British Journal of Haematology最新文献_第8页

Early fatal tumour lysis syndrome. 早期致命肿瘤溶解综合征。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-29 DOI: 10.1111/bjh.20113

Healson Ihuoma, Mark Williams, Samar Kulkarni, Richard Chasty

引用次数: 0

Blastic erythrophagocytosis in monocytic AML with translocation t(8;22) 单核细胞AML伴t易位的母细胞吞噬（8;22）

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-29 DOI: 10.1111/bjh.20094

Lisa Faucheux, Emeline Voirin, Emmanuelle Rault, Olivier Herault

{"title":"Blastic erythrophagocytosis in monocytic AML with translocation t(8;22)","authors":"Lisa Faucheux, Emeline Voirin, Emmanuelle Rault, Olivier Herault","doi":"10.1111/bjh.20094","DOIUrl":"https://doi.org/10.1111/bjh.20094","url":null,"abstract":"A 72-year-old man was referred with anaemia and thrombocytopenia without peripheral blasts. The haemoglobin level was 88 g/L; platelet count, 23 × 109/L; and leucocyte count, 2.5 × 109/L including 1.4 × 109/L neutrophils. A bone marrow (BM) spread showed signs of dysplasia (karyorrhexis and hypogranularity). BM was infiltrated by 75% of CD4+CD11b+CD14+ large monocytic blasts exhibiting erythrophagocytosis (upper row, morphology images, magnification: objective ×100, May-Grünwald Giemsa stain). High-throughput sequencing identified a complex mutational profile in which pathogenic variants were TET2 c.4824T>G (variant allele frequency [VAF] 55%), TET2 c.2370del (VAF 50%), CDKN2A c.330G>A (VAF 18%), TP53 c.742C>T (VAF 16%), ASXL1 c.3117del (VAF 6%) and RUNX1 c.556C>T (VAF 5%).BM cytogenetic analysis revealed a complex karyotype (six abnormalities) including t(8;22)(p11;q13) (middle row, karyotype, magnification: objective ×63, RHG-banding). The t(8;22) led to a rearrangement between KAT6A (alias MOZ) and EP300 genes mapped on 8p11.21 and 22q13.2 respectively (lower row, dual fusion probe, Sysmex, Kobe, Japan).The t(8;22)(p11;q13), which results in a chimeric MOZ-p300 protein, is a variant of the t(8;16)(p11;p13) where MOZ is fused to CBP. This is the fourth case of MOZ::EP300 rearrangement reported in acute myelomonocytic/monocytic leukaemia, associated with erythrophagocytosis and yielding a poor prognosis, yet liable to targeted therapy.This study was supported by the French Association AHB ‘Association d'hématologie biologique de Tours’ (# W372006363).The patient provided informed consent for the use of the analyses, and the data were anonymized to ensure they could not be identified.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":"206 5","pages":"1270-1271"},"PeriodicalIF":5.1,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/bjh.20094","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143950238","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Underlying disease is the main risk factor in post-splenectomy complication risk: Data from a national database. 基础疾病是脾切除术后并发症的主要危险因素：来自国家数据库的数据。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-29 DOI: 10.1111/bjh.20114

Maddalena Casale, Raffaella Colombatti, Manuela Balocco, Paola Corti, Susanna Barella, Giovanna Graziadei, Loredana Farinasso, Tommaso Mina, Simone Cesaro, Tommaso Casini, Fiorina Giona, Saverio Ladogana, Pellegrina Pugliese, Lucia Dora Notarangelo, Antonella Sau, Simone Ferrero, Giovanni Palazzi, Giovanna Russo, Ilaria Lazzareschi, Marilena Serra, Saveria Campisi, Gianluca Boscarol, Elena Facchini, Carlo Baronci, Maria Caterina Putti, Domenico Roberti, Marzia Manilia, Antonio Ivan Lazzarino, Gian Luca Forni, Silverio Perrotta

