British Journal of Haematology最新文献

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Factors associated with vaso-occlusive crisis among patients with sickle cell disease in India: An exploratory person-fortnight analysis from a prospective community-based multicentric cohort. 印度镰状细胞病患者血管闭塞危像相关因素:来自前瞻性社区多中心队列的探索性两周分析
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-08-13 DOI: 10.1111/bjh.70753
Yogita Sharma, Shaily B Surti, Deepa Bhat, Parikipandla Sridevi, Jatin Sarmah, Madhusmita Bal, Manoranjan Ranjit, Rabindra K Jena, Bontha V Babu
{"title":"Factors associated with vaso-occlusive crisis among patients with sickle cell disease in India: An exploratory person-fortnight analysis from a prospective community-based multicentric cohort.","authors":"Yogita Sharma, Shaily B Surti, Deepa Bhat, Parikipandla Sridevi, Jatin Sarmah, Madhusmita Bal, Manoranjan Ranjit, Rabindra K Jena, Bontha V Babu","doi":"10.1111/bjh.70753","DOIUrl":"10.1111/bjh.70753","url":null,"abstract":"","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1945-1949"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148756844","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
Survival outcomes and treatment patterns in myelofibrosis in the JAK inhibitor era. JAK抑制剂时代骨髓纤维化的生存结局和治疗模式。
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-08-04 DOI: 10.1111/bjh.70723
Juan-Carlos Hernández-Boluda, Eduardo Arellano-Rodrigo, Manuel Pérez-Encinas, José-María Raya, María-Teresa Gómez-Casares, Francisca Ferrer-Marín, Alberto Hernández-Sánchez, María-Laura Fox, Anna Angona, Beatriz Cuevas, María-Isabel Mata-Vázquez, Rosa Ayala, Natalia de Las Heras, Elvira Mora, Valentín García-Gutiérrez, Alicia Senín, Jasson Villarreal, Blanca Xicoy, Raúl Pérez-López, María García-Fortes, Sonia González de Villambrosia, Elena Magro, María-José Ramírez, Paula Gómez, Angel Ramírez-Payer, Clara Martínez-Valverde, Lucía Pérez-Lamas, Carmen García-Hernández, Mercedes Gasior, Luis-Miguel Juárez-Salcedo, Francisco Ibáñez, María-Luisa Antelo, Lucía Guerrero, Alberto Alvarez-Larrán, Arturo Pereira
{"title":"Survival outcomes and treatment patterns in myelofibrosis in the JAK inhibitor era.","authors":"Juan-Carlos Hernández-Boluda, Eduardo Arellano-Rodrigo, Manuel Pérez-Encinas, José-María Raya, María-Teresa Gómez-Casares, Francisca Ferrer-Marín, Alberto Hernández-Sánchez, María-Laura Fox, Anna Angona, Beatriz Cuevas, María-Isabel Mata-Vázquez, Rosa Ayala, Natalia de Las Heras, Elvira Mora, Valentín García-Gutiérrez, Alicia Senín, Jasson Villarreal, Blanca Xicoy, Raúl Pérez-López, María García-Fortes, Sonia González de Villambrosia, Elena Magro, María-José Ramírez, Paula Gómez, Angel Ramírez-Payer, Clara Martínez-Valverde, Lucía Pérez-Lamas, Carmen García-Hernández, Mercedes Gasior, Luis-Miguel Juárez-Salcedo, Francisco Ibáñez, María-Luisa Antelo, Lucía Guerrero, Alberto Alvarez-Larrán, Arturo Pereira","doi":"10.1111/bjh.70723","DOIUrl":"10.1111/bjh.70723","url":null,"abstract":"<p><p>Data on real-world survival and treatment patterns in large, unselected myelofibrosis (MF) cohorts remain limited. We analysed 1649 patients with MF diagnosed from 2010 onwards and included in the Spanish Myelofibrosis Registry across 64 centres. Janus kinase (JAK) inhibitors were administered to approximately half of the patients. Among those aged ≤70 years with intermediate-2/high-risk International Prognostic Scoring System (IPSS), only 25% ultimately underwent transplantation. Median survival was 6.6 years, with no significant differences between primary and secondary MF; acute leukaemia was the most frequent cause of death, accounting for 20% of cases. Compared with a matched general population, relative survival at 5 and 10 years was 68% and 44% respectively. The reduction in relative survival was more pronounced in patients aged >65 years and in male patients, two subgroups that also exhibited a higher burden of comorbidities and a greater frequency of high molecular risk mutations. Remarkably, patients in the high-risk IPSS category had a 10-year relative survival of only 12%; however, survival reduction was not confined to high-risk disease, with divergence from expected survival emerging early among lower-risk categories. These findings highlight the persistent impact of MF on life expectancy in the contemporary JAK inhibitor era and the need for improvement in its overall management.