{"title":"The progression of multiple myeloma is regulated by LILRB1 via the GATA2-SAGE1 pathway.","authors":"Chiqi Chen, Xuanyi Zhou, Liyuan Cao, Wenqian Yang, Lietao Weng, Jin Yuan, Wen Zhou, Zhuo Yu, Junke Zheng","doi":"10.1111/bjh.20144","DOIUrl":"https://doi.org/10.1111/bjh.20144","url":null,"abstract":"<p><p>Multiple myeloma (MM) is a haematological malignancy characterized by the clonal expansion of plasma cells within the bone marrow, thus resulting in the overproduction of monoclonal immunoglobulins. Despite the availability of various immunotherapeutic strategies, patient survival rates remain disappointingly low, thus underscoring the need for innovative immunotherapies to improve outcomes. Leukocyte Ig-like receptor subfamily B (LILRB1), which is a recently identified immune checkpoint, has an undefined role and molecular mechanism in MM. Herein, we demonstrated that LILRB1 was significantly upregulated in MM patients and MM cell lines and was negatively correlated with patient survival. The knockdown of LILRB1 promoted apoptosis in MM cells, enhanced sensitivity to bortezomib and diminished tumourigenicity in a subcutaneous mouse model. Mechanistically, LILRB1 triggers downstream GATA Binding Protein 2 (GATA2) and sustains MM cell proliferation via the GATA2-Sarcoma Antigen 1 (SAGE1) signalling pathway. Consequently, the targeting of LILRB1 may represent a promising therapeutic approach for MM.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144141224","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}
{"title":"Robust CAR T-cell expansion and superior outcomes in DLBCL patients in complete response at infusion.","authors":"Ryo Hanajiri, Hiroya Wakabayashi, Kohei Ishigiwa, Fumiya Ohara, Shiho Hirano, Hirofumi Yokota, Shihomi Kuwano, Katsuya Furukawa, Kazuyuki Shimada, Takahiko Sato, Seitaro Terakura, Hitoshi Kiyoi","doi":"10.1111/bjh.20186","DOIUrl":"https://doi.org/10.1111/bjh.20186","url":null,"abstract":"<p><p>Historically, the presence of measurable disease has been considered essential to stimulate CAR T-cell expansion and persistence. However, the kinetics of CAR T cells in patients achieving complete response (CR) before infusion remain poorly understood. This study aimed to evaluate the outcomes and CAR T-cell kinetics in relapsed/refractory diffuse large B-cell lymphoma (DLBCL) patients stratified by pre-infusion disease status. In this retrospective analysis of 87 patients treated at a single institution, 23 (26.4%) were in CR and 64 (73.6%) were in non-CR prior to CAR T-cell infusion. Patients in CR exhibited significantly better progression-free survival (PFS) and overall survival compared to non-CR patients. Peripheral blood CAR T-cell kinetics, including the proportion and absolute counts of CAR T cells, CD4+ CAR T cells and CD8+ CART cells, showed no significant differences between CR and non-CR groups. These findings were consistent across different CAR T-cell products, whether 4-1BB- or CD28-based. Moreover, an analysis of patients achieving complete metabolic response (CMR) by PET-CT confirmed comparable CAR T-cell expansion and persistence in both CMR and non-CMR patients. Our findings demonstrate that CAR T-cell therapy achieves robust expansion and favourable survival outcomes in CR patients, even in the absence of measurable disease.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144141186","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}
Ke Xu, Carolyn Maharaj, Eleanor Kaffo, Evan Vitsaras, Karen Orfinada, Robert Baker, Elisabeth Nacheva, Andrew Wilson, Jenny O'Nions, Rajeev Gupta
{"title":"Diagnosis and treatment of early T-cell precursor acute lymphoblastic leukaemia.","authors":"Ke Xu, Carolyn Maharaj, Eleanor Kaffo, Evan Vitsaras, Karen Orfinada, Robert Baker, Elisabeth Nacheva, Andrew Wilson, Jenny O'Nions, Rajeev Gupta","doi":"10.1111/bjh.20163","DOIUrl":"https://doi.org/10.1111/bjh.20163","url":null,"abstract":"","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144126320","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}
