British Journal of Haematology最新文献_第2页

Comparative analysis of mortality patterns and treatment strategies in thalassaemia and sickle cell disease patients: A 12-year study.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-16 DOI: 10.1111/bjh.20043

Sophia Delicou, Konstantinos Manganas, Michael D Diamantidis, Theodora Maria Venou, Polyxeni Delaporta, Despoina Pantelidou, Eirini Spachiou, Sofia Tsagia, Vasiliki Pappi, Foteini Petropoulou, Eleni Kapsali, Loukia Evliati, Konstantina Papaioannou, Marianna Katsatou, Evangelos Klironomos, Artemis Vasiliadi, Angelos Gkoutzouvelidis, Panagiota Giasari, Christos Zisis, Ioannis Lafiatis, Anastasia Goula, Aikaterini Xydaki, Despina Papadopoulou, Christos Chatzoulis, Stylianos Lafioniatis, Dimitra Vini, Anastasia Serpanou, Chrysoula Kalkana, Stavroula Kyriakaki, Maria Drandaki, Alexandra Kouraklis, Antonis Kattamis, Efthymia Vlachaki

{"title":"Comparative analysis of mortality patterns and treatment strategies in thalassaemia and sickle cell disease patients: A 12-year study.","authors":"Sophia Delicou, Konstantinos Manganas, Michael D Diamantidis, Theodora Maria Venou, Polyxeni Delaporta, Despoina Pantelidou, Eirini Spachiou, Sofia Tsagia, Vasiliki Pappi, Foteini Petropoulou, Eleni Kapsali, Loukia Evliati, Konstantina Papaioannou, Marianna Katsatou, Evangelos Klironomos, Artemis Vasiliadi, Angelos Gkoutzouvelidis, Panagiota Giasari, Christos Zisis, Ioannis Lafiatis, Anastasia Goula, Aikaterini Xydaki, Despina Papadopoulou, Christos Chatzoulis, Stylianos Lafioniatis, Dimitra Vini, Anastasia Serpanou, Chrysoula Kalkana, Stavroula Kyriakaki, Maria Drandaki, Alexandra Kouraklis, Antonis Kattamis, Efthymia Vlachaki","doi":"10.1111/bjh.20043","DOIUrl":"https://doi.org/10.1111/bjh.20043","url":null,"abstract":"This study examined mortality rates among 2475 patients with thalassaemia and sickle cell disease (SCD) per year over 12 years in Greece, recording 335 deaths (27.92/year), with an overall mortality rate of 1.13% per year. The primary aim was to identify causes of death, comorbidities, treatment efficacy and iron overload prevalence. Of the deaths, 239 were attributed to thalassaemia and 96 to SCD. For thalassaemia patients, cardiac failure, hepatocellular carcinoma and sepsis were the leading causes of death, with no neoplasms observed in β+/β+ genotypes. In SCD, sepsis, liver failure and stroke were the predominant causes, with sepsis-related deaths higher in frequently transfused patients. The median age of death was significantly lower in thalassaemia (50 years) compared to SCD (58.49 years, p < 0.001). Differences in comorbidities and treatment effectiveness highlight the need for improved management strategies. Addressing iron overload, optimizing chelation therapy and expanding hydroxyurea use in SCD patients could enhance survival and quality of life. Strengthening treatment protocols and monitoring may reduce mortality, emphasizing the importance of targeted interventions in haemoglobinopathies.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143639377","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

BCL2 inhibition for multiple myeloma and AL amyloidosis. 抑制 BCL2 治疗多发性骨髓瘤和 AL 淀粉样变性病。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-16 DOI: 10.1111/bjh.20046

Lorenzo Cani, Vikas A Gupta, Jonathan L Kaufman

引用次数: 0

Monitoring the KMT2A gene post-chemotherapy independently predicts the relapse and survival risk after allogeneic haematopoietic stem cell transplantation.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-13 DOI: 10.1111/bjh.20036