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1715-1724"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674115/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148700252","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
Comorbid conditions after allogeneic haematopoietic stem cell transplantation in young adults in Germany-A prospective, multicentre trial. 德国年轻人异体造血干细胞移植后的合并症——一项前瞻性、多中心试验
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-08-10 DOI: 10.1111/bjh.70738
Pauline Rocke, Lena Tandetzky, Daniel Wolff, Katharina Egger-Heidrich, Daniela Heidenreich, Kerstin Schaefer-Eckart, Eva Wagner-Drouet, Jochen J Frietsch, Andreas Hochhaus, Inken Hilgendorf
{"title":"Comorbid conditions after allogeneic haematopoietic stem cell transplantation in young adults in Germany-A prospective, multicentre trial.","authors":"Pauline Rocke, Lena Tandetzky, Daniel Wolff, Katharina Egger-Heidrich, Daniela Heidenreich, Kerstin Schaefer-Eckart, Eva Wagner-Drouet, Jochen J Frietsch, Andreas Hochhaus, Inken Hilgendorf","doi":"10.1111/bjh.70738","DOIUrl":"10.1111/bjh.70738","url":null,"abstract":"<p><p>Young adults (YA) are particularly affected by comorbid conditions following allogeneic haematopoietic stem cell transplantation (alloHSCT), as they have to live with them for many years. This is the first multicentre prospective study investigating sequelae after alloHSCT in YA in Germany. In total, 107 patients who survived relapse-free until day 100 after alloHSCT were enrolled. Median patient age was 29.3 years. Chronic graft-versus-host disease (cGvHD) occurred in 40.2%. Comorbidities were captured at days 100, 180, 365 and 910 by applying the post-transplant multimorbidity index (PTMI). Considering all time points, 12.5%-23.5% of patients had no comorbidity, while 39.2%-49.5% had 1-3, 26.3%-33.0% had 4-6 and 5.7%-8.4% had >6 PTMI-defined comorbidities respectively. The most prevalent PTMI-based comorbidities were iron overload, hypogonadism, arterial hypertension, infections, hepatic dysfunction and osteopenia/osteoporosis. However, the spectrum of PTMI-recorded comorbidities and those concurrently self-reported by patients differed substantially. Factors linked to a higher PTMI were total body irradiation and active cGvHD. Investigating single PTMI-defined comorbidities, we found significant associations of osteopenia/osteoporosis with cGvHD, ongoing immunosuppression and hypogonadism, which was more common among female survivors. These results provide a detailed analysis of the occurrence of a vast spectrum of PTMI-defined comorbidities in YA after alloHSCT.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1807-1817"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674112/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148704796","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
Identification of predictive factors for reversal of cerebral vasculopathy in an original longitudinal cohort study in newborns with sickle cell anaemia. 在镰状细胞性贫血新生儿的一项原始纵向队列研究中,确定脑血管病逆转的预测因素。
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-08-06 DOI: 10.1111/bjh.70726
Julie Sommet, Priscilla Boizeau, Zinedine Haouari, Berengère Koehl, Monique Elmaleh, Emmanuelle Lesprit, Florence Missud, Ghislaine Ithier, Laurent Holvoet, Valentine Brousse, Christophe Delclaux, André Denjean, Suzanne Verlhac, Corinne Alberti, André Baruchel, Nathalie Couque, Malika Benkerrou
