British Journal of Haematology最新文献_第3页

Correction to "Abstracts of the 65th Annual Scientific Meeting of the British Society for Haematology". 更正“英国血液学学会第65届年度科学会议摘要”。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-20 DOI: 10.1111/bjh.20161

引用次数: 0

Venetoclax-based non-intensive combinations for relapsed/refractory acute myeloid leukaemia-Real-world data from a UK-wide programme. 基于venetoclax的非强化联合治疗复发/难治性急性髓性白血病——来自英国范围内项目的真实数据

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-20 DOI: 10.1111/bjh.20149

Henry Wood, Jenny O'Nions, Wei Yee Chan, Albert Borg, Scott Veitch, Patrick Elder, Manish Jain, James Aries, Steven Leak, Emily Mitchell, Jiri Pavlu, Jad Othman, Richard Dillon, Priyanka Mehta, Vidhya Murthy, Steven Knapper, Victoria Potter, Mhairi Copland, Charles Craddock, Pramila Krishnamurthy

引用次数: 0

Combination of fixed low-dose nivolumab and bendamustine in children with high-risk relapsed/refractory classical Hodgkin lymphoma. 固定低剂量纳武单抗联合苯达莫司汀治疗高危复发/难治性经典霍奇金淋巴瘤儿童。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-20 DOI: 10.1111/bjh.20148

Shyam Srinivasan, Aditya Narayan, Chetan Dhamne, Akanksha Chichra, Poonam Khemani, Nidhi Dhariwal, Raghwesh Ranjan, Sneha Shah, Tanuja Shet, Venkata Rama Mohan Gollamudi, Nirmalya Roy Moulik, Shripad D Banavali, Gaurav Narula

引用次数: 0

Long-term efficacy and safety of mitapivat in non-transfusion-dependent α- or β-thalassaemia: An open-label phase 2 study. 米他伐在非输血依赖性α-或β-地中海贫血中的长期疗效和安全性：一项开放标签的2期研究

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-20 DOI: 10.1111/bjh.20058

Kevin H M Kuo, D Mark Layton, Ashutosh Lal, Elliott P Vichinsky, Jayme L Dahlin, Shihao Shen, Gavrielle M Price, Keely S Gilroy, Jeremie H Estepp, Hanny Al-Samkari

{"title":"Long-term efficacy and safety of mitapivat in non-transfusion-dependent α- or β-thalassaemia: An open-label phase 2 study.","authors":"Kevin H M Kuo, D Mark Layton, Ashutosh Lal, Elliott P Vichinsky, Jayme L Dahlin, Shihao Shen, Gavrielle M Price, Keely S Gilroy, Jeremie H Estepp, Hanny Al-Samkari","doi":"10.1111/bjh.20058","DOIUrl":"https://doi.org/10.1111/bjh.20058","url":null,"abstract":"Non-transfusion-dependent thalassaemia (NTDT) can result in serious complications and comorbidities that can impact patients' quality of life. Mitapivat, a first-in-class, oral, small-molecule allosteric activator of red blood cell pyruvate kinase, is under investigation in adults with thalassaemia. Through its mechanism of action, mitapivat increases adenosine triphosphate, leading to improvements in red blood cell health, ineffective erythropoiesis and haemolysis. An open-label, multicentre, phase 2 study (NCT03692052) is evaluating mitapivat 100 mg twice daily in adults with NTDT. We previously reported a statistically significant haemoglobin response (a ≥1.0 g/dL increase in haemoglobin concentration from baseline at ≥1 assessments between Weeks 4 and 12 [inclusive]) during the 24-week core period. Here, we report efficacy and safety results up to Week 156 and to data cut-off date respectively. Of 20 patients enrolled, 17 continued in the extension period. Median change from baseline in haemoglobin concentration at Week 156 was 1.2 g/dL. Patients receiving mitapivat demonstrated sustained improvements in haemoglobin concentrations and markers of erythropoietic activity, haemolysis and iron homeostasis. Five patients (29%) had a grade ≥3 treatment-emergent adverse event; none were considered treatment related. Treatment with mitapivat was well tolerated, with a safety profile consistent with previous studies of mitapivat in pyruvate kinase deficiency.","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":"144109056","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

Management of relapsed or refractory large B-cell lymphoma: A British Society for Haematology Guideline. 复发或难治性大b细胞淋巴瘤的治疗：英国血液学学会指南。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-19 DOI: 10.1111/bjh.20129

Sridhar Chaganti, Christopher P Fox, Bernard D Maybury, Cathy Burton, Sally F Barrington, Timothy Illidge, Nagesh Kalakonda, Toby A Eyre, Pam McKay, Andrea Kuhnl, Kate Cwynarski, Andrew J Davies

