British Journal of Haematology最新文献_第9页

Gene expression signature of IKZF1 deleted B-cell acute lymphoblastic leukaemia reveals a unique regulation of MUC4. IKZF1缺失的b细胞急性淋巴细胞白血病的基因表达特征揭示了MUC4的独特调控。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-28 DOI: 10.1111/bjh.70014

Preity Sharma, Gadha K Leons, Vivek Singh, Saadia Naseer, Louis Ribeyron, Sameer Bakhshi, Ritu Gupta, Smeeta Gajendra, Debraj Singh Thoudam, Surender Kumar Sarawat, Maroof Ahmed Khan, Deepam Pushpam, Ranjit Kumar Sahoo, Anita Roy, Sanjeev Kumar Gupta

{"title":"Gene expression signature of IKZF1 deleted B-cell acute lymphoblastic leukaemia reveals a unique regulation of MUC4.","authors":"Preity Sharma, Gadha K Leons, Vivek Singh, Saadia Naseer, Louis Ribeyron, Sameer Bakhshi, Ritu Gupta, Smeeta Gajendra, Debraj Singh Thoudam, Surender Kumar Sarawat, Maroof Ahmed Khan, Deepam Pushpam, Ranjit Kumar Sahoo, Anita Roy, Sanjeev Kumar Gupta","doi":"10.1111/bjh.70014","DOIUrl":"https://doi.org/10.1111/bjh.70014","url":null,"abstract":"The IKAROS (IKZF1) transcription factor regulates the differentiation of pre-B cells to mature B lymphocytes. The loss of IKZF1 expression has been linked with increased matrix adhesion along with increased expression of stemness markers that form a hallmark of B-lymphoblasts. However, the spectrum of differentially regulated genes has not been investigated in detail in B-cell acute lymphoblastic leukaemia (B-ALL) patients expressing IKZF1 deletion. In this study, we analysed the gene expression signature of 24 B-ALL patients with IKZF1 deletion against 32 B-ALL patients with wild-type IKZF1. RNA sequencing revealed an adhesion signature corresponding to deregulation of MUC4, secreted phosphoprotein 1 (SPP1), LAMB3, CLSTN2 and MERTK. The upregulation of MUC4 expression was further validated in a B-ALL patient cohort. Moreover, by chromatin immunoprecipitation, we observed the direct binding of IKZF1 on MUC4 promoter and enhancer sequences. Thus, the increase in MUC4 expression upon IKZF1 deletion could be explained by a loss of IKZF1 repression on the MUC4 locus. Finally, using the TARGET-ALL-P2 database, we observed a correlation between decreased survival and high MUC4 expression in B-ALL patients. Thus, we propose MUC4 expression as an indicator of patient prognosis that correlates with the loss of IKZF1 in B-ALL.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144937080","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

Paediatric-inspired regimen reduces central nervous system relapse in adult acute lymphoblastic leukaemia. 儿科启发方案减少中枢神经系统复发的成人急性淋巴细胞白血病。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-27 DOI: 10.1111/bjh.70067

Haimei Liang, Bailin He, Junjie Chen, Baiwei Luo, Zihong Cai, Chenhao Ding, Jieping Lin, Zhixiang Wang, Xuan Zhou, Xiaoli Liu, Li Xuan, Qifa Liu, Hongsheng Zhou