{"title":"RETRACTION: IPO-trimethylation of Histone H3-lysine<sup>9</sup> Associated with P210 BCR-ABL Tyrosine Kinase of Chronic Myeloid Leukaemia.","authors":"","doi":"10.1111/bjh.20189","DOIUrl":"https://doi.org/10.1111/bjh.20189","url":null,"abstract":"<p><strong>Retraction: </strong>M. Mancini, E. Zuffa, N. Veljkovic, G. Brusa, P. Corrado, V. Corradi, G. Martinelli, E. Barbieri, and M. A. Santucci, \"IPO-trimethylation of Histone H3-lysine<sup>9</sup> Associated with P210 BCR-ABL Tyrosine Kinase of Chronic Myeloid Leukaemia,\" British Journal of Haematology 141, no. 6 (2006): 899-902, https://doi.org/10.1111/j.1365-2141.2008.07134.x. The above article, published online on 22 April 2008 in Wiley Online Library (wileyonlinelibrary.com), has been retracted by agreement between the journal Editor-in-Chief Andrew Evans; the British Society for Haematology; and John Wiley & Sons Ltd. A third party notified the journal that they had found evidence of image manipulation and duplication within Figure 2. The third party also reported that bands in Figure 2 of this article had been reused from 2 other articles by some of the same authors (Mancini et al. 2006 [https://doi.org/10.1111/j.1365-2141.2005.05873.x] and Mancini et al. 2007 [https://doi.org/10.1016/j.leukres.2006.09.022]). An investigation by the publisher confirmed that some cytoplasm bands in Figure 1 had been duplicated, that some HIST1H1 bands in Figure 2A had been duplicated, and that the HIST4H4 panel in Figure 2B had been inserted. The investigation also confirmed that bands had been reused from other articles by the same authors, each of which describes different experimental conditions. Authors M. Mancini and N. Calonghi responded to an inquiry by the publisher, but due to the length of time since publication they were not able to provide an explanation for the evidence of image manipulation or the original data. The authors did not respond to our notice regarding the retraction.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144126322","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}
{"title":"Screening for monoclonal gammopathy of undetermined significance is contraindicated.","authors":"Gurmukh Singh","doi":"10.1111/bjh.20175","DOIUrl":"https://doi.org/10.1111/bjh.20175","url":null,"abstract":"<p><p>Incidence of multiple myeloma is about 1.8/100 000. About 3%-5% of adults >50 years of age are diagnosed with monoclonal gammopathy of undetermined significance (MGUS). The prevalence is higher if diagnosis is based on an abnormal ratio of serum free light chains. 300-500 people/10 000 get labelled with a diagnosis of this pre-malignant disorder by using faulty criterion of abnormal light chain ratio. Diagnoses based on an abnormal light chain ratio are incorrect in 96% of instances. However, a large number of people are subjected to psychological trauma and financial toxicity by subjecting them to repeated and invasive testing. If strict criteria requiring the presence of a monoclonal immunoglobulin were used, only about 10 people/10 000 may have MGUS and only one person may progress to multiple myeloma in 10 years. Even that one person gains no benefit from an 'early' diagnosis. Thus, there is only harm and no benefit from screening for MGUS.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144118427","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}
Talha Badar, James M Foran, Jan Philipp Bewersdorf, Yu-Hung Wang, Alexander Coltoff, Mobachir El Kettani, Kashish Shah, Francyess Denis Oliva, Chenyu Lin, Omer Jamy, Kendall Diebold, Rory M Shallis, Alexa Siddon, Daniil Katkov, Alexa Schoen, Irum Khan, Charles E Foucar, Ehab Atallah, Aaron D Goldberg, Anand A Patel
{"title":"Heterogeneity in outcomes of TP53-mutated myeloproliferative neoplasms based on disease phenotype and mutational status.","authors":"Talha Badar, James M Foran, Jan Philipp Bewersdorf, Yu-Hung Wang, Alexander Coltoff, Mobachir El Kettani, Kashish Shah, Francyess Denis Oliva, Chenyu Lin, Omer Jamy, Kendall Diebold, Rory M Shallis, Alexa Siddon, Daniil Katkov, Alexa Schoen, Irum Khan, Charles E Foucar, Ehab Atallah, Aaron D Goldberg, Anand A Patel","doi":"10.1111/bjh.20187","DOIUrl":"https://doi.org/10.1111/bjh.20187","url":null,"abstract":"","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144118426","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}
Yiling Ye, Xinyu Liu, Huajuan Dai, Jing Hu, Guopan Yu, Yu Zhang, Guangyang Weng, Dongjun Lin, Xin Du, Jie Xiao, Zhiqiang Sun, Hongyu Zhang, Xinquan Liang, Ziwen Guo, Na Xu, Zhiping Fan, Li Xuan, Ren Lin, Zhao Yin, Fen Huang, Min Dai, Zhuan Li, Qifa Liu, Hua Jin