{"title":"Identification of predictive factors for reversal of cerebral vasculopathy in an original longitudinal cohort study in newborns with sickle cell anaemia.","authors":"Julie Sommet, Priscilla Boizeau, Zinedine Haouari, Berengère Koehl, Monique Elmaleh, Emmanuelle Lesprit, Florence Missud, Ghislaine Ithier, Laurent Holvoet, Valentine Brousse, Christophe Delclaux, André Denjean, Suzanne Verlhac, Corinne Alberti, André Baruchel, Nathalie Couque, Malika Benkerrou","doi":"10.1111/bjh.70726","DOIUrl":"10.1111/bjh.70726","url":null,"abstract":"<p><p>Cerebral macrovasculopathy (CV) is a major complication in children with sickle cell anaemia (SCA) and usually requires a long-term transfusion programme (TP) to prevent stroke. This study aimed to identify factors predicting reversal of CV on TP in a single-centre newborn cohort. Among 375 patients, 50 presented CV and received TP. Median age at diagnosis was 4.9 years [3.6-6.1], with median follow-up of 13.3 years (915 patient-years). Transcranial Doppler (TCD) velocities and magnetic resonance angiography (MRA) normalised in 35 patients, that is, they had reversal of CV (RCV group). Fifteen patients had no reversal of CV (No-RCV group). Kaplan-Meier analysis estimated that 48% (95% confidence interval [CI] [33-62]) still had abnormal TCD and/or MRA 5 years after diagnosis. In a multivariate Cox model, three baseline factors independently predicted CV reversal: age at CV onset, initial severity of stenosis and bronchial obstruction. Older age at diagnosis increased the likelihood of CV reversal (hazard ratio [HR] 1.31 per year, 95% CI [1.08-1.59], p = 0.01). In contrast, severe stenosis (HR 0.72 per 10% increase, 95% CI [0.62-0.85], p < 0.0001) and bronchial obstruction (HR 0.14, 95% CI [0.03-0.63], p = 0.001) reduced the likelihood of CV reversal. These results suggest that, beyond TP, managing bronchial obstruction may improve vascular outcomes.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1856-1865"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674129/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148682688","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
Serum thioredoxin at allogeneic haematopoietic stem cell transplantation predicts acute graft-versus-host disease. 同种异体造血干细胞移植血清硫氧还蛋白预测急性移植物抗宿主病。
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-09-06 DOI: 10.1111/bjh.70739
Masaharu Tamaki, Hideki Nakasone, Kosuke Takano, Shunto Kawamura, Yukiko Misaki, Kazuki Yoshimura, Yosuke Okada, Aki Tanihara, Koki Hashino, Kaisuke Yamamoto, Yuya Nakata, Takuto Ishikawa, Akari Matsuoka, Tomohiro Meno, Yuhei Nakamura, Masakatsu Kawamura, Junko Takeshita, Nozomu Yoshino, Ayumi Gomyo, Machiko Kusuda, Shun-Ichi Kimura, Shinichi Kako, Yoshinobu Kanda
{"title":"Serum thioredoxin at allogeneic haematopoietic stem cell transplantation predicts acute graft-versus-host disease.","authors":"Masaharu Tamaki, Hideki Nakasone, Kosuke Takano, Shunto Kawamura, Yukiko Misaki, Kazuki Yoshimura, Yosuke Okada, Aki Tanihara, Koki Hashino, Kaisuke Yamamoto, Yuya Nakata, Takuto Ishikawa, Akari Matsuoka, Tomohiro Meno, Yuhei Nakamura, Masakatsu Kawamura, Junko Takeshita, Nozomu Yoshino, Ayumi Gomyo, Machiko Kusuda, Shun-Ichi Kimura, Shinichi Kako, Yoshinobu Kanda","doi":"10.1111/bjh.70739","DOIUrl":"10.1111/bjh.70739","url":null,"abstract":"<p><p>Thioredoxin (TXN) is a component of a major reductive system in various eukaryotes. Although oxidative stress is considered to contribute to the development of acute graft-versus-host disease (GVHD), the clinical significance of TXN remains to be elucidated. The current study included 118 patients who had undergone their first allogeneic haematopoietic stem cell transplantation (allo-HSCT) between 2022 and 2025, and their serum samples at day 0 were