British Journal of Haematology最新文献

Prognostic impact of valemetostat in relapsed/refractory adult T-cell leukaemia-lymphoma. 缬美他汀对复发/难治性成人t细胞白血病淋巴瘤预后的影响。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-23 DOI: 10.1111/bjh.70219

Takafumi Shichijo, Hiro Tatetsu, Yusuke Higuchi, Toshikazu Miyakawa, Kisato Nosaka, Takahisa Nakamura, Shinya Endo, Asami Yamada, Atsushi Wada, Yuki Okamoto, Toshiro Kawakita, Hitoshi Suzushima, Masao Matsuoka, Jun-Ichirou Yasunaga

{"title":"Prognostic impact of valemetostat in relapsed/refractory adult T-cell leukaemia-lymphoma.","authors":"Takafumi Shichijo, Hiro Tatetsu, Yusuke Higuchi, Toshikazu Miyakawa, Kisato Nosaka, Takahisa Nakamura, Shinya Endo, Asami Yamada, Atsushi Wada, Yuki Okamoto, Toshiro Kawakita, Hitoshi Suzushima, Masao Matsuoka, Jun-Ichirou Yasunaga","doi":"10.1111/bjh.70219","DOIUrl":"https://doi.org/10.1111/bjh.70219","url":null,"abstract":"The prognosis for patients with relapsed/refractory (R/R) adult T-cell leukaemia-lymphoma (ATL) remains dismal. Recently, valemetostat, a dual inhibitor for enhancer of zeste homologue (EZH) 1 and 2, was approved in Japan for R/R aggressive ATL. However, there is no real-world data on the efficacy and prognosis of valemetostat. We therefore analysed clinical outcomes in 28 patients with R/R aggressive ATL who received valemetostat at three hospitals in Kumamoto Prefecture between February 2023 and January 2025. The overall response rate (ORR) and complete response (CR) rate were 54.2% and 33.3% respectively. With a median follow-up of 19.2 months (7.3-21.7) for surviving patients, the median survival time was 15.8 months and median progression-free survival (PFS) was 8.3 months. The probabilities of 1-year overall survival (OS) and PFS were 55.8% and 44.1% respectively. Furthermore, the probabilities of 1-year duration of response and duration of CR were 56.2% and 71.4% respectively. Notably, the probability of 1-year OS was 90.9% in patients who achieved CR/partial response (PR) ('responders') compared to 17.0% in patients who did not achieve CR/PR (p < 0.001). Thus, valemetostat has the potential to be a reliable therapy for patients with R/R aggressive ATL, especially for responders.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145342199","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 DIC in paediatric APL: Insights from the CCLG-APL 2016 study. 儿科APL DIC的危险因素：来自CCLG-APL 2016研究的见解

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-23 DOI: 10.1111/bjh.70211

Qingyuan Xu, Linya Wang, Shaoyan Hu, Ning Liao, Diying Shen, Xin Tian, Guoping Hao, Runming Jin, Jianxin Li, Yongjun Fang, Xiuli Ju, Ansheng Liu, Yuanyuan Zhang, Pengli Huang, Jiaole Yu, Ying Wu, Wei Lin, Peijing Qi, Jia Fan, Ruidong Zhang, Yaguang Peng, Huyong Zheng

{"title":"Risk factors for DIC in paediatric APL: Insights from the CCLG-APL 2016 study.","authors":"Qingyuan Xu, Linya Wang, Shaoyan Hu, Ning Liao, Diying Shen, Xin Tian, Guoping Hao, Runming Jin, Jianxin Li, Yongjun Fang, Xiuli Ju, Ansheng Liu, Yuanyuan Zhang, Pengli Huang, Jiaole Yu, Ying Wu, Wei Lin, Peijing Qi, Jia Fan, Ruidong Zhang, Yaguang Peng, Huyong Zheng","doi":"10.1111/bjh.70211","DOIUrl":"https://doi.org/10.1111/bjh.70211","url":null,"abstract":"To identify early risk factors for disseminated intravascular coagulation (DIC), particularly severe DIC (grade 4-5), in paediatric acute promyelocytic leukaemia (APL). One hundred and eighty-six paediatric APL patients enrolled in the Chinese Children Leukemia Group (CCLG)-APL 2016 study across 38 hospitals nationwide were grouped based on the occurrence and severity of DIC during induction therapy. DIC occurred in 52.2% of patients during induction therapy, with 7.5% developing grade 4-5 DIC. Significant differences were observed between the DIC and non-DIC groups in the proportion of patients with initial white blood cells (WBC) ≥5 × 109/L, initial platelets (PLT) ≤26 × 109/L and arsenic trioxide (ATO) use (p < 0.05). Multivariate analysis identified initial PLT ≤26 × 109/L (p = 0.002, odds ratio [OR] = 2.679, 95% confidence interval [CI]: 1.438-4.992) as an independent risk factor, while induction therapy using realgar-indigo naturalis formula (RIF) was a protective factor (p = 0.030, OR = 0.465, 95% CI: 0.232-0.929). Further analysis revealed that Fms-like tyrosine kinase 3 (FLT3) mutation (p = 0.023, OR = 11.742, 95% CI: 1.405-98.149), initial PLT ≤26 × 109/L (p = 0.017, OR = 13.784, 95% CI: 1.598-118.905) and initial bone marrow blasts ≥90% (p = 0.030, OR = 5.289, 95% CI: 1.178-23.744) were significant risk factors for grade 4-5 DIC. Initial WBC ≥5 × 109/L and PLT ≤26 × 109/L are associated with an increased risk of DIC, with PLT ≤26 × 109/L as an independent risk factor. Compared with ATO, RIF is a protective factor during induction therapy. Additionally, FLT3 mutation, PLT ≤26 × 109/L and initial bone marrow blasts ≥90% are independent risk factors for grade 4-5 DIC.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145342178","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 efficacy and safety of immunochemotherapy in cold agglutinin-mediated autoimmune haemolytic anaemia. 免疫化疗治疗凝血素介导的自身免疫性溶血性贫血的实际疗效和安全性