{"title":"Underlying disease is the main risk factor in post-splenectomy complication risk: Data from a national database.","authors":"Maddalena Casale, Raffaella Colombatti, Manuela Balocco, Paola Corti, Susanna Barella, Giovanna Graziadei, Loredana Farinasso, Tommaso Mina, Simone Cesaro, Tommaso Casini, Fiorina Giona, Saverio Ladogana, Pellegrina Pugliese, Lucia Dora Notarangelo, Antonella Sau, Simone Ferrero, Giovanni Palazzi, Giovanna Russo, Ilaria Lazzareschi, Marilena Serra, Saveria Campisi, Gianluca Boscarol, Elena Facchini, Carlo Baronci, Maria Caterina Putti, Domenico Roberti, Marzia Manilia, Antonio Ivan Lazzarino, Gian Luca Forni, Silverio Perrotta","doi":"10.1111/bjh.20114","DOIUrl":"https://doi.org/10.1111/bjh.20114","url":null,"abstract":"Splenectomy is required for many haematological conditions and causes an increased risk of severe infections and vascular events. The association between underlying haematological disease, age at splenectomy and post-splenectomy complications was explored among 1348 splenectomized patients, followed with a median follow-up time of 13 years and affected by transfusion-dependent thalassaemia, non-transfusion-dependent thalassaemia (NTDT), sickle cell anaemia (SCA), congenital haemolytic anaemias, autoimmune haematological disorders and trauma. Our main statistical approach was based on interaction analyses within competing-risk survival models. The baseline risk profile differed across diagnostic categories, with SCA being particularly susceptible to infectious complications and NTDT and SCA to vascular events (p < 0.001). The age at splenectomy did not impact on infectious risk but rather older age at splenectomy was associated with increased risk for vascular complications. Furthermore, the risk of developing a post-splenectomy complication was persistent throughout the observation period and not limited to the first 2-3 years after splenectomy. The probability of a post-splenectomy complication was highly dependent on the underlying disease and not on the age at splenectomy, so the indications for splenectomy must be based on careful assessment of pros and cons in the individual disease, with no need to delay surgery after a certain age when clinically indicated.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143951618","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

Expect the unexpected: Tangier disease and monoclonal gammopathy of undetermined significance. 意料之外：丹吉尔病和单克隆γ病意义不明。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-29 DOI: 10.1111/bjh.20096

Radu Chiriac

引用次数: 0

The role of red blood cell characteristics and viscosity in sickle cell retinopathy and maculopathy. 红细胞特性和黏度在镰状细胞性视网膜病变和黄斑病变中的作用。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-29 DOI: 10.1111/bjh.20124

Rajani P Brandsen, Roselie M H Diederen, Ingeborg Klaassen, Martijn Veldthuis, Herbert Korsten, Rob van Zwieten, Reinier O Schlingemann, Erfan Nur, Bart J Biemond

{"title":"The role of red blood cell characteristics and viscosity in sickle cell retinopathy and maculopathy.","authors":"Rajani P Brandsen, Roselie M H Diederen, Ingeborg Klaassen, Martijn Veldthuis, Herbert Korsten, Rob van Zwieten, Reinier O Schlingemann, Erfan Nur, Bart J Biemond","doi":"10.1111/bjh.20124","DOIUrl":"https://doi.org/10.1111/bjh.20124","url":null,"abstract":"Sickle cell disease (SCD), encompassing genotypes such as HbSS and HbSC, causes chronic haemolysis and microvascular occlusion, leading to organ damage. The retina is particularly vulnerable, often resulting in sickle cell retinopathy (SCR) or sickle cell maculopathy (SCM). The precise underlying mechanisms are unclear, though various factors are suggested to contribute. This study explored the role of whole blood viscosity and red blood cell (RBC) deformability in SCR and SCM. Adult HbSS (n = 34) and HbSC patients (n = 34) were offered an ophthalmic examination to determine SCR stage. A venous ethylenediaminetetraacetic acid (EDTA) sample was collected from each participant. Whole blood viscosity was measured using a Brookfield viscometer and RBC deformability was assessed using the Oxygenscan feature of the Laser Optical Rotational Red Cell Analyser as a function of the (varying) partial oxygen pressure. HbSC patients with proliferative sickle cell retinopathy (PSCR) had a lower delta elongation index (p = 0.012) and point of sickling (p = 0.002) than those without PSCR, suggesting that RBC sickling might not play a central role in the pathogenesis of PSCR in HbSC patients. Despite hyperviscosity being a commonly proposed mechanism, no associations were found between blood viscosity, SCR and SCM. These results point to alternative mechanisms contributing to SCR and SCM, highlighting the complexity and need for further research to fully understand the underlying factors.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143951916","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