sequentially collected (training cohort). The study also assessed 47 patients who had undergone their first allo-HSCT between 2012 and 2015 and whose serum at around day 0 had previously been cryopreserved and was available (validation cohort). The high-TXN group showed a lower cumulative incidence of grade II-IV acute GVHD (100-day incidence: 47.5% vs. 59.2%), although the difference did not reach statistical significance (p = 0.058). The validation cohort demonstrated similar results (0% vs. 35.8%, p = 0.053). However, multivariate analysis revealed that high-TXN group was significantly associated with a reduced risk of acute GVHD (hazard ratio [HR] 0.48, p = 0.031). In addition, the combination of TXN with clinical factors enhanced the predictive potential for acute GVHD compared with that of clinical factors alone (p = 0.0039). TXN might be useful for predicting acute GVHD and could contribute to the establishment of a prediction model.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1762-1772"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148899836","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
Avatrombopag for persistent and chronic immune thrombocytopenia in children: A post hoc analysis of clinical characteristics of response from a phase 3b clinical trial. Avatrombopag治疗儿童持续性和慢性免疫性血小板减少症:一项3b期临床试验临床反应特征的事后分析
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-07-21 DOI: 10.1111/bjh.70709
Rachael F Grace, Göksel Leblebisatan, Yesim Aydinok, Sule Unal, John Grainger, Amanda B Grimes, Michele P Lambert, Jessica Zhang, Brian D Jamieson, Faizan Sattar, Scott Kolodny, Michael Vredenburg
{"title":"Avatrombopag for persistent and chronic immune thrombocytopenia in children: A post hoc analysis of clinical characteristics of response from a phase 3b clinical trial.","authors":"Rachael F Grace, Göksel Leblebisatan, Yesim Aydinok, Sule Unal, John Grainger, Amanda B Grimes, Michele P Lambert, Jessica Zhang, Brian D Jamieson, Faizan Sattar, Scott Kolodny, Michael Vredenburg","doi":"10.1111/bjh.70709","DOIUrl":"10.1111/bjh.70709","url":null,"abstract":"<p><p>Avatrombopag treatment resulted in significantly higher platelet response rates in patients when compared with placebo in a phase 3b study of patients with paediatric immune thrombocytopenia (ITP) (NCT04516967). These post hoc analyses of the 12-week core phase further assessed the efficacy of avatrombopag, considering the influence of baseline characteristics and protocol-defined dose adjustments on the achievement and sustainability of response. The cumulative percentage of patients who achieved a platelet response (2 consecutive platelet counts ≥50 × 10<sup>9</sup>/L) by days 15 and 22 was 70.4% and 75.9%, respectively, with avatrombopag (placebo, 0% at both days). Only minor differences were observed by baseline characteristics. Among the 92.6% of patients (50/54) treated with avatrombopag who achieved a clinically meaningful platelet response (platelet count ≥30 × 10<sup>9</sup>/L), the mean percentage of time with response was 62.5%. Protocol-specified dose holds due to a platelet count >250 × 10<sup>9</sup>/L occurred for 61.1% of patients, which may have impacted the efficacy outcomes. 78.3% of non-durable responders had ≥1 dose holds prompted by a platelet count of 250-400 × 10<sup>9</sup>/L, a platelet count that would not prompt a dose hold in real-world clinical practice. Avatrombopag demonstrated a clinically meaningful platelet response across the clinical and demographic subgroups in this heavily pre-treated and refractory paediatric ITP population.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1818-1827"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674185/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148547168","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