Jing Liu, Xiao-Su Zhao, Ying-Jun Chang, Ya-Zhen Qin, Qian Jiang, Hao Jiang, Xiao-Hui Zhang, Lan-Ping Xu, Yu Wang, Meng Lv, Kai-Yan Liu, Xiao-Jun Huang, Xiang-Yu Zhao

{"title":"Monitoring the KMT2A gene post-chemotherapy independently predicts the relapse and survival risk after allogeneic haematopoietic stem cell transplantation.","authors":"Jing Liu, Xiao-Su Zhao, Ying-Jun Chang, Ya-Zhen Qin, Qian Jiang, Hao Jiang, Xiao-Hui Zhang, Lan-Ping Xu, Yu Wang, Meng Lv, Kai-Yan Liu, Xiao-Jun Huang, Xiang-Yu Zhao","doi":"10.1111/bjh.20036","DOIUrl":"https://doi.org/10.1111/bjh.20036","url":null,"abstract":"This study evaluated the kinetics of KMT2A-r during chemotherapy and its impact on allogeneic haematopoietic stem cell transplantation (allo-HSCT) outcomes. KMT2A-r was assessed post-induction (MRD1), after the first (MRD2) and second (MRD3) consolidations and pre-transplant (MRD4) in 52 patients with acute myeloid leukaemia (AML). KMT2A-r significantly decreased from diagnosis to MRD2 (p < 0.001 for diagnosis vs. MRD1; p = 0.019 for MRD1 vs. MRD2). The incidence of KMT2A-r negativity (57.5%) peaked at MRD2. KMT2A-r status at each time point significantly affected post-transplant outcomes. Cluster analysis identified four KMT2A-r kinetic profiles: persistently negative (-/-), turned negative at transplant (+/-), turned positive at transplant (-/+) and persistently positive (+/+). The (-/-) group had the best outcomes, with a cumulative incidence of relapse (CIR) of 13.0%, overall survival (OS) of 82.0% and leukaemia-free survival (LFS) of 81.7%. The (+/+) group had the worst prognosis, with a CIR of 58.8%, OS of 29.4% and LFS of 23.5%. KMT2A dynamics were an independent risk factor for CIR (Hazard ratio [HR] = 11.070, 95%CI 2.395-51.165, p = 0.002), LFS (HR = 9.316, 95%CI 2.656-32.668, p < 0.001) and OS (HR = 7.172, 95%CI 1.999-25.730, p = 0.003). In conclusion, KMT2A-r status after chemotherapy and its kinetics are significant HSCT prognostic indicators.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143622870","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

Pancytopenia associated with parvovirus B19 infection in an AIDS patient.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-11 DOI: 10.1111/bjh.20015

Luis Veloza, Laurence de Leval

引用次数: 0

One pathology, two morphologies: Plasma cell crystalline inclusions in monoclonal gammopathy of undetermined significance.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-11 DOI: 10.1111/bjh.20017

Radu Chiriac

引用次数: 0

Effects of orelabrutinib, a BTK inhibitor, on antibody-mediated platelet destruction in primary immune thrombocytopenia.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-11 DOI: 10.1111/bjh.20045

Tian-Shu Yu, Shou-Qing Han, Ling-Jun Wang, Hao-Yi Wang, Xiao-Fei Ni, Ru-Ting Wang, Guo-Sheng Li, Yu Hou, Jun Peng, Zhen-Yu Yan, Ya-Jing Zhao, Ming Hou, Xin-Guang Liu