Anti-PF4 mediated thrombocytopenia and thrombosis associated with acute cytomegalovirus infection displays both HIT-like and VITT-like characteristics. 与急性巨细胞病毒感染相关的抗pf4介导的血小板减少和血栓形成具有hit样和vitt样特征。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-29 DOI: 10.1111/bjh.20092

Phillip L R Nicolson, Samantha J Montague, Richard J Buka, Anthony Calvert, Jo-Ann I Sheppard, Yi Zhang, Jing Jing Wang, Jack Sharman, Eman Hassan, James Harrison, Errin Lawrence, Phillip El-Dalil, Dhruv Parekh, Husam Osman, Tom P Gordon, Ishac Nazy, Theodore E Warkentin, Will A Lester

引用次数: 0

Charcot-Leyden crystals in myeloproliferative neoplasm with ETV6-ABL1 and t(7;8). Charcot-Leyden晶体在骨髓增生性肿瘤中与ETV6-ABL1和t的关系（7;8）。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-29 DOI: 10.1111/bjh.20098

Wenying Wu, Mi Jiang

引用次数: 0

PF4-associated immune thrombocytopenia and thrombosis (PITT)-More than heparin and vaccines. pf4相关的免疫性血小板减少和血栓形成(PITT)-超过肝素和疫苗。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-28 DOI: 10.1111/bjh.20125

Michael Makris

引用次数: 0

Atezolizumab, obinutuzumab and venetoclax for the treatment of patients with relapsed/refractory B non-Hodgkin lymphoma: Final analysis of a phase II trial from the LYSA group. Atezolizumab， obinutuzumab和venetoclax用于治疗复发/难治性B型非霍奇金淋巴瘤患者：来自LYSA组的II期试验的最终分析

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-26 DOI: 10.1111/bjh.20109

Charles Herbaux, Emmanuel Bachy, Reda Bouabdallah, Stéphanie Guidez, Olivier Casasnovas, Pierre Feugier, Gandhi Damaj, Hervé Tilly, Loïc Ysebaert, Steven Le Gouill, Nadine Morineau, Emmanuel Gyan, Rémy Gressin, Roch Houot, Morgane Cheminant, Franck Morschhauser, Catherine Thieblemont, Corinne Haioun, Emmanuelle Nicolas-Virelizier, Luc-Matthieu Fornecker, Nicolas Daguindau, Guillaume Cartron