Long-term results of brentuximab vedotin and bendamustine for first-line treatment of Hodgkin lymphoma in the elderly (HALO trial). brentuximab vedotin和苯达莫司汀一线治疗老年人霍奇金淋巴瘤的长期结果(HALO试验)。
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-08-26 DOI: 10.1111/bjh.70763
Andrea Gallamini, Simonetta Viviani, Davide Rapezzi, Caterina Patti, Pierre Feugier, Alessandro Rambaldi, Fontanet Bijou, Anna Borra, Cécile Borel, Tanguy Pace-Loscos, Renaud Schiappa, Stephane Chauvie, Colin Debaigt, Anna Guidetti, Olivier Humbert, Jean-Marc Schiano De Colella
{"title":"Long-term results of brentuximab vedotin and bendamustine for first-line treatment of Hodgkin lymphoma in the elderly (HALO trial).","authors":"Andrea Gallamini, Simonetta Viviani, Davide Rapezzi, Caterina Patti, Pierre Feugier, Alessandro Rambaldi, Fontanet Bijou, Anna Borra, Cécile Borel, Tanguy Pace-Loscos, Renaud Schiappa, Stephane Chauvie, Colin Debaigt, Anna Guidetti, Olivier Humbert, Jean-Marc Schiano De Colella","doi":"10.1111/bjh.70763","DOIUrl":"10.1111/bjh.70763","url":null,"abstract":"<p><p>The Hodgkin lymphoma treatment in the elderly (eHL) is a challenge due to a narrow therapeutic window between therapy effectiveness and toxicity. Both bendamustine (Be) and brentuximab vedotin (BV) are well-tolerated, effective drugs in relapsing HL, but no data exist on Be-BV frontline treatment in eHL. The prospective, open-label, phase I/II Hodgkin lymphoma combining Adcetris® and Levact® in Old patients (HALO) study was launched (Clinical Trial.Gov Id. 02467946) to test safety and efficacy of Be-BV in untreated eHL. The mean age was 70.75 (62-79), stage IIB in 12, III in 14 and IV in 31 patients. Most had B-symptoms and an International Prognostic Score (IPS) ≥3 (39). Despite a good performance status and both a high activity of daily living (ADL) (≥6 in 85%) and instrumental activities of daily living (IADL) scores (≥8 in 77%), 68% of patients had ≥5 comorbidities, with a Cumulative Illness Rating Scale-Geriatric (CIRS)-G score >3 in 75%. A complete metabolic response (CMR) was achieved in 44/57 of patients. The 6-year overall survival (OS) and progression-free survival (PFS) in per-protocol (PP) analysis were 67% (95% confidence intertvals [CI] 53-84) and 45% (95% CI 32-65) respectively. A PFS event was observed in 35: 13 progressions (10 deaths), 13 relapses (6 deaths) and 9 deaths. Be-BV, when given in a full dose schedule, is an effective regimen for unselected, poor-risk elderly Hodgkin lymphoma (HL) patients.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1619-1628"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674205/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816893","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
The association between inborn errors of immunity and Hodgkin lymphoma-A register-based case-control study in Sweden. 先天免疫缺陷与霍奇金淋巴瘤之间的关系——瑞典一项基于登记的病例对照研究
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-08-12 DOI: 10.1111/bjh.70743
Agnes Winsa, Ingemar Lagerlöf, Fredrik Ellin, Mats Fredriksson, Åsa Nilsdotter-Augustinsson, Marcus Bendtsen, Jörg Cammenga, Sofia Nyström
{"title":"The association between inborn errors of immunity and Hodgkin lymphoma-A register-based case-control study in Sweden.","authors":"Agnes Winsa, Ingemar Lagerlöf, Fredrik Ellin, Mats Fredriksson, Åsa Nilsdotter-Augustinsson, Marcus Bendtsen, Jörg Cammenga, Sofia Nyström","doi":"10.1111/bjh.70743","DOIUrl":"10.1111/bjh.70743","url":null,"abstract":"<p><p>Immune dysfunction is implicated in the aetiology of Hodgkin lymphoma (HL), but the association between inborn errors of immunity (IEI) and HL remains poorly understood. We conducted a retrospective, register-based case-control study in Sweden over five decades to investigate the relationship between IEI and HL. Individuals diagnosed with HL in the Swedish Cancer Register between 1969 and 2019 were matched with population