{"title":"Effects of orelabrutinib, a BTK inhibitor, on antibody-mediated platelet destruction in primary immune thrombocytopenia.","authors":"Tian-Shu Yu, Shou-Qing Han, Ling-Jun Wang, Hao-Yi Wang, Xiao-Fei Ni, Ru-Ting Wang, Guo-Sheng Li, Yu Hou, Jun Peng, Zhen-Yu Yan, Ya-Jing Zhao, Ming Hou, Xin-Guang Liu","doi":"10.1111/bjh.20045","DOIUrl":"https://doi.org/10.1111/bjh.20045","url":null,"abstract":"Primary immune thrombocytopenia (ITP) is a haemorrhagic disorder with a complex pathogenesis, wherein autoreactive B-cell-mediated platelet destruction plays a crucial role. Bruton's tyrosine kinase (BTK) is widely expressed and essential for immune cells. Several BTK inhibitors have been used clinically to treat haematological malignancies, while few studies are focusing on the regulatory role of BTK in ITP. This study aims to explore the feasibility and underlying mechanisms of a novel BTK inhibitor orelabrutinib in the treatment of ITP through in vitro and in vivo experiments. Orelabrutinib could inhibit B-cell receptor-mediated B-cell activation, proliferation, differentiation and pro-inflammatory cytokine secretion. Transcriptome sequencing revealed that B cells of ITP patients were more hyper-responsive in inflammation and secretion activity compared to healthy controls, and orelabrutinib might alter B-cell status through downregulating ribosome and mitochondrial metabolism. Fcγ receptor-mediated platelet phagocytosis and pro-inflammatory cytokine production by macrophages were also suppressed by orelabrutinib. Furthermore, orelabrutinib treatment considerably elevated the platelet count in active ITP murine models by inhibiting plasma cell differentiation, anti-platelet antibody production, pro-inflammatory factor secretion and platelet phagocytosis in the livers and spleens. Taken together, orelabrutinib could serve as a potential therapeutic agent for ITP by blocking antibody-mediated platelet destruction.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143603088","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

Indirect presentation of mismatched human leukocyte antigen-B associates with outcomes of cord blood transplantation.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-10 DOI: 10.1111/bjh.20035

Takeshi Sugio, Kohta Miyawaki, Naoyuki Uchida, Matthias Niemann, Eric Spierings, Kyohei Mori, Yuju Ohno, Tetsuya Eto, Yasuo Mori, Goichi Yoshimoto, Yoshikane Kikushige, Yuya Kunisaki, Shinichi Mizuno, Koji Nagafuji, Hiromi Iwasaki, Tomohiko Kamimura, Ryosuke Ogawa, Toshihiro Miyamoto, Shuichi Taniguchi, Koichi Akashi, Koji Kato

{"title":"Indirect presentation of mismatched human leukocyte antigen-B associates with outcomes of cord blood transplantation.","authors":"Takeshi Sugio, Kohta Miyawaki, Naoyuki Uchida, Matthias Niemann, Eric Spierings, Kyohei Mori, Yuju Ohno, Tetsuya Eto, Yasuo Mori, Goichi Yoshimoto, Yoshikane Kikushige, Yuya Kunisaki, Shinichi Mizuno, Koji Nagafuji, Hiromi Iwasaki, Tomohiko Kamimura, Ryosuke Ogawa, Toshihiro Miyamoto, Shuichi Taniguchi, Koichi Akashi, Koji Kato","doi":"10.1111/bjh.20035","DOIUrl":"https://doi.org/10.1111/bjh.20035","url":null,"abstract":"Cord blood transplantation (CBT) is a valuable donor source for patients without human leukocyte antigen (HLA)-matched donors. While CBT has a lower risk of graft-versus-host disease and requires less stringent histocompatibility, it is associated with a higher transplantation-related mortality (TRM) compared to other donor sources. We hypothesized that assessing the immunogenicity of mismatched HLA could reveal non-permissive mismatches contributing to increased TRM. We retrospectively analysed 1498 single-unit CBT cases from 2000 to 2018 across eight Japanese institutions, evaluating the immunogenicity of mismatched HLA using the PIRCHE algorithm to examine binding affinities of HLA-derived epitopes to donor or recipient HLA. Results indicated that Class I epitopes from mismatched recipient HLA-B were significantly associated with poor outcomes due to higher TRM and lower neutrophil engraftment, particularly when presented on matched HLA class I. Notably, epitopes from HLA-B exon 1 showed stronger prognostic significance, with HLA-B alleles carrying M-type leader peptides exhibiting higher affinity for these epitopes. Patients with a matched M-type HLA-B and Class I epitopes derived from mismatched HLA-B exon 1 had worse outcomes. These findings suggest that immunogenicity-informed donor selection could improve CBT outcomes.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143595864","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

