Haematologica最新文献_第4页

Immune mediated marrow failure in very young children: horse ATG once again out of the magician's hat. 免疫介导的骨髓衰竭在非常年幼的儿童：马ATG再一次走出魔术师的帽子。

IF 7.9 1区医学

Haematologica Pub Date : 2026-04-30 DOI: 10.3324/haematol.2026.301004

Marc Bierings, Kirsten Thus

引用次数: 0

Idiopathic multicentric Castleman - TAFRO syndrome can mimic sepsis and myeloid neoplasia. 特发性多中心Castleman - TAFRO综合征可以模拟脓毒症和髓系肿瘤。

IF 7.9 1区医学

Haematologica Pub Date : 2026-04-30 DOI: 10.3324/haematol.2025.300349

Ole Hudowenz, Jan Vorwerk, Cyrus Khandanpour, Nikolas Von Bubnoff, Wolfram Klapper, Ilske Oschlies, Jonas Ströder, Niklas Gebauer, Philip Muck, Martin Nitschke, Tobias Graf

引用次数: 0

The evolution of the complete blood count: have we gone too far? Perhaps we have. Comment on: "The evolution of the complete blood count: have we gone too far?" 全血细胞计数的进化：我们是否走得太远了？也许是这样。评论：“全血细胞计数的演变：我们是否走得太远了？”

IF 7.9 1区医学

Haematologica Pub Date : 2026-04-30 DOI: 10.3324/haematol.2026.301048

Daniel Mazza Matos

引用次数: 0

SIRT3 deSUMOylation sustains hematopoietic stem cell activities under stressful conditions via the DHX58-IRF7 axis. SIRT3去苏酰化通过DHX58-IRF7轴在应激条件下维持造血干细胞的活性。

IF 7.9 1区医学

Haematologica Pub Date : 2026-04-30 DOI: 10.3324/haematol.2025.300025

Xiaoxiao He, Jing Yu, Yilu Xu, Tianshi Wang, Yang Lu, Jiaxin Wu, Sijia Dan, Yiqiu Xia, Xingmei Mu, Li Xie, Chiqi Chen, Zhuo Yu, Jing Ye, Eric Gilson, Yaping Zhang, Jinke Cheng, Junke Zheng

{"title":"SIRT3 deSUMOylation sustains hematopoietic stem cell activities under stressful conditions via the DHX58-IRF7 axis.","authors":"Xiaoxiao He, Jing Yu, Yilu Xu, Tianshi Wang, Yang Lu, Jiaxin Wu, Sijia Dan, Yiqiu Xia, Xingmei Mu, Li Xie, Chiqi Chen, Zhuo Yu, Jing Ye, Eric Gilson, Yaping Zhang, Jinke Cheng, Junke Zheng","doi":"10.3324/haematol.2025.300025","DOIUrl":"https://doi.org/10.3324/haematol.2025.300025","url":null,"abstract":"Hematopoietic stem cells (HSCs) sustain physiological hematopoiesis by generating all hematopoietic cells. However, the connection between HSC stemness/activities and organismal lifespan, especially under stress, remains incompletely understood. We previously revealed a unique mutation at the SUMOylation site of SIRT3 (lysine 223 to arginine, K223R) that could enhance its deacetylase activity. Here, using Sirt3-K223R transgenic mice, we demonstrate that SIRT3 deSUMOylation promotes HSC self-renewal, inhibits myeloid differentiation, and delays HSC senescence under multiple stress conditions. Notably, these effects are associated with extended lifespan in mice, though the underlying link between preserved HSC function and lifespan extension needs further investigation. Mechanistically, SIRT3 regulates mitochondrial metabolic activity and promotes the deacetylation of H3K9 and H3K27. This epigenetic modification attenuates the RIG-I signaling pathway by downregulating DHX58 and IRF7, sustaining HSC activities. Our study uncovers a unique SIRT3-DHX58-IRF7 axis that sustains HSC activities to delay organismal aging under stress, providing insights into potential anti-aging strategies targeting hematopoietic homeostasis.","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":""},"PeriodicalIF":7.9,"publicationDate":"2026-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147770036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Novel KAT6B::NUTM2B fusion oncogene in congenital acute myeloid leukemia and implications for menin inhibitor therapy. 新型KAT6B::NUTM2B融合癌基因在先天性急性髓性白血病中的作用及其对脑膜蛋白抑制剂治疗的意义。

IF 10.1 1区医学

Haematologica Pub Date : 2026-04-23 DOI: 10.3324/haematol.2025.300350

Rebecca K Voss,Wenqing Qi,La'Ron Browne,Ruth W Wang'ondu,Mahsa Khanlari,Jing Ma,Xuefei Ma,Josi Lott,Juan M Barajas,Melvin Thomas Iii,Victor B Pastor Loyola,Hui Cao,Larissa V Furtado,Swati Naik,Sakshi Bami,Seth E Karol,Jeffrey E Rubnitz,Jeffery M Klco,Yen-Chun Liu,Lu Wang

引用次数: 0

Prevalence and impact of frailty assessed using standard geriatrics tools in older adults with blood cancers. 使用标准老年病学工具评估患有血癌的老年人的患病率和虚弱的影响

IF 10.1 1区医学

Haematologica Pub Date : 2026-04-23 DOI: 10.3324/haematol.2025.300233

Clark DuMontier,Angel M Cronin,Tammy T Hshieh,Ameya Sanyal,Lee Mozessohn,Maya M Abdallah,Daniel J DeAngelo,Nikhil C Munshi,Richard M Stone,Robert J Soiffer,Jane A Driver,Gregory A Abel