{"title":"Paediatric-inspired regimen reduces central nervous system relapse in adult acute lymphoblastic leukaemia.","authors":"Haimei Liang, Bailin He, Junjie Chen, Baiwei Luo, Zihong Cai, Chenhao Ding, Jieping Lin, Zhixiang Wang, Xuan Zhou, Xiaoli Liu, Li Xuan, Qifa Liu, Hongsheng Zhou","doi":"10.1111/bjh.70067","DOIUrl":"https://doi.org/10.1111/bjh.70067","url":null,"abstract":"Central nervous system (CNS) is the common site of extramedullary relapse in adult acute lymphoblastic leukaemia (ALL) and the outcome of CNS relapse patients remains poor. This study aimed to investigate whether a paediatric-inspired chemotherapy regimen can reduce CNS relapse in adult ALL compared to the adult regimen. A total of 1005 newly diagnosed adult patients with ALL were enrolled in this study, including 596 patients who received the Hyper-CVAD (cyclophosphamide, vincristine, doxorubicin[adriamycin], and dexamethason) adult regimen and 409 patients who treated with the Precision Classification-Directed Target Total Therapy-ALL-2016 (PDT-ALL-2016) paediatric-inspired chemotherapy regimen. 11.24% of patients treated with the adult regimen (67/596) experienced a CNS recurrence, whereas only 7.09% of patients (29/409) had CNS relapse under the paediatric-inspired chemotherapy regimen. The 5-year cumulative incidence of relapse in the CNS for the paediatric cohort and adult cohort was 7.09% and 11.24% respectively (p = 0.025). Even for patients in the high-risk group, the 5-year cumulative incidence of CNS relapse was significantly lower in the paediatric cohort compared with the adult cohort (6.75% vs. 13.7%, p = 0.003). The univariate analysis revealed that the paediatric-inspired regimen is a protective factor for reducing CNS relapse (OR = 0.6, p = 0.029). Collectively, our data suggest that paediatric-inspired chemotherapy may reduce the risk of CNS relapse in adult ALL compared to adult regimens.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144937069","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

A decade of experience using ATG and PTCy for graft-versus-host disease prophylaxis in matched unrelated donor allogeneic haematopoietic cell transplantation. ATG和PTCy在配对非亲属供体异体造血细胞移植中预防移植物抗宿主病的十年经验

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-27 DOI: 10.1111/bjh.70124

Nihar Desai, Sergio Rodriguez-Rodriguez, Eshrak Al-Shaibani, Tommy Alfaro Moya, Arjun Datt Law, Igor Novitzky-Basso, Ivan Pasic, Fotios V Michelis, Rajat Kumar, Dennis Dong Hwan Kim, Jonas Mattsson, Auro Viswabandya

{"title":"A decade of experience using ATG and PTCy for graft-versus-host disease prophylaxis in matched unrelated donor allogeneic haematopoietic cell transplantation.","authors":"Nihar Desai, Sergio Rodriguez-Rodriguez, Eshrak Al-Shaibani, Tommy Alfaro Moya, Arjun Datt Law, Igor Novitzky-Basso, Ivan Pasic, Fotios V Michelis, Rajat Kumar, Dennis Dong Hwan Kim, Jonas Mattsson, Auro Viswabandya","doi":"10.1111/bjh.70124","DOIUrl":"https://doi.org/10.1111/bjh.70124","url":null,"abstract":"Anti-thymocyte globulin (ATG) and post-transplant cyclophosphamide (PTCy) are both effective drugs for graft-versus-host disease (GvHD) prophylaxis, but their combined use remains underexplored. We conducted a retrospective analysis of 582 patients with haematological malignancies who underwent unrelated donor transplantation with Status GvHD prophylaxis consisting of low-dose ATG (2 mg/kg) and PTCy over a 10-year period. The median time to neutrophil and platelet engraftment was 18 days (95% CI, 17-19) and 20 days (95% CI, 19-21) respectively. The cumulative incidence of grade III-IV acute Status GvHD at D+100 was 7% (95% CI: 5-9), and the 24-month incidence of moderate-to-severe chronic GvHD was 15.7% (95% CI: 13-19). Clinically significant cytomegalovirus (CMV) and Epstein-Barr virus (EBV) reactivation by D+180 occurred in 20.4% (95% CI: 17-24) and 22.7% (95% CI: 19-26) respectively. At 24 months, the cumulative incidence of NRM was 16.2% (95% CI: 13-19), and relapse was 21.3% (95% CI: 18-25). Graft-versus-host disease-free, relapse-free survival (GRFS) at 24 months was 49% (95% CI: 44-53), and overall survival (OS) was 68.3% (95% CI: 64-72). Our decade-long experience supports the safety and efficacy of combining low-dose ATG with PTCy for GvHD prophylaxis in unrelated donor transplantation. These findings warrant further validation in prospective studies.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144937051","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

