchbinder, Saba Azarnoush, Sophie Pertuisel, Caroline Thomas, Jens Kuhle, Aleksandra Maleska, Claire Desplantes, Perrine Marec-Bérard, Anne Pagnier, Fiorentina Isfan, Nathalie Aladjidi, Paul Saultier, Eric Jeziorski, Julian Thalhammer, Ahmed Idbaih, Jean-François Emile, Sébastien Heritier, Jean Donadieu

{"title":"Neurofilament as a biomarker of neurodegeneration in childhood Langerhans cell histiocytosis: A feasibility study on 273 patients.","authors":"Solenne Le Louet, Camille Brunaud, Mohamed Barkaoui, Aurore Chevallier, François Chalard, Ludovic Mansuy, Alexandra Salmon, Nimrod Buchbinder, Saba Azarnoush, Sophie Pertuisel, Caroline Thomas, Jens Kuhle, Aleksandra Maleska, Claire Desplantes, Perrine Marec-Bérard, Anne Pagnier, Fiorentina Isfan, Nathalie Aladjidi, Paul Saultier, Eric Jeziorski, Julian Thalhammer, Ahmed Idbaih, Jean-François Emile, Sébastien Heritier, Jean Donadieu","doi":"10.1111/bjh.70466","DOIUrl":"https://doi.org/10.1111/bjh.70466","url":null,"abstract":"Neurodegeneration (ND) is a severe complication of Langerhans cell histiocytosis (LCH), often leading to progressive neurological decline. We evaluated the usefulness of using plasma and cerebrospinal fluid neurofilament light chain (p- and CSF-NFL) levels as biomarkers to identify and monitor ND-LCH. NFL levels were measured using the single-molecule array for a subset of patients from the French National LCH Registry. NFL levels in 692 plasma and 115 CSF samples from 273 registry-enrolled children were analysed. Based on 84 paired plasma and CSF samples from 67 patients, p- and CSF-NFL levels were strongly correlated (p < 0.0001). The areas under the receiver operating characteristics curves for ND-LCH were 72% (95% confidence interval [CI], 64%-78%) for p-NFL and 94% (95% CI, 88%-98%) for CSF-NFL. The highest p-NFL (13.7 vs. 7.2 pg/mL; z-score 2.3 vs. 0.6) and CSF-NFL (436.9 vs. 65.2 pg/mL) levels were significantly higher for ND-LCH than in no-ND-LCH patients, respectively (p < 0.0001). Plasma NFL levels were not elevated at LCH diagnosis. At LCH diagnosis, p-NFL was not predictive of ND, but it may serve as a minimally invasive screening tool for ND in LCH, although optimal timing remains to be determined.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147621297","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

Short-course blood transfusion therapy with hydroxyurea, a functional strategy in the management of stroke in children with sickle cell disease. 短期输血治疗羟基脲治疗镰状细胞病患儿脑卒中的功能策略

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-05 DOI: 10.1111/bjh.70399

Edamisan Temiye, Adeseye Akinsete, Adetokunbo Joacquim, Ugonna Fakile, Olivia Ashefor, Chibuzor Ogamba, Ann Ogbenna, Nimota Adeyemo, Oladunni Okodugha, Ehiosu Edokpolo, Olaide Elemo, Abideen Salako

{"title":"Short-course blood transfusion therapy with hydroxyurea, a functional strategy in the management of stroke in children with sickle cell disease.","authors":"Edamisan Temiye, Adeseye Akinsete, Adetokunbo Joacquim, Ugonna Fakile, Olivia Ashefor, Chibuzor Ogamba, Ann Ogbenna, Nimota Adeyemo, Oladunni Okodugha, Ehiosu Edokpolo, Olaide Elemo, Abideen Salako","doi":"10.1111/bjh.70399","DOIUrl":"https://doi.org/10.1111/bjh.70399","url":null,"abstract":"Cerebrovascular accidents are serious complications of sickle cell disease (SCD). Children with abnormal transcranial Doppler (TCD) readings are at higher risk for stroke, and those with prior strokes have increased risk of recurrence. Chronic blood transfusion therapy (BTT) is the standard treatment for stroke prevention; hydroxyurea (hydroxycarbamide [HU]), a recommended alternative, is less effective. This study explores the use of modified short BTT combined with HU for stroke prevention in SCD. We reviewed medical records of 170 children (ages 2-16) with abnormal TCD or prior stroke treated with monthly BTT for 6-8 months and HU. TCD results and stroke incidence were assessed 1 year after discontinuing BTT. Among the children, 136 (80%) had abnormal TCD, 15 (11.2%) had current stroke and 19 (8.8%) had prior stroke. After discontinuation of BTT, abnormal TCD readings decreased significantly to 18.7% and 16.9% (p < 0.05); those with normal TCD increased significantly from 38.8% to 52.8% (p < 0.05) after 1 year. TCD velocity decreased significantly during the same period (p < 0.05). Only 2 (1.5%) children with abnormal TCD experienced stroke. Overall, 13 (7.8%) had stroke by 1-year post BTT. In conclusion, the combination of short-course BTT and HU significantly reduced stroke risk and incidence of stroke in children with SCD.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147621322","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

