British Journal of Haematology最新文献_第2页

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

Oral iptacopan for paroxysmal nocturnal haemoglobinuria-First real-world experience.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-19 DOI: 10.1111/bjh.20024

Richard J Kelly, Austin G Kulasekararaj, Louise M Arnold, Catherine Barnfield, Isla Storey, Jessica Molyneux, Nora L Youngs, Joanna Large, Lindsay Mitchell, Sateesh K Nagumantry, Shreyans Gandhi, Roochi Trikha, Abraham Varghese, Talha Munir, Petra Muus, Morag Griffin

引用次数: 0

Longitudinal analysis of lentiviral and retroviral chimeric antigen receptors' integration sites reveals distinct clonal evolutionary patterns.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-19 DOI: 10.1111/bjh.20020

Vincent Guiraud, Jerome Alexandre Denis, Ghizlane Benhafoun, Erwan Ablin, Sophie Sayon, Laetitia Souchet, Nabih Azar, Adrien Grenier, Carole Metz, Ronan Legrand, Anne-Geneviève Marcelin, Sylvain Choquet, Vincent Calvez, Eve Todesco

{"title":"Longitudinal analysis of lentiviral and retroviral chimeric antigen receptors' integration sites reveals distinct clonal evolutionary patterns.","authors":"Vincent Guiraud, Jerome Alexandre Denis, Ghizlane Benhafoun, Erwan Ablin, Sophie Sayon, Laetitia Souchet, Nabih Azar, Adrien Grenier, Carole Metz, Ronan Legrand, Anne-Geneviève Marcelin, Sylvain Choquet, Vincent Calvez, Eve Todesco","doi":"10.1111/bjh.20020","DOIUrl":"https://doi.org/10.1111/bjh.20020","url":null,"abstract":"T-cell malignancies following chimeric antigen receptor (CAR) therapies are partly related to insertional mutagenesis, but the longitudinal evolution of CAR-integration sites (IS) remains understudied. We performed an IS analysis in blood from three tisagenlecleucel (lentiviral), one axicabtagene-ciloleucel and one brexucabtagene-autoleucel (gammaretrovirals) patient at peak expansion and 1-year follow-up. All were complete responders. Peak expansion IS patterns were vector dependent: lentiviral CAR integrated mostly in introns and gammaretrovirals in intergenic regions, closer to transcription start sites. At 1-year post-infusion, lentiviral CAR showed no major clonal proliferation. Gammaretroviral CARs had divergent outcomes: no detectable CAR (axicabtagene-ciloleucel) or low-level oligoclonal persistence (brexucabtagene-autoleucel). Whether this latter evolution is at risk of further CAR malignancies needs further investigations.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143456428","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

Zanubrutinib for the treatment of Bing-Neel syndrome.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-18 DOI: 10.1111/bjh.20016

Shayna Sarosiek, Andres Ramirez-Gamero, Catherine A Flynn, Steven P Treon, Jorge J Castillo

引用次数: 0

Understanding variations in the use of tranexamic acid in surgery: A qualitative interview study.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-18 DOI: 10.1111/bjh.20008

Louise Strickland, Hayley G Evans, Antony Palmer, Samantha Warnakulasuriya, Michael F Murphy, Simon J Stanworth, Robbie Foy

{"title":"Understanding variations in the use of tranexamic acid in surgery: A qualitative interview study.","authors":"Louise Strickland, Hayley G Evans, Antony Palmer, Samantha Warnakulasuriya, Michael F Murphy, Simon J Stanworth, Robbie Foy","doi":"10.1111/bjh.20008","DOIUrl":"https://doi.org/10.1111/bjh.20008","url":null,"abstract":"Despite robust supporting evidence, around a third of eligible surgical patients do not receive tranexamic acid (TXA). Effective strategies based on an understanding of clinical behaviour are needed to increase use and improve patient outcomes. We conducted semi-structured interviews with clinicians involved in perioperative care to explore perceived influences on TXA use. We identified key influences on practice using the theoretical domains framework. We matched these to behaviour change techniques and evidence-informed implementation intervention components. Across 22 interviews, we identified eight key influences within three overarching themes of capability, opportunity and motivation. Capability influences included the clinical context and variable familiarity with TXA. Opportunity concerned the availability of both TXA and checklists to support decision-making and whether TXA use was consistent with professional expectations and perceived responsibilities. Motivation concerned confidence in administering TXA, perceived benefits and risks and training received around potential risk factors. These influences varied across participants and specialities. Our resulting proposed implementation strategy included training, clinical prompts, comparative performance feedback and opinion leadership supported by specialty-specific guidance. Any strategy to increase TXA use that improves knowledge and skills without addressing wider influences on clinical behaviour is only likely to meet with limited success.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143447792","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