Improved identification of T-ALL paediatric patients with low MRD but poor prognosis using a transcriptional signature. 利用转录标记提高对低MRD但预后不良的T-ALL儿科患者的识别。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-27 DOI: 10.1111/bjh.70118

Antonio Lahera, Laura Vela-Martín, Pablo Fernández-Navarro, José Luis López-Lorenzo, Javier Cornago, Pilar Llamas, Manuel Fresno, Javier Santos, María Villa-Morales

引用次数: 0

Comprehensive risk stratification for adult T-cell lymphoblastic lymphoma undergoing allogeneic haematopoietic stem cell transplantation. 接受同种异体造血干细胞移植的成人t细胞淋巴母细胞淋巴瘤的综合风险分层。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-26 DOI: 10.1111/bjh.70102

Fangfang Yu, Xiaolin Yu, Xiaoxia Hu, Erlie Jiang, Jian Zhou, Ying Lu, Xiaoyu Zhu, Jiangbo Wan, Kourong Miao, Yuewen Fu, Jiahua Niu, Liping Wan, Fang Zhou, Xianmin Song

{"title":"Comprehensive risk stratification for adult T-cell lymphoblastic lymphoma undergoing allogeneic haematopoietic stem cell transplantation.","authors":"Fangfang Yu, Xiaolin Yu, Xiaoxia Hu, Erlie Jiang, Jian Zhou, Ying Lu, Xiaoyu Zhu, Jiangbo Wan, Kourong Miao, Yuewen Fu, Jiahua Niu, Liping Wan, Fang Zhou, Xianmin Song","doi":"10.1111/bjh.70102","DOIUrl":"https://doi.org/10.1111/bjh.70102","url":null,"abstract":"Relapse remains the primary cause of transplant failure in T-cell lymphoblastic lymphoma (T-LBL) after allogeneic haematopoietic stem cell transplantation (allo-HSCT), yet prognostic data are scarce. We conducted a multicentre retrospective study of 104 adult T-LBL patients in complete metabolic remission (CMR) undergoing allo-HSCT. Pretransplant Deauville score (DS) was evaluated, and a risk model integrating DS, immunophenotype and donor type was developed. Patients with DS of 3 had significantly inferior 2-year overall survival (OS) (70.5% vs. 77.4% vs. 86.8%, p < 0.001), progression-free survival (PFS) (51.5% vs. 73.0% vs. 81.1%, p < 0.001) and higher relapse rates (42.1% vs. 15.4% vs. 9.8%, p < 0.001) compared to DS of 1-2. Multivariable analysis identified pretransplant DS, immunophenotype and donor type as independent risk factors for post-transplant relapse. These variables were integrated to construct a novel risk model stratifying patients into low- (66.0%), intermediate- (21.3%) and high-risk (12.7%) groups, strongly discriminating OS (p < 0.001), PFS (p < 0.001) and relapse (p < 0.001), with a C-index of 0.706. Internal validation confirmed that the risk stratification model had good discrimination, calibration and clinical benefit. Our findings propose a clinically actionable tool to identify high-risk T-LBL patients for tailored post-transplant strategies.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144937019","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

Quality of life the first year after allogeneic haematopoietic stem cell transplantation-A randomized controlled trial examining extracorporeal photophoresis to prevent graft-versus-host disease. 同种异体造血干细胞移植后第一年的生活质量——一项检查体外光导入预防移植物抗宿主病的随机对照试验

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-26 DOI: 10.1111/bjh.70119

Per Ole Iversen, Tobias Gedde-Dahl, Geir Erland Tjønnfjord, Maryan Mohamed Ali

引用次数: 0

Back to life: Biological parenthood of young adult acute leukaemia survivors. 回归生活：年轻成年急性白血病幸存者的亲生父母。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-26 DOI: 10.1111/bjh.70107

Katharina Egger-Heidrich, Katja Sockel, Johannes Schetelig

引用次数: 0

Outcomes and risk factors for complications in deletional haemoglobin H disease: A retrospective longitudinal cohort study in British Columbia, Canada. 缺失型血红蛋白H病并发症的结局和危险因素：加拿大不列颠哥伦比亚省的一项回顾性纵向队列研究