引用次数: 0

Physical activity, vaso-occlusive crises and pain in patients with sickle cell anaemia in Senegal. 塞内加尔镰状细胞贫血患者的身体活动、血管闭塞危象和疼痛

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-19 DOI: 10.1111/bjh.20150

Mor Diaw, Mame Saloum Coly, Keyne Charlot, Matthieu Gallou-Guyot, Motohiko Miyachi, Tsukasa Yoshida, Macoura Gadji, Saliou Diop, Blaise Felix Faye, Arame Mbengue, Abdoulaye Samb, Sarah Skinner, Elie Nader, Brigitte Ranque, Philippe Connes, Julien Tripette

{"title":"Physical activity, vaso-occlusive crises and pain in patients with sickle cell anaemia in Senegal.","authors":"Mor Diaw, Mame Saloum Coly, Keyne Charlot, Matthieu Gallou-Guyot, Motohiko Miyachi, Tsukasa Yoshida, Macoura Gadji, Saliou Diop, Blaise Felix Faye, Arame Mbengue, Abdoulaye Samb, Sarah Skinner, Elie Nader, Brigitte Ranque, Philippe Connes, Julien Tripette","doi":"10.1111/bjh.20150","DOIUrl":"https://doi.org/10.1111/bjh.20150","url":null,"abstract":"Acute physical exercise may trigger vaso-occlusive crises (VOC) in patients with sickle cell anaemia (SCA), creating uncertainty around physical activity (PA) recommendations. This cross-sectional study examined the relationships between PA, VOC and steady-state pain in 104 Senegalese male patients with SCA. PA was objectively measured over 5 weeks, recording daily steps and time spent in different PA intensities (expressed in metabolic equivalent of task, MET). VOC occurrence was tracked, and steady-state days excluded VOC days plus 2 days before and after. Pain frequency and intensity on steady-state days were recorded via diaries, and blood viscosity was measured. Ninety-eight patients (29 ± 8 years old) completed the study, averaging 9611 ± 4040 steps/day, with 293 ± 108, 64 ± 69 and 28 ± 32 min in ≥1.5, ≥3.0, and ≥6.0 MET PA respectively. Median daily step count and PA duration were not associated with VOC occurrence. However, higher step counts and more time in ≥1.5 and ≥6.0 MET PA correlated with lower pain frequency and intensity (on steady-state days) and lower blood viscosity. These findings suggest that PA may benefit patients with SCA, but further research is needed to establish guidelines.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144101205","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

Hairy cell leukaemia and pregnancy: A systematic review and review of literature. 毛细胞白血病与妊娠：系统回顾和文献回顾。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-19 DOI: 10.1111/bjh.20174

Oscar F Borja-Montes, Alejandro Toro-Pedroza, Narendranath Epperla, Leslie A Andritsos

{"title":"Hairy cell leukaemia and pregnancy: A systematic review and review of literature.","authors":"Oscar F Borja-Montes, Alejandro Toro-Pedroza, Narendranath Epperla, Leslie A Andritsos","doi":"10.1111/bjh.20174","DOIUrl":"https://doi.org/10.1111/bjh.20174","url":null,"abstract":"Hairy cell leukaemia (HCL) in pregnancy is exceptionally uncommon. A systematic review of the literature found 15 reported cases from 1960 to 2025. Eighty percent of cases were first diagnosed during pregnancy at a median maternal age of 32 years and a median gestational age of 14 weeks. Thrombocytopenia was universal and pancytopenia frequent. Forty-six percent of patients received therapy while pregnant, generally within 3 weeks of diagnosis. Interferon-alpha and splenectomy were the most common first-line options; pegylated interferon and rituximab were used less often. No maternal deaths, spontaneous abortions or stillbirths were reported. Median gestational age at delivery was 37 weeks, and 53.8% of births were vaginal. Post-partum therapy was administered in roughly half of the patients, most frequently cladribine, with favourable responses and preserved fertility in documented cases. Overall, current evidence supports carefully selected interferon-alpha, splenectomy or delayed systemic therapy as viable strategies that can control HCL without compromising obstetric outcomes, although individualized management and further evidence is needed.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144101201","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 'Stop TPO-RA in ITP Patients' study: Clinical and immune modulatory effects of romiplostim tapering. “ITP患者停止TPO-RA”研究：罗米普罗stim逐渐减少的临床和免疫调节作用。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-19 DOI: 10.1111/bjh.20100

Vivianne S Nelson, Sufia N Amini, Tanja Netelenbos, Marina S Kartachova, Roger E G Schutgens, Otto Visser, Peter E Westerweel, Jaap J Zwaginga, Suzanne Hofstede-van Egmond, Rick Kapur, Masja de Haas, Leendert Porcelijn, Martin R Schipperus