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-22 DOI: 10.1111/bjh.70215

Femke V M Mulder, Anne-Marie L Becking, Sophie J Bernelot Moens, Marit Jalink, Dorothea Evers, Liane Te Boome, Anouk Breedijk, Jolanda Droogendijk, René van der Griend, Koen de Heer, Anja B U Mäkelburg, Caroline M P W Mandigers, Laurens Nieuwenhuizen, Rimke Oostvogels, J F M Hans Pruijt, Josien Regelink, Josien Rozenberg, Leonie Strobbe, Fleur M van der Valk, Birgit I Lissenberg-Witte, Josephine M I Vos

引用次数: 0

Aligning patient-reported priorities in immune thrombocytopenia with clinical and regulatory advances: Insights from rilzabrutinib. 将患者报告的免疫性血小板减少症优先事项与临床和监管进展相一致：来自利扎布替尼的见解

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-22 DOI: 10.1111/bjh.70222

Muhammad Ammar Hasan, Mobeen Abid, Hamza Maqsood Khan

引用次数: 0

CLL-like stereotyped BCRs are equally produced in young and old healthy adults, share features of public clonotypes and may be stuck at a pre-germinal centre stage. 类似cll的定型bcr在年轻和年老的健康成人中同样产生，具有公共克隆型的特征，并且可能停留在生发前的中心阶段。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-21 DOI: 10.1111/bjh.70204

Marine Dupont, Paco Derouault, Virginie Pascal, Nataliya Dmytruk, Maxime Roubinet, Charlotte Rivière, Milène Parquet, Kenza Guiyedi, Melanie Boulin, Sophie Peron, Jasmine Chauzeix, Nathalie Gachard, Jean Feuillard, David Rizzo

{"title":"CLL-like stereotyped BCRs are equally produced in young and old healthy adults, share features of public clonotypes and may be stuck at a pre-germinal centre stage.","authors":"Marine Dupont, Paco Derouault, Virginie Pascal, Nataliya Dmytruk, Maxime Roubinet, Charlotte Rivière, Milène Parquet, Kenza Guiyedi, Melanie Boulin, Sophie Peron, Jasmine Chauzeix, Nathalie Gachard, Jean Feuillard, David Rizzo","doi":"10.1111/bjh.70204","DOIUrl":"https://doi.org/10.1111/bjh.70204","url":null,"abstract":"Chronic lymphocytic leukaemia (CLL) is a B-cell lymphoproliferative disorder with ~40% of cases harbouring stereotyped B-cell receptors (BCRs), defined by common features in their immunoglobulin sequences. Major CLL stereotypes are associated with specific genetic abnormalities that may influence prognosis. To explore their physiological counterparts, we analyzed total circulating BCR repertoires from 69 healthy volunteers (HVs) aged 18-78 years. In healthy repertoires, mean abundances of 'typical' CLL-like stereotyped immunoglobulins (CLS-IG) and 'non-typical' CLS-IG (expected CDR3 sequences but different IGHV or IGHJ genes) were 0.053% and 0.46%, respectively, suggesting that CLL may arise from B cells expressing BCR patterns frequently shared between individuals. Stereotype distribution differed markedly from CLL cases: as an example, subset #2 was found in over 60% of HVs, while subset #1, the second most common in CLL, was absent. While typical CLS-IGs were rare in HVs, they dominated in CLL. Then, we longitudinally analyzed BCR repertoires within sorted circulating B-cell subpopulations in a second dataset. This revealed that the vast majority of healthy B cells carrying major CLS-IGs appeared to be stuck at pre-germinal centre stages. Similar to t(14;18) translocations in healthy individuals, stereotyped BCRs may lack intrinsic oncogenic potential and require additional selection events to drive CLL transformation.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145336139","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