Therapeutic drug monitoring of imatinib in paediatric chronic myeloid leukaemia: Data from a real-world setting.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-10 DOI: 10.1111/bjh.20047

Meinolf Suttorp, Stephanie Sembill, Phyllis Lensker, Verena Hildebrand, Elke Schirmer, Axel Karow, Manuela Krumbholz, Manfred Rauh, Markus Metzler

{"title":"Therapeutic drug monitoring of imatinib in paediatric chronic myeloid leukaemia: Data from a real-world setting.","authors":"Meinolf Suttorp, Stephanie Sembill, Phyllis Lensker, Verena Hildebrand, Elke Schirmer, Axel Karow, Manuela Krumbholz, Manfred Rauh, Markus Metzler","doi":"10.1111/bjh.20047","DOIUrl":"https://doi.org/10.1111/bjh.20047","url":null,"abstract":"Imatinib (IMA) therapy for paediatric chronic myeloid leukemia (pCML) requires age-dependent dose adjustments. Assessment of therapeutic drug monitoring (TDM) under 'real-world' conditions was performed. Collection of blood and TDM-relevant data, calculation of individual dosage exposure, measurement of plasma Cmin (IMA, Nor-IMA) by HPLC/MS-MS and recording of adverse event (AE). Two hundred and forty-six specimens from 66 patients were analysed. Individual median IMA dosage exposure was 253 mg/m2 (range: 128-504). Children <13 years received a median of 43 mg/m2 more than older patients (p < 0.0001). Median Cmin of IMA and Nor-IMA was 1017 ng/mL (range: 51-3976) and 269 ng/mL (range: 21-981), respectively, correlating significantly with the prescribed dose. At 5/246 visits, non-adherence was confirmed by very low IMA Cmin in 3/66 patients, all ≥13 years old. Correlation of IMA Cmin >1000 ng/mL with achieving OMR demonstrated in each 63% (N = 24/37, N = 27/43, respectively) patients at months 3 and 6. In the cohort with lower levels, only 23% and 50%, respectively, achieved these milestones. This difference was significant only at month 3. Of 66 patients, 30 reported 125 AEs with gastrointestinal and musculoskeletal as leading complaints. In 9.3% of AEs, the correlated IMA Cmin was ≥3000 ng/mL. TDM is a simple and rapid additional tool for managing pCML under 'real-world conditions'.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143584007","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

Comprehensive clinico-genetic analysis reveals prognostic factors of blastic plasmacytoid dendritic cell neoplasm.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-10 DOI: 10.1111/bjh.20022

Ji Hyun Lee, Sung-Yong Oh, Taek Gyu Lee, Seung-Tae Lee, Namhee Kim, Min Gyoung Pak, Sung-Soo Yoon, Youngil Koh, Ja Min Byun, Dok Hyun Yoon, Jae-Cheol Jo, Deok-Hwan Yang, Seo-Yeon Ahn, Hyeon Seok Eom, Hyewon Lee, Ji Yun Lee, Jong Ho Won, Ho-Young Yhim, Ho Sup Lee, Won Seog Kim, Gyeong-Sin Park, Saeam Shin, Seok Jin Kim

引用次数: 0

A pilot study tracking immune cell subsets and activation profiles in adults with haemophagocytic lymphohistiocytosis treated with anakinra.

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-03-09 DOI: 10.1111/bjh.20034

Mark J Bishton, Emma Hadfield, David Onion

引用次数: 0