Clinical and molecular characteristics of paediatric mature B-cell acute lymphocytic leukaemia and non-Hodgkin lymphoma with bone marrow involvement: A joint study between the CCCG leukaemia and lymphoma groups.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-17 DOI: 10.1111/bjh.20011

Jie Zhao, Tian-Feng Liu, Ke-Fei Wu, Liang-Chun Yang, Xue-Ju Xu, Jun Lu, Jing-Bo Shao, Fu Li, Fu-Tian Ma, Xia Guo, Hui Li, Ai-Guo Liu, Ning-Ling Wang, He-Ping Shen, Yang Li, Si-Xi Liu, Chang-Da Liang, Shu-Hong Shen, Yong-Jun Fang, Yi-Jin Gao

{"title":"Clinical and molecular characteristics of paediatric mature B-cell acute lymphocytic leukaemia and non-Hodgkin lymphoma with bone marrow involvement: A joint study between the CCCG leukaemia and lymphoma groups.","authors":"Jie Zhao, Tian-Feng Liu, Ke-Fei Wu, Liang-Chun Yang, Xue-Ju Xu, Jun Lu, Jing-Bo Shao, Fu Li, Fu-Tian Ma, Xia Guo, Hui Li, Ai-Guo Liu, Ning-Ling Wang, He-Ping Shen, Yang Li, Si-Xi Liu, Chang-Da Liang, Shu-Hong Shen, Yong-Jun Fang, Yi-Jin Gao","doi":"10.1111/bjh.20011","DOIUrl":"https://doi.org/10.1111/bjh.20011","url":null,"abstract":"Mature B-cell acute lymphocytic leukaemia (B-ALL) is distinguished from B-cell non-Hodgkin lymphoma (B-NHL) by the arbitrariness of the 25% cut-off, and given that the percentage of bone marrow (BM) blasts can vary according to site of aspirate, we refrained from differentiating mature B-ALL from B-NHL with BM infiltration. A total of 156 patients from the Chinese Children Cancer Group with BM blasts of more than 5% and consistent with immunophenotypic features of mature B cells were included in this study. The 2-year progression-free survival, 2-year event-free survival and 2-year overall survival were 76.6 ± 3.6%, 69.7 ± 3.7% and 80.1 ± 3.3% respectively. Central nervous system (CNS) involvement, serum ferritin levels higher than four times normal and rituximab no more than two doses were associated with lower PFS. Male, bulky disease and head/neck region involvement were associated with higher rate of CNS invasion. We performed an integrative transcriptomic characterization of 36 cases. Structure variant included IG::MYC, IGH::CACS11, MEF2D::BCL9, IGH::VPS53 and ACIN1::NUTM1. SNV analysis uncovered driver variations affecting 10 recurrently mutated genes including ID3, TP53, MYC, ARID1A, SMARCA4, DDX3X, CCND3, RHOA, SMARCB1, FOXO1 and GNA13. Mature B-ALL/B-NHL with BM involvement was a heterogeneous group of malignancies in both clinical features and genetic alternations. Genetics analysis was helpful for making accurate diagnoses and guiding appropriate therapeutic strategies.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143439650","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

Immunoglobulin inclusions were released from lymphoma cells and deposited as cryoglobulin crystals in a peripheral blood film.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-17 DOI: 10.1111/bjh.20005

Geng Wang, Fei Wang

引用次数: 0

Immunosuppressive therapy as first-line and relapse treatment in paediatric subcutaneous panniculitis-like T-cell lymphoma.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-02-16 DOI: 10.1111/bjh.20014

Young Kwon Koh, Su Hyun Yoon, Sung Han Kang, Kyung-Nam Koh, Ho Joon Im, Hyery Kim

引用次数: 0

Characteristics of 'high-user' adult patients with sickle cell disease in France: Lessons to be learned.