Reversing the sequence: Efficacy of BCMA-directed therapy following progression on GPRC5D-directed therapies in relapsed multiple myeloma. 逆转顺序：bcma定向治疗在gprc5d定向治疗进展后对复发性多发性骨髓瘤的疗效

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-19 DOI: 10.1111/bjh.70176

Laura Joiner, Smith Giri, Gayathri Ravi, Kelly Godby, Caitlin Hagedorn, Luciano J Costa, Susan Bal

引用次数: 0

Paradoxical role and therapeutic potential of autophagy in lymphomas. 自噬在淋巴瘤中的矛盾作用和治疗潜力。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-19 DOI: 10.1111/bjh.70214

Qian-Qian Yu, Chuyun Quan, Yijing Li, Ruifang Sun

引用次数: 0

Immune reconstitution after CD7 CAR-T cell therapy for refractory/relapsed acute T-lymphoblastic leukaemia/lymphoblastic lymphoma (R/R T-ALL/LBL). CD7 CAR-T细胞治疗难治性/复发性急性t淋巴细胞白血病/淋巴母细胞淋巴瘤（R/R T-ALL/LBL）后的免疫重建

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-19 DOI: 10.1111/bjh.70201

Xian Zhang, Lin Wang, Na Kuang, Junfang Yang, Hui Wang, Liyuan Qiu, Dongchu Wang, Jian Sun, Jing Long, Peihua Lu

{"title":"Immune reconstitution after CD7 CAR-T cell therapy for refractory/relapsed acute T-lymphoblastic leukaemia/lymphoblastic lymphoma (R/R T-ALL/LBL).","authors":"Xian Zhang, Lin Wang, Na Kuang, Junfang Yang, Hui Wang, Liyuan Qiu, Dongchu Wang, Jian Sun, Jing Long, Peihua Lu","doi":"10.1111/bjh.70201","DOIUrl":"https://doi.org/10.1111/bjh.70201","url":null,"abstract":"CD7 Chimeric Antigen Receptor-T cell (CAR-T) therapy demonstrates efficacy in relapsed/refractory (R/R) acute T-lymphoblastic leukaemia (ALL)T-ALL/lymphoblastic lymphoma (LBL), but concerns about T-cell depletion and severe immunodeficiency persist. We compared infection rates and immune cell subsets in 60 R/R T-ALL/LBL patients receiving naturally selected CD7 CAR-T (NS7CAR-T) with 60 R/R B-ALL patients undergoing CD19 CAR-T. Infections were monitored from infusion until allogeneic haematopoietic stem cell transplantation (HSCT) or up to 3 months. Overall infection rates did not significantly differ between groups (36.67% vs. 24.56%, p = 0.24), although the incidence of early immune effector cell-associated haematotoxicity (ICAHT) grade III-IV was higher in the CD7 CAR-T group than in the CD19 CAR-T group (33.9% vs. 16.7%, p = 0.03). Post-CD7 CAR-T infusion analysis showed a significant decline in CD7(+) T cells and an increase in non-CAR-T-derived CD7(-) T cells, particularly non-CAR-T cells, which rose to a median proportion of 84.4% (range: 22.1%-99.9%) by day 28; meanwhile, CD7(-) natural killer (NK) cells approached nearly 100% following the depletion of CD7(+) NK cells. This study indicates that while CD7 CAR-T therapy significantly reduces CD7(+) T cells, it does not lead to increased short-term infection rates. The notable expansion of non-CAR-T-derived CD7(-) T and NK cells helps preserve immune function, highlighting distinct therapeutic mechanisms between CD7 CAR-T and CD19 CAR-T due to their different lineage restrictions.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145327859","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

Impact of methotrexate-dosing regimens for GVHD prophylaxis on clinical outcomes of HLA-matched allogeneic HSCT. 甲氨蝶呤给药方案预防GVHD对hla匹配异体造血干细胞移植临床结果的影响。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-19 DOI: 10.1111/bjh.70213

Tomotaka Suzuki, Tomoyasu Jo, Kota Yoshifuji, Tadakazu Kondo, Noriko Doki, Yoshinobu Kanda, Tetsuya Nishida, Yasushi Onishi, Noboru Asada, Takahiro Fukuda, Masashi Sawa, Yuta Hasegawa, Kentaro Serizawa, Shuichi Ota, Masatsugu Tanaka, Makoto Yoshimitsu, Yoshiko Atsuta, Junya Kanda

