British Journal of Haematology最新文献

Origins of T-cell-mediated autoimmunity in acquired aplastic anaemia. 获得性再生障碍性贫血中t细胞介导自身免疫的起源。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-21 DOI: 10.1111/bjh.19993

Aura Enache, Shannon A Carty, Daria V Babushok

引用次数: 0

Fibrinogen genotypes and their impact on recurrence of venous thromboembolism and family history: A prospective population-based study. 纤维蛋白原基因型及其对静脉血栓栓塞复发和家族史的影响：一项基于人群的前瞻性研究。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-19 DOI: 10.1111/bjh.19999

Ashfaque A Memon, Bengt Zöller, Peter J Svensson, Jan Sundquist, Kristina Sundquist

{"title":"Fibrinogen genotypes and their impact on recurrence of venous thromboembolism and family history: A prospective population-based study.","authors":"Ashfaque A Memon, Bengt Zöller, Peter J Svensson, Jan Sundquist, Kristina Sundquist","doi":"10.1111/bjh.19999","DOIUrl":"https://doi.org/10.1111/bjh.19999","url":null,"abstract":"Venous thromboembolism (VTE) involves blood clot formation in veins, resulting in serious health issues. Fibrinogen, a crucial clotting protein, consists of three polypeptides encoded by the fibrinogen genes: alpha (FGA), beta (FGB) and gamma (FGG). We genotyped most common missense variants in the fibrinogen genes in relation to VTE, recurrence and family history in Malmö Thrombophilia Study, including 1465 VTE patients followed for ~10 years and 429 healthy donors. FGG (rs6063) was significantly associated with increased odds of primary VTE (odds ratio [OR] = 8.2; 95% confidence interval [CI] = 1.05-63.6) after adjusting for age and sex. For recurrent VTE, Cox-regression analysis indicated a higher risk associated with FGA (rs6050) (hazard ratio [HR] = 1.8; 95% CI = 1.1-2.8), with even greater risk for unprovoked recurrent VTE (HR = 2.3; 95% CI = 1.3-4.2), surpassing the well-known factor V Leiden (FVL) (HR = 1.9; 95% CI = 1.2-3.0). Combining risk alleles from FVL and FGA (rs6050) significantly raised the risk for unprovoked recurrent VTE: ≥3 risk alleles (HR = 4.6; 95% CI = 1.9-11.3), two risk alleles (HR = 2.6; 95% CI = 1.4-4.8) and one risk allele (HR = 1.5; 95% CI = 0.8-2.7) compared to 0 risk allele. Prevalence of FGA (rs6050) risk allele was significantly higher in cases with a family history of VTE. We propose FGA (rs6050) as a novel predictor for unprovoked recurrent VTE and it may contribute to the familial occurrence of VTE.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996835","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

CAR T access and outcomes in large B-cell lymphoma according to ethnicity and socioeconomic deprivation in the UK. 在英国，根据种族和社会经济剥夺，大b细胞淋巴瘤的CAR - T获取和结果

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-16 DOI: 10.1111/bjh.19997

Diana Dragoi, Samuel Cusworth, Laura Oldham, Robin Sanderson, Jane Norman, Joht Chandan, Amrith Mathew, Emil Kumar, Shankara Paneesha, Eleni Tholouli, Andres Moya Davila, Styliani Bouziana, Piers Patten, Prudence Hardefeldt, Deborah Yallop, Sridhar Chaganti, David Burns, Andrea Kuhnl

{"title":"CAR T access and outcomes in large B-cell lymphoma according to ethnicity and socioeconomic deprivation in the UK.","authors":"Diana Dragoi, Samuel Cusworth, Laura Oldham, Robin Sanderson, Jane Norman, Joht Chandan, Amrith Mathew, Emil Kumar, Shankara Paneesha, Eleni Tholouli, Andres Moya Davila, Styliani Bouziana, Piers Patten, Prudence Hardefeldt, Deborah Yallop, Sridhar Chaganti, David Burns, Andrea Kuhnl","doi":"10.1111/bjh.19997","DOIUrl":"https://doi.org/10.1111/bjh.19997","url":null,"abstract":"Data on the impact of ethnic and socioeconomic factors on Chimeric antigen receptor (CAR) T-cell therapy (access and outcomes are limited, but key to understand whether results from the registration trials are generalizable to real-world patient populations. Here, we analysed ethnicity, socioeconomic deprivation and referral patterns in a cohort of 314 large B-cell lymphoma patients approved for third-line CD19 CAR-T across three large UK CAR-T centres. Patients from deprived areas had a lower infusion rate compared to low deprivation areas (73% vs. 86%, p = 0.04). CAR-T response rates, toxicities, progression-free survival or non-relapse mortality were similar with respect to ethnicity or deprivation. We did not find evidence of referral barriers according to ethnicity, but potential regional barriers for socioeconomically deprived patients in two of three centres. Intention-to-treat overall survival was significantly inferior in patients from deprived areas (1-year OS 44.5% vs. 58% for high vs. low deprivation; p = 0.02), likely reflecting general health disparities and higher drop-out rates in this group. Our data suggest similar outcomes of CD19 CAR-T-treated patients across a socioeconomically and ethnically heterogeneous real-world population. Results demonstrate broad access to CAR-T within the UK national delivery system, but the high drop-out rate and potential regional referral barriers for deprived communities should be further investigated.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996832","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