{"title":"Venetoclax plus azacitidine with or without homoharringtonine followed by allogeneic haematopoietic cell transplantation in patients with relapsed/refractory acute myeloid leukaemia: A multicentre cohort study.","authors":"Yiling Ye, Xinyu Liu, Huajuan Dai, Jing Hu, Guopan Yu, Yu Zhang, Guangyang Weng, Dongjun Lin, Xin Du, Jie Xiao, Zhiqiang Sun, Hongyu Zhang, Xinquan Liang, Ziwen Guo, Na Xu, Zhiping Fan, Li Xuan, Ren Lin, Zhao Yin, Fen Huang, Min Dai, Zhuan Li, Qifa Liu, Hua Jin","doi":"10.1111/bjh.20147","DOIUrl":"https://doi.org/10.1111/bjh.20147","url":null,"abstract":"<p><p>The appropriate salvage regimen followed by allogeneic haematopoietic stem cell transplantation (allo-HSCT) for relapsed/refractory (R/R) acute myeloid leukaemia (AML) patients remains unclear. Three hundred and fifty R/R AML patients receiving venetoclax and azacitidine (VA) regimen or VA plus homoharringtonine (VAH) regimen as salvage therapy were enrolled in this study, with a higher composite complete remission rate in the VAH group (69.9%) than in the VA group (46.1%). A total of 105 patients underwent allo-HSCT, with a median follow-up post-transplantation of 37.2 months. The 3-year cumulative incidence of transplant-related mortality was 18.2% in the VA group and 9.8% in the VAH group. The 3-year cumulative incidence of relapse was lower in the VAH group (19.7%) than in the VA group (43.2%). The 3-year overall survival and event-free survival (EFS) were 82.0% and 70.5%, respectively, in the VAH group, which were higher than 59.1% and 38.6%, respectively, in the VA group. Multivariate analysis revealed the VAH regimen and MRD-negative at transplantation were protective factors for relapse (HR = 0.427 and HR = 0.368) and EFS (HR = 0.469 and HR = 0.384). In conclusion, the VAH regimen is an effective and safe salvage therapy bridge to allo-HSCT for R/R AML patients compared with the VA regimen.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144118429","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}
Anna Ogneva, Yulia Rodina, Dmitry Pershin, Kirill Voronin, Anastasia Prozvetkina, Alexey Maschan, Galina Novichkova, Nikolay Grachev, Anna Shcherbina
{"title":"Efficacy and safety of romiplostim and eltrombopag in management of thrombocytopenia in Wiskott-Aldrich syndrome patients.","authors":"Anna Ogneva, Yulia Rodina, Dmitry Pershin, Kirill Voronin, Anastasia Prozvetkina, Alexey Maschan, Galina Novichkova, Nikolay Grachev, Anna Shcherbina","doi":"10.1111/bjh.20182","DOIUrl":"https://doi.org/10.1111/bjh.20182","url":null,"abstract":"<p><p>Wiskott-Aldrich syndrome (WAS) is a life-threatening inborn error of immunity associated with bleeding risk due to profound thrombocytopenia. We previously demonstrated that romiplostim is effective for thrombocytopenia treatment in 60% of WAS patients. In this prospective trial, 28 WAS patients (aged 0-17 years) were treated with thrombopoietin receptor agonists (TPO-RAs) to evaluate the comparative efficacy and safety of romiplostim versus eltrombopag and to assess the benefit of switching these molecules in individual subjects. We demonstrate that both drugs are effective, with the probability of achieving an overall platelet response (complete response-platelet count of 100 × 10<sup>9</sup>/L or more and partial response-platelet count increase of 30 × 10<sup>9</sup>/L or higher above baseline) of 73% for romiplostim and 43% for eltrombopag. Switching to an alternative TPO-RA allowed the achievement of a complete platelet response in 33% of patients after switching to eltrombopag and in 43% after romiplostim initiation. The cumulative efficacy of TPO-RAs, taking into account alternative therapy, was 87%. There was a grade 2 adverse event (AE) and a severe grade 4 AE related to eltrombopag-reversible acute liver failure associated with metabolic acidosis and encephalopathy. TPO-RAs proved to be an effective treatment for WAS patients waiting for stem cell transplantation or opting for a conservative treatment.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144118424","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}
E Laspa, D Neofytos, D Boutboul, F Giannotti, S Masouridi-Levrat, F Simonetta, C Royer-Chardon, L de Leval, S Blum, Y Chalandon, A C Mamez
{"title":"Kaposi sarcoma and Kaposi inflammatory cytokine syndrome following allogeneic haematopoietic stem cell transplantation: A case report.","authors":"E Laspa, D Neofytos, D Boutboul, F Giannotti, S Masouridi-Levrat, F Simonetta, C Royer-Chardon, L de Leval, S Blum, Y Chalandon, A C Mamez","doi":"10.1111/bjh.20158","DOIUrl":"https://doi.org/10.1111/bjh.20158","url":null,"abstract":"","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144109053","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}
{"title":"Correction to \"Abstracts of the 65th Annual Scientific Meeting of the British Society for Haematology\".","authors":"","doi":"10.1111/bjh.20161","DOIUrl":"https://doi.org/10.1111/bjh.20161","url":null,"abstract":"","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144101197","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}