controls. Data were linked to the National Patient Register to identify recorded the International Classification of Diseases (ICD)-coded IEI diagnoses. Odds ratios (ORs) for HL associated with any IEI and IEI subgroups were estimated using conditional logistic regression. The study included 10 549 HL patients and 42 225 controls. Median age at HL diagnosis was 49 years, and 57% were male. Twenty-two HL cases and 11 controls had a recorded IEI diagnosis; most were predominantly antibody deficiencies (PAD; 14 cases and 8 controls). Any IEI was associated with increased odds of HL (OR 8.0; 95% confidence interval [CI] 3.9-16.5). No clear differences in HL risk were observed between IEI subtypes. IEI was strongly associated with increased odds of HL. These findings support a role for IEI in HL pathogenesis and may suggest a distinct aetiological pathway for HL compared with other IEI-associated lymphomas.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1629-1637"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674171/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148719495","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
LTB-TREAT: A validated international prognostic model for time to treatment in low tumour burden follicular lymphoma. LTB-TREAT:一种有效的低肿瘤负荷滤泡性淋巴瘤治疗时间国际预后模型。
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-08-27 DOI: 10.1111/bjh.70748
Luani Barge, Lachlan Webb, Anita Pelecanos, Niamh Waters, Ross Salvaris, Sam Lai, Tessa Potezny, Helen Cashman, Caitlyn Nguyen-Ngo, Denise Lee, Costas K Yannakou, Nada Hamad, Adrien Chauchet, Sylvain Choquet, Cédric Rossi, Adélie Herbin, Lukshe Kanagaratnam, Cyrielle Rodier, Constantine S Tam, Abir Bhattacharyya, Chan Y Cheah, Eliza A Hawkes, Kirk Morris, Eric Durot, Joshua Tobin, Greg Hapgood
{"title":"LTB-TREAT: A validated international prognostic model for time to treatment in low tumour burden follicular lymphoma.","authors":"Luani Barge, Lachlan Webb, Anita Pelecanos, Niamh Waters, Ross Salvaris, Sam Lai, Tessa Potezny, Helen Cashman, Caitlyn Nguyen-Ngo, Denise Lee, Costas K Yannakou, Nada Hamad, Adrien Chauchet, Sylvain Choquet, Cédric Rossi, Adélie Herbin, Lukshe Kanagaratnam, Cyrielle Rodier, Constantine S Tam, Abir Bhattacharyya, Chan Y Cheah, Eliza A Hawkes, Kirk Morris, Eric Durot, Joshua Tobin, Greg Hapgood","doi":"10.1111/bjh.70748","DOIUrl":"10.1111/bjh.70748","url":null,"abstract":"<p><p>Watchful waiting (WW) in low tumour burden follicular lymphoma (LTB FL) defers treatment. There is a need to develop a model to predict time to treatment (TTT). We aimed to develop and externally validate a model for TTT in LTB FL. We conducted a retrospective analysis across eight centres within the Australasian Lymphoma Alliance. Two hundred and twenty-nine patients were included between 2004 and 2022 with a median follow-up of 5.0 years (range 0.5-17.8 years) and a median age was 64 years. All patients were Group d'Etude des Lymphomes Folliculaires (GELF) negative and managed with WW. Elevated lactate dehydrogenase and >4 nodal areas were independent prognostic factors associated with shorter TTT. These factors formed a prognostic model identifying three groups: low 59% (median TTT 6.34 years), intermediate 36% (median TTT 4.08 years), high risk 5% (median TTT 1.49 years) (p < 0.001). There was a higher incidence of serious complications among risk groups (low 7.5% vs. intermediate 13.3% vs. high 25%; p = 0.096). The prognostic value of the LTB-TREAT model was confirmed in an external validation cohort. The low-risk group is predicted to have a prolonged TTT with WW and a high-risk group is predicted to experience early progression in whom treatment should be considered.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1673-1683"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674130/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148823500","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