Endothelial activation and stress index score as a predictor of transplant-associated thrombotic microangiopathy in patients with sickle cell disease. 内皮活化和应激指数评分作为镰状细胞病患者移植相关血栓性微血管病变的预测因子

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-02 DOI: 10.1111/bjh.70465

Reethu Krishnan, Hari Sankaran, David Jacobsohn, Robert Nickel, Kelly Snelling, Anant Vatsayan, Alexandra Dreyzin

引用次数: 0

Differential complement activation by alloantibodies in sickle cell hyperhaemolysis syndrome may influence disease course. 镰状细胞高溶血综合征的异体抗体活化差异补体可能影响病程。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-02 DOI: 10.1111/bjh.70459

Mamie M Thant, Jahnavi Gollamudi, Stefanie W Benoit, Satheesh Chonat, Jose A Cancelas

引用次数: 0

Short and complex-Telomeres and genomes in CLL. 短而复杂的端粒与CLL的基因组。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-01 Epub Date: 2026-03-11 DOI: 10.1111/bjh.70430

Billy Michael Chelliah Jebaraj, Stephan Stilgenbauer

引用次数: 0

Low serum transferrin predicts adverse outcome in COVID-19 and is related to coagulation activity. 低血清转铁蛋白可预测COVID-19的不良结局，并与凝血活性有关。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-01 Epub Date: 2026-02-10 DOI: 10.1111/bjh.70354

Lukas Lanser, Francesco Robert Burkert, Lis Thommes, Rosa Bellmann-Weiler, Guenter Weiss

引用次数: 0

Equal outcomes after human leukocyte antigen-matched unrelated versus sibling donor transplants-Results of the AML SCT-BFM 2007 trial and consecutive 'real-world routine'. 人类白细胞抗原匹配的非亲属与兄弟姐妹供体移植的结果相同——AML SCT-BFM 2007试验和连续的“现实世界常规”的结果。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-01 Epub Date: 2026-01-07 DOI: 10.1111/bjh.70324

Martin G Sauer, Peter J Lang, Michael H Albert, Peter Bader, Matthias Eyrich, Bernd Gruhn, Thomas Klingebiel, Christine Mauz-Körholz, Roland Meisel, Ingo Müller, Charlotte M Niemeyer, Christina Peters, Birgit Burkhardt, Dirk Reinhardt, Petr Sedlacek, Brigitte Strahm, Wilhelm Woessmann, Rupert Handgretinger, Martin Zimmermann, Arndt Borkhardt

引用次数: 0

Suboptimal screening and correction of maternal iron deficiency anaemia in the general population and in inherited bleeding disorders: A population-based cohort study. 普通人群和遗传性出血性疾病中母亲缺铁性贫血的次优筛查和纠正：一项基于人群的队列研究

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-01 Epub Date: 2026-01-27 DOI: 10.1111/bjh.70348

Arafat Ul Alam, Padma Kaul, Venu Jain, Cynthia Wu, Haowei Linda Sun

{"title":"Suboptimal screening and correction of maternal iron deficiency anaemia in the general population and in inherited bleeding disorders: A population-based cohort study.","authors":"Arafat Ul Alam, Padma Kaul, Venu Jain, Cynthia Wu, Haowei Linda Sun","doi":"10.1111/bjh.70348","DOIUrl":"10.1111/bjh.70348","url":null,"abstract":"Iron deficiency anaemia (IDA) is associated with adverse pregnancy outcomes globally. Women with inherited bleeding disorders are at increased risk, with scarce data on rates of IDA screening and correction during pregnancy. The impact of correction on outcomes is unclear. To assess the frequency of IDA screening, the frequency of IDA in pregnancies with and without bleeding disorders and the impact of correction on outcomes. This retrospective population-based cohort study includes all hospitalized pregnancies using the Alberta Pregnancy Birth Cohort. IDA was defined as haemoglobin <110 g/L in first/third trimester, <105 g/L in second trimester and ferritin <30 μg/L. Logistic regression was performed to assess the association between IDA, corrected IDA and pregnancy outcomes. 36 500/207 355 (18%) pregnancies and 14/58 (24%) pregnancies in the bleeding disorder subgroup had anaemia. Only one of three pregnancies with anaemia had a concurrent ferritin test, and over 80% demonstrated IDA. Ferritin screening was performed in 60% of the overall cohort and 79% in bleeding disorders. Young maternal age, multiparity, lower socioeconomic status and mothers from African and Asian countries significantly predict IDA. Despite the increased risk for adverse outcomes, only 43 (8%) first-trimester and 96 (9%) third-trimester IDA were corrected. IDA screening and correction remained suboptimal among pregnant women. Updated screening guidelines may promote better identification and outcomes.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1389-1397"},"PeriodicalIF":3.8,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13071478/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146049640","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

Use of platelets and the challenge of adherence to guidelines: Who, when and why? 血小板使用和遵守指南的挑战：何人、何时、为何？

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2026-04-01 Epub Date: 2026-03-12 DOI: 10.1111/bjh.70433

Joel L Addams, Simon J Stanworth, Ryan A Metcalf

引用次数: 0