{"title":"The 'Stop TPO-RA in ITP Patients' study: Clinical and immune modulatory effects of romiplostim tapering.","authors":"Vivianne S Nelson, Sufia N Amini, Tanja Netelenbos, Marina S Kartachova, Roger E G Schutgens, Otto Visser, Peter E Westerweel, Jaap J Zwaginga, Suzanne Hofstede-van Egmond, Rick Kapur, Masja de Haas, Leendert Porcelijn, Martin R Schipperus","doi":"10.1111/bjh.20100","DOIUrl":"https://doi.org/10.1111/bjh.20100","url":null,"abstract":"Sustained remissions off-treatment (SROTs) after tapering of thrombopoietin receptor agonists (TPO-RAs) have been reported in 15%-50% of patients with immune thrombocytopenia (ITP). The STIP (Stop TPO-Receptor Agonist in ITP Patients) study is a prospective trial aimed to investigate the clinical effects of romiplostim tapering. Adult patients (22/40) with ITP ≥3 months received romiplostim for 1 year, were tapered and followed for 1 year. Anti-platelet antibodies (APAs), TPO levels and indium-111 platelet scintigraphy were assessed before, during and after romiplostim. Censored survival analysis showed that the probability of SROT at 1 year after tapering was 23.6% (95% confidence interval: 11.0%-50.5%). Patients with SROT had higher platelet levels on romiplostim (median: 332.5 vs. 84.5 × 109/L) and lower romiplostim doses at the start of tapering (median: 1.0 vs. 4.5 μg/kg) compared to those with a non-sustained response (NSR). APAs were detected in 8/25 patients at baseline, of which 5 showed a substantial decrease during romiplostim. The indium-111 scan revealed an improved platelet survival at the start of tapering for 50% of patients with SROT (2/4, missing n = 1) versus none with an NSR (0/14, missing n = 3). Overall, the STIP study demonstrated a probability of SROT of 23.6% in a diverse and largely chronic group of adult patients with ITP.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092163","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-HSP90α IgG may protect against antiphospholipid syndrome in systemic lupus erythematosus by reducing platelet activation. 抗hsp90 α IgG可能通过降低血小板活化来预防系统性红斑狼疮的抗磷脂综合征。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-19 DOI: 10.1111/bjh.20156

Marina Barguil Macêdo, Andreas Jönsen, Anders A Bengtsson, Iva Gunnarsson, Elisabet Svenungsson, Christian Lood

{"title":"Anti-HSP90α IgG may protect against antiphospholipid syndrome in systemic lupus erythematosus by reducing platelet activation.","authors":"Marina Barguil Macêdo, Andreas Jönsen, Anders A Bengtsson, Iva Gunnarsson, Elisabet Svenungsson, Christian Lood","doi":"10.1111/bjh.20156","DOIUrl":"https://doi.org/10.1111/bjh.20156","url":null,"abstract":"Natural autoantibodies (NAAbs) are immunoglobulins generated in the absence of antigen provocation, with no inherent pathogenic effect, but instead involved in maintaining immune homeostasis. We aimed to evaluate the implication of immunoglobulin G (IgG) antibodies against heat shock protein 90α (HSP90α) in systemic lupus erythematosus (SLE) patients, focusing on how those NAAbs may interfere with the immunothrombosis process. Anti-HSP90α IgG was measured by an enzyme-linked immunosorbent assay in the plasma of 308 SLE individuals, paired with 308 healthy controls (HC). Validation was performed in another cohort, comprised of serum from 125 SLE and 77 HC. In an in vitro experiment, platelets were cultured with mitochondria (abundant sources of HSP90α) treated with anti-HSP90α IgG, and the activation markers thrombospondin-1 and platelet factor 4 were measured in the supernatant. Levels of anti-HSP90α IgG were comparable in SLE and HC, but decreased in the SLE patients with secondary antiphospholipid syndrome (APS), both in the exploratory and the validation cohort (p = 0.0265, and p = 0.0472 respectively). Mitochondria triggered platelet activation in a TLR2- and HSP90α-dependent manner (p = 0.0152 and p < 0.001 respectively). Anti-HSP90α IgG emerges as a prominent NAAb-modulating immunothrombosis in SLE and holds promise as a biomarker of APS risk in these patients.","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092246","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

Splenectomy and complications: A complex different disease background, yet a common denominator exists. 脾切除术和并发症：一个复杂的不同的疾病背景，但一个共同点存在。

IF 5.1 2区医学

British Journal of Haematology Pub Date : 2025-05-19 DOI: 10.1111/bjh.20159

Michael D Diamantidis

引用次数: 0