Parvovirus B19 infections in paediatric sickle cell disease patients: Genotype and hydroxyurea treatment influence disease severity. 小儿镰状细胞病患者细小病毒B19感染:基因型和羟基脲治疗影响疾病严重程度
IF 3.6 2区 医学
British Journal of Haematology Pub Date : 2026-10-01 Epub Date: 2026-09-13 DOI: 10.1111/bjh.70809
Matthias Bleeke, Arne Simon, Beate Winkler, Sabine Heine, Anna Eichinger, Ulrich von Both, Meinolf Siepermann, Sonya El Amouri, Arndt Borkhardt, Yvonne Pritschow, Wolfgang Novak, Michael Dworzak, Sarah Goretzki, Clara Held, Karin Beutel, Franziska Gams, Henriette Rudolph, Leila Koscher, Piet E Beusker, Eckard Hamelmann, Stefan Weichert, Svenja Häuser, Maximilian Mauritz, Malik Aydin, Verena Wiegering, Regine Grosse, Rainer Misgeld, Martina Stiefel, Nicole Töpfner, Uta Falke, Lara Imhof, Laura Tagliaferri, Daniel Ebrahimi-Fakhari, Andreas Groll, Marion Appel, Nora Naumann-Bartsch, Michaela Nathrath, Olaf Beck, Solvej Heidtmann, Philipp Tischer, Ulf Schulze-Sturm, Urs Mücke, Holger Cario
{"title":"Parvovirus B19 infections in paediatric sickle cell disease patients: Genotype and hydroxyurea treatment influence disease severity.","authors":"Matthias Bleeke, Arne Simon, Beate Winkler, Sabine Heine, Anna Eichinger, Ulrich von Both, Meinolf Siepermann, Sonya El Amouri, Arndt Borkhardt, Yvonne Pritschow, Wolfgang Novak, Michael Dworzak, Sarah Goretzki, Clara Held, Karin Beutel, Franziska Gams, Henriette Rudolph, Leila Koscher, Piet E Beusker, Eckard Hamelmann, Stefan Weichert, Svenja Häuser, Maximilian Mauritz, Malik Aydin, Verena Wiegering, Regine Grosse, Rainer Misgeld, Martina Stiefel, Nicole Töpfner, Uta Falke, Lara Imhof, Laura Tagliaferri, Daniel Ebrahimi-Fakhari, Andreas Groll, Marion Appel, Nora Naumann-Bartsch, Michaela Nathrath, Olaf Beck, Solvej Heidtmann, Philipp Tischer, Ulf Schulze-Sturm, Urs Mücke, Holger Cario","doi":"10.1111/bjh.70809","DOIUrl":"10.1111/bjh.70809","url":null,"abstract":"<p><p>In patients with sickle cell disease (SCD), parvovirus B19 infection (B19V) leads to acute anaemia (aplastic crisis), but may also be associated with other serious complications. We retrospectively analysed clinical data from paediatric SCD patients with B19V infections between 2023 and 2025, including symptoms, laboratory parameters, diagnostic procedures and treatment details to detect differences between SCD genotypes (HbSS and HbS/β<sup>0</sup>-thalassaemia, i.e. HbSS/Sβ<sup>0</sup>, and HbSC) and to determine the effect of hydroxyurea (HU; hydroxycarbamide) treatment in HbSS/Sβ<sup>0</sup> patients. Ninety-eight patients (76 HbSS/Sβ<sup>0</sup>, 22 HbSC) were included in the study. There were significant differences in the symptoms of infection between genotypes, with a high prevalence of back pain as well as higher C-reactive protein and lactate dehydrogenase levels in HbSC. HbSC patients also received chest X-ray more frequently and required longer inpatient treatment. In addition, HbSC patients showed a significantly higher rate of immunoglobulin G seroconversion at the onset of symptoms, suggesting that antibody formation influences clinical appearance. In HbSS/Sβ<sup>0</sup> patients, HU treatment was associated with higher haemoglobin and reduced transfusion requirements, but more frequently with neutropenia. In summary, B19V infections induce different clinical presentations depending on SCD genotypes, with HbSC patients showing a distinct phenotypic presentation. HU treatment reduces the need for transfusions in patients with HbSS/Sβ<sup>0</sup>.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":"1845-1855"},"PeriodicalIF":3.6,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674147/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148962763","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
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