{"title":"Atezolizumab, obinutuzumab and venetoclax for the treatment of patients with relapsed/refractory B non-Hodgkin lymphoma: Final analysis of a phase II trial from the LYSA group.","authors":"Charles Herbaux, Emmanuel Bachy, Reda Bouabdallah, Stéphanie Guidez, Olivier Casasnovas, Pierre Feugier, Gandhi Damaj, Hervé Tilly, Loïc Ysebaert, Steven Le Gouill, Nadine Morineau, Emmanuel Gyan, Rémy Gressin, Roch Houot, Morgane Cheminant, Franck Morschhauser, Catherine Thieblemont, Corinne Haioun, Emmanuelle Nicolas-Virelizier, Luc-Matthieu Fornecker, Nicolas Daguindau, Guillaume Cartron","doi":"10.1111/bjh.20109","DOIUrl":"https://doi.org/10.1111/bjh.20109","url":null,"abstract":"Developing new therapeutic regimens for relapsed/refractory (R/R) B non-Hodgkin lymphoma (NHL) patients remains a significant unmet clinical need. Our objective was to evaluate atezolizumab (ATE), obinutuzumab (OBI) and venetoclax (VEN) combination in patients with R/R NHL who had received at least one prior anti-CD20-containing immunochemotherapy regimen. We report here the final analysis of the phase II LYSA-promoted multicentre trial (NCT03276468) of this combination in follicular lymphoma (FL, n = 58), diffuse large B-cell lymphoma (DLBCL, n = 58) and marginal zone lymphoma (MZL, n = 20). The primary end-point for FL and DLBCL was not met: ATE, OBI, and VEN resulted in an overall response rate (ORR) at the end of induction (EOI) of 53.6% for FL (cohort 1) and 23.6% for DLBCL (cohort 2) when a minimum of 70% and 48% was expected respectively. The median progression-free survival was 11.0 and 2.7 months in cohorts 1 and 2 respectively. In cohort 3 (MZL), the ORR at the EOI was evaluated at 66.7%. The most frequent adverse events (AEs) were cytopenias. We also observed 7.4% of autoimmune AE imputable to the combination. In this phase II study, ATE, OBI and VEN demonstrated moderate efficacy and a manageable toxicity profile when used as induction and maintenance therapy.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143953841","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

Fetal-neonatal alloimmune thrombocytopenia: Mothers are affected too. 胎儿-新生儿同种免疫性血小板减少症：母亲也会受到影响。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-04-25 DOI: 10.1111/bjh.20112

Eleanore A McFarland, Emilie L Vander Haar, Margaret H McKelvy, Katherine A Knightly, Stephanie V Volpe, Thea D Palmer, Stacy Corke, James B Bussel

{"title":"Fetal-neonatal alloimmune thrombocytopenia: Mothers are affected too.","authors":"Eleanore A McFarland, Emilie L Vander Haar, Margaret H McKelvy, Katherine A Knightly, Stephanie V Volpe, Thea D Palmer, Stacy Corke, James B Bussel","doi":"10.1111/bjh.20112","DOIUrl":"https://doi.org/10.1111/bjh.20112","url":null,"abstract":"Fetal-neonatal alloimmune thrombocytopenia (FNAIT) results from parental platelet antigen incompatibility, with human platelet antigen (HPA)-1a the most common. Human leukocyte antigen (HLA)-DRB3*01:01 is strongly associated with the development of high titre anti-HPA-1a antibodies and B8DR3 with autoimmunity. Investigation of FNAIT has focused on fetal-neonatal haemorrhagic, placental and neurodevelopmental effects but has not included mothers. Symptomatic complaints pointing to autoimmunity from FNAIT-affected mothers along with HLA overlap for FNAIT/autoimmunity (HLA-DRB3*01:01, B8DR3) prompted exploration of autoimmune disorders. A de-identified survey sent to NAITbabies mothers included medical review of systems with additional questions on autoimmunity. The frequency of autoimmunity in FNAIT mothers plus its fetal impact was explored. 125/232 (54%) of FNAIT mothers reported psychological problems >6 weeks, with 90% reporting 'anxiety' and >50% reporting each of stress, depression and sleep disturbances. Symptoms were more frequent in mothers whose child did not survive their intracranial hemorrhage (ICH) and those with multiple FNAIT-affected children. The next most common medical symptoms were gastrointestinal and musculoskeletal with 30% frequency. Autoimmunity was mildly increased in mothers with FNAIT, for example, thyroiditis and three other B8DR3-related diseases. Autoimmunity did not worsen neonatal ICH incidence or birthweight. By exploring affected mothers, this study expands the spectrum of diseases associated with FNAIT to include maternal autoimmunity and especially psychological symptoms.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143960710","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