{"title":"Impact of methotrexate-dosing regimens for GVHD prophylaxis on clinical outcomes of HLA-matched allogeneic HSCT.","authors":"Tomotaka Suzuki, Tomoyasu Jo, Kota Yoshifuji, Tadakazu Kondo, Noriko Doki, Yoshinobu Kanda, Tetsuya Nishida, Yasushi Onishi, Noboru Asada, Takahiro Fukuda, Masashi Sawa, Yuta Hasegawa, Kentaro Serizawa, Shuichi Ota, Masatsugu Tanaka, Makoto Yoshimitsu, Yoshiko Atsuta, Junya Kanda","doi":"10.1111/bjh.70213","DOIUrl":"https://doi.org/10.1111/bjh.70213","url":null,"abstract":"Severe graft-versus-host disease (GVHD) remains a major complication of allogeneic haematopoietic stem cell transplantation (allo-HSCT), necessitating optimal immunosuppressive strategies. This retrospective study used data from the Japanese Transplant Registry Unified Management Program to compare three methotrexate (MTX)-dosing regimens for GVHD prophylaxis in patients undergoing human leucocyte antigen (HLA)-matched allo-HSCT: a low-dose 3-day regimen (Ld3:10 mg/m2 on day 1, 7 mg/m2 on days 3 and 6), a low-dose 4-day regimen (Ld4: Ld3 with an additional 7 mg/m2 on day 11) and an original-dose 3-day regimen (Od3: 15 mg/m2 on day 1, 10 mg/m2 on days 3 and 6). Among 2537 analysed patients, Ld3 was the most commonly used regimen. Multivariate analyses showed no significant differences in the cumulative incidence of grade II-IV acute GVHD among regimens. However, Od3 was associated with an increased risk of grade III-IV acute GVHD, and Ld4 was linked to delayed neutrophil engraftment. This study is the first large-scale retrospective analysis of the impact of different MTX-dosing regimens on the outcomes of HLA-matched allo-HSCT, providing valuable insights into optimal MTX-dosing strategies in clinical practice.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145327889","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

Cardiovascular complications and their association with short- and long-term outcomes in patients with multiple myeloma undergoing chimeric antigen receptor T-cell therapy. 接受嵌合抗原受体t细胞治疗的多发性骨髓瘤患者的心血管并发症及其与短期和长期预后的关系

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-10-19 DOI: 10.1111/bjh.70216

Osnat Itzhaki Ben Zadok, Panagiotis Simitsis, Caron Jacobson, Omar Nadeem, Matthew J Frigault, Noopur Raje, Caitlyn Duffy, Patrick Costello, Jamie Dela Cruz, Andrew Looka, Anju Nohria

{"title":"Cardiovascular complications and their association with short- and long-term outcomes in patients with multiple myeloma undergoing chimeric antigen receptor T-cell therapy.","authors":"Osnat Itzhaki Ben Zadok, Panagiotis Simitsis, Caron Jacobson, Omar Nadeem, Matthew J Frigault, Noopur Raje, Caitlyn Duffy, Patrick Costello, Jamie Dela Cruz, Andrew Looka, Anju Nohria","doi":"10.1111/bjh.70216","DOIUrl":"https://doi.org/10.1111/bjh.70216","url":null,"abstract":"Studies of cardiovascular (CV) events after chimeric antigen receptor T-cell (CAR-T) therapy have mostly focused on patients with lymphoma. We evaluated the incidence, timing and prognostic significance of in-hospital and post-discharge CV events in patients with multiple myeloma (MM) receiving CAR-T therapy. We conducted a retrospective analysis of MM patients treated with CAR-T between 2018 and 2024. CV events (heart failure, arrhythmias, myocardial infarction and stroke) and all-cause mortality were assessed separately during hospitalization and post-discharge. Time-dependent Cox regression was used to evaluate associations with mortality. Among 256 patients, 11.7% (n = 30) experienced in-hospital CV events, most commonly atrial arrhythmias (5.5%) and new left ventricular (LV) dysfunction (3.9%), with a median onset of 8 (Q1, Q3: 5, 11) days. Ninety percent of those with LV dysfunction recovered function. Independent predictors of in-hospital CV events included age >65, prior atrial fibrillation, antiplatelet use, cytokine release syndrome grade ≥2 and higher immune effector cell-associated neurotoxicity syndrome grade. Post-discharge CV events were less common (7.8%), occurring at a median of 13 (Q1, Q3: 7, 22) months. Only post-discharge CV events were associated with higher post-discharge all-cause mortality. There were no in-hospital CV deaths and one post-discharge CV death. In this large MM CAR-T cohort, in-hospital CV events were relatively uncommon, reversible and not linked to mortality. In contrast, post-discharge CV events, while rare, predicted worse survival.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145327847","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