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-26 DOI: 10.1111/bjh.70123

Jacqueline Blunt, Melissa Braschel, Heather McCartney, Navdeep Sandhu, Hayley Merkeley, Ali Amid

引用次数: 0

Reconsidering 'primary myelofibrosis with increased haemoglobin' within the myeloproliferative neoplasms biological continuum. 在骨髓增殖性肿瘤生物学连续体中重新考虑“原发性骨髓纤维化伴血红蛋白增高”。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-26 DOI: 10.1111/bjh.70103

Hans Carl Hasselbalch

引用次数: 0

Real-world efficacy and safety of fostamatinib in ITP patients: Italian multicentre experience. GIMEMA ITP1122 study. 福司他替尼在ITP患者中的实际疗效和安全性：意大利多中心经验。GIMEMA ITP1122研究。

IF 3.8 2区医学

British Journal of Haematology Pub Date : 2025-08-25 DOI: 10.1111/bjh.70069

Elisa Lucchini, Nicola Vianelli, Ugo Consoli, Giuseppe Carli, Valentina Carrai, Monica Carpenedo, Valerio De Stefano, Gianluca Gaidano, Francesca Palandri, Giuseppe Auteri, Elena Rossi, Andrea Patriarca, Vanessa Innao, Gessica Marchesini, Mario Ballerini, Maria Chiara Rossetti, Giulia Mosini, Patrizia Sciancalepore, Jacopo Agnelli Giacchello, Pellegrino Musto, Elena Rivolti, Giulia Soverini, Alessandra Borchiellini, Cristina Clissa, Fabrizio Pane, Sara Galimberti, Vincenzo Pavone, Antonella Poloni, Francesco Buccisano, Maurizio Musso, Paola Fazi, Francesca Paoloni, Livia Gorreo Renzulli, Francesco Zaja

{"title":"Real-world efficacy and safety of fostamatinib in ITP patients: Italian multicentre experience. GIMEMA ITP1122 study.","authors":"Elisa Lucchini, Nicola Vianelli, Ugo Consoli, Giuseppe Carli, Valentina Carrai, Monica Carpenedo, Valerio De Stefano, Gianluca Gaidano, Francesca Palandri, Giuseppe Auteri, Elena Rossi, Andrea Patriarca, Vanessa Innao, Gessica Marchesini, Mario Ballerini, Maria Chiara Rossetti, Giulia Mosini, Patrizia Sciancalepore, Jacopo Agnelli Giacchello, Pellegrino Musto, Elena Rivolti, Giulia Soverini, Alessandra Borchiellini, Cristina Clissa, Fabrizio Pane, Sara Galimberti, Vincenzo Pavone, Antonella Poloni, Francesco Buccisano, Maurizio Musso, Paola Fazi, Francesca Paoloni, Livia Gorreo Renzulli, Francesco Zaja","doi":"10.1111/bjh.70069","DOIUrl":"https://doi.org/10.1111/bjh.70069","url":null,"abstract":"The efficacy and safety of fostamatinib in adult patients with persistent/chronic immune thrombocytopenia (p/c ITP) were demonstrated in the FIT-1/FIT-2 trials. This retrospective, multicentre observational study evaluated real-world outcomes in consecutive p/c ITP patients treated with fostamatinib in Italy. The primary end-point, serving as a surrogate for both efficacy and safety, was the proportion of patients receiving fostamatinib for at least 6 months. Ninety-five patients were enrolled across 20 Italian centres, 59% female, median age 64 years (range 21-86). The median time from ITP diagnosis to fostamatinib initiation was 7.7 years; the median number of prior therapies was 4, including 54% of patients who received more than one thrombopoietin receptor agonist (TPO-RA) and 23% who underwent splenectomy. The overall response rate was 73%, with 32% complete responses. Most patients (74%) achieved a response within the first month. Concerning the primary end-point, 45% of patients received fostamatinib for 6 months; the median treatment duration was 7.3 months. The main cause of discontinuation was treatment failure (43%). Fifty-nine adverse events were reported in 38 patients, mostly grade 1-2, leading to fostamatinib discontinuation in 8% of patients. This real-world data confirm fostamatinib as an effective and safe option in relapsed/refractory ITP.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144937052","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