JAK2 is a critical therapeutic target in VEXAS syndrome treated with ruxolitinib. JAK2是ruxolitinib治疗VEXAS综合征的关键治疗靶点。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-15 DOI: 10.1111/bjh.19979

Andrea Ceccardi, Martina Dameri, Francesco Ravera, Nicolò Gilardi, Mario Stabile, Isabella Lombardo, Davide Ceresa, Monica Colombo, Tiziana Vigo, Benedetta Cigolini, Giulia Rivoli, Matteo Dragani, Irene Solimano, Anna Garuti, Andrea Bellodi, Alberto Ballestrero, Lorenzo Ferrando, Gabriele Zoppoli

引用次数: 0

Metastatic melanoma presenting with leucoerythroblastic blood film. 转移性黑色素瘤表现为白血病红细胞血膜。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-15 DOI: 10.1111/bjh.19974

Carlo Zaninetti, Maria Einweg, Alexandra Almann, Adrian Schwarzer

引用次数: 0

Hypomethylating agents for patients with VEXAS without myelodysplastic syndrome: Clinical outcome and longitudinal follow-up of vacuolization and UBA1 clonal dynamics. 低甲基化药物治疗无骨髓增生异常综合征的VEXAS患者：空泡化和UBA1克隆动态的临床结果和纵向随访

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-13 DOI: 10.1111/bjh.19953

Jose R Álamo, Lucía Mont-de Torres, Sandra Castaño-Díez, Anna Mensa-Vilaró, M Mónica López-Guerra, Ines Zugasti, Johana Díaz, Carlos Jiménez-Vicente, Susana Plaza, Virginia Fabregat, Iñaki Ortiz de Landazuri, Jordi Yagüe, Gerard Espinosa, Raimon Sanmartí, Maria Rozman, Francesca Guijarro, Albert Cortes, Ana Triguero, Aina Cardús, Adriana Cuartas, Marina Cornejo, Jordi Esteve, Juan I Aróstegui, Marina Díaz-Beyá

{"title":"Hypomethylating agents for patients with VEXAS without myelodysplastic syndrome: Clinical outcome and longitudinal follow-up of vacuolization and UBA1 clonal dynamics.","authors":"Jose R Álamo, Lucía Mont-de Torres, Sandra Castaño-Díez, Anna Mensa-Vilaró, M Mónica López-Guerra, Ines Zugasti, Johana Díaz, Carlos Jiménez-Vicente, Susana Plaza, Virginia Fabregat, Iñaki Ortiz de Landazuri, Jordi Yagüe, Gerard Espinosa, Raimon Sanmartí, Maria Rozman, Francesca Guijarro, Albert Cortes, Ana Triguero, Aina Cardús, Adriana Cuartas, Marina Cornejo, Jordi Esteve, Juan I Aróstegui, Marina Díaz-Beyá","doi":"10.1111/bjh.19953","DOIUrl":"https://doi.org/10.1111/bjh.19953","url":null,"abstract":"VEXAS syndrome is a haemato-inflammatory disease caused by somatic UBA1 mutations and characterized by cytoplasmic vacuoles in myeloid and erythroid precursor cells. Although there is currently no standard treatment algorithm for VEXAS, patients are generally treated with anti-inflammatory therapies focused on symptom management, with only partial effectiveness. Hypomethylating agents (HMA) have shown promise in VEXAS patients with concomitant myelodysplastic syndrome (MDS), while the efficacy of HMA in VEXAS patients without MDS is largely unknown. Furthermore, the usefulness of monitoring the variant allele frequency (VAF) of UBA1 or vacuolization in precursor cells over the course of treatment has not been extensively investigated. We have evaluated the efficacy of HMA in four VEXAS patients without MDS and performed longitudinal analyses of the VAF of UBA1 and vacuolization during treatment. HMA treatment led to overall clinical improvement, a dramatic reduction in the VAF of UBA1, normalization of haematological and inflammatory markers and a quantifiable decrease in vacuolization, leading us to speculate that unlike anti-inflammatory therapies, HMA may well act as a disease-modifying treatment. If these findings are confirmed in further studies, it could lead to the early use of HMA in the treatment of all VEXAS patients-with or without MDS.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142976875","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 anaplastic large-cell lymphoma with cardiac tamponade. 小儿无细胞大细胞淋巴瘤伴心脏填塞。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-12 DOI: 10.1111/bjh.19980

Takahiro Shiwaku, Kosuke Tamefusa, Hisashi Ishida, Kaori Fujiwara, Kana Washio, Hirokazu Tsukahara

引用次数: 0

Eosinophils gone missing: The analyser mix-up mystery. 嗜酸性粒细胞失踪：分析仪混淆之谜

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-01-12 DOI: 10.1111/bjh.19977

Radu Chiriac, Fanélie Mestrallet, Cécile Dumas, Sandrine Girard, Marie-Virginie Larcher, Laurent Jallades

引用次数: 0

The higher initial dose and accelerated titration regimen of ropeginterferon as a treatment option for certain patients with polycythaemia vera. 对于真性红细胞增多症的某些患者，高初始剂量和加速滴定方案的ropeg干扰素作为一种治疗选择。