{"title":"Prevalence and impact of frailty assessed using standard geriatrics tools in older adults with blood cancers.","authors":"Clark DuMontier,Angel M Cronin,Tammy T Hshieh,Ameya Sanyal,Lee Mozessohn,Maya M Abdallah,Daniel J DeAngelo,Nikhil C Munshi,Richard M Stone,Robert J Soiffer,Jane A Driver,Gregory A Abel","doi":"10.3324/haematol.2025.300233","DOIUrl":"https://doi.org/10.3324/haematol.2025.300233","url":null,"abstract":"Geriatric assessment is recommended for older adults undergoing cancer treatment, but the best frailty assessment tools for blood cancers are unknown. We conducted a prospective cohort study of three gold standard approaches: the cumulative deficit index, phenotypic frailty, and four-meter gait speed. From February 2015 to January 2025, a research assistant approached patients aged ≥ 73 years attending new consultations for leukemia, lymphoma, and multiple myeloma. A total of 1011 patients underwent frailty assessment with a median age of 78 years (IQR, 76-82 years) and a median follow-up among survivors of 43 months (IQR, 18-74 months). For example, when classified by phenotypic frailty, 11% were frail, 59% pre-frail, and 30% robust; using this tool, frail patients lived 24 months compared to 92 months among the robust. While frailty classification varied across measures, all three tools were associated with mortality in a dose-dependent manner, independent of age and sex (cumulative deficit index: ref=robust, pre-frail HR 1.86 [95% CI 1.54, 2.26]; frail HR 2.42 [1.85, 3.17]; phenotypic frailty: ref=robust; pre-frail HR 1.88 [1.50, 2.37], frail HR 3.01, [2.22, 4.07]); gait speed: ref=≥ 0.8 m/s; 0.6 to < 0.8 m/s HR 1.44 [1.18, 1.76], < 0.6 m/s HR 2.06 [1.61, 2.63]). Associations were similar when analyzed by disease clinic; cumulative deficit further stratified risk in patients assessed by phenotypic frailty and gait speed. Our findings provide a new benchmark on frailty and its impact on survival in older adults with blood cancers, with care delivery implications for operationalization in clinical practice and research.","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":"45 1","pages":""},"PeriodicalIF":10.1,"publicationDate":"2026-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147733459","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Phase Ib/II trial of anti-CD33 monoclonal antibody BI 836858 and azacitidine in previously untreated older acute myeloid leukemia patients: Beat AML S2 sub-study results. 抗cd33单克隆抗体BI 836858和阿扎胞苷在先前未治疗的老年急性髓性白血病患者中的Ib/II期试验：Beat AML S2亚研究结果

IF 10.1 1区医学

Haematologica Pub Date : 2026-04-23 DOI: 10.3324/haematol.2026.300627

Michael J Hochman,Amy S Ruppert,Ying Huang,Alice Mims,Uma Borate,Ronan Swords,Elie Traer,Maria R Baer,Vu H Duong,Wendy Stock,Toyosi Odenike,Prapti Patel,Yazan Madanat,Eytan Stein,Michael W Deininger,Gary Schiller,Daniel A Pollyea,James M Foran,Rebecca Olin,Tara Lin,Michael M Boyiadzis,Shivani Handa,Bjoern Rueter,Theophilus J Gana,Nyla A Heerema,Timothy Chen,Molly Martycz,Mona Stefanos,Sonja Marcus,Leonard Rosenberg,Brian Druker,Ross Levine,Amy Burd,Ashley O Yocum,John C Byrd,William Blum

引用次数: 0

Bedaquiline amplifies proteasome inhibitor efficacy and overcomes resistance in multiple myeloma. 贝达喹啉增强蛋白酶体抑制剂疗效，克服多发性骨髓瘤耐药。

IF 7.9 1区医学

Haematologica Pub Date : 2026-04-23 DOI: 10.3324/haematol.2025.300157

Michela Cumerlato, Monica Maccagno, María Labrador, Beatrice Luciano, Elisabetta Mereu, Mariangela Porro, Cecilia Bandini, Mattia Furlan, Domenico Maisano, Mattia D'Agostino, Alessandra Larocca, Francesca Gay, Annalaura Tamburrini, Francesca Anselmi, Paolo Ettore Porporato, Salvatore Oliviero, Benedetto Bruno, Eugenio Morelli, Nikhil Munshi, Roberto Piva

{"title":"Bedaquiline amplifies proteasome inhibitor efficacy and overcomes resistance in multiple myeloma.","authors":"Michela Cumerlato, Monica Maccagno, María Labrador, Beatrice Luciano, Elisabetta Mereu, Mariangela Porro, Cecilia Bandini, Mattia Furlan, Domenico Maisano, Mattia D'Agostino, Alessandra Larocca, Francesca Gay, Annalaura Tamburrini, Francesca Anselmi, Paolo Ettore Porporato, Salvatore Oliviero, Benedetto Bruno, Eugenio Morelli, Nikhil Munshi, Roberto Piva","doi":"10.3324/haematol.2025.300157","DOIUrl":"https://doi.org/10.3324/haematol.2025.300157","url":null,"abstract":"Proteasome inhibitors (PIs) are cornerstone therapies for multiple myeloma (MM), yet resistance remains a major barrier to durable responses. To identify druggable vulnerabilities that enhance PI efficacy, we performed a small-molecule chemical screen in the presence of carfilzomib (CFZ). We identified bedaquiline (BDQ), an FDAapproved antimycobacterial agent, as a potent synergistic partner. BDQ and its fumarate salt (BDQ-F) significantly amplified CFZ-induced cytotoxicity in PI-sensitive and PI-resistant MM cells, in AL amyloidosis and other B-cell malignancies, with minimal toxicity toward normal cells. Mechanistic studies confirmed that BDQ specifically targets the ATP5F1C subunit of mitochondrial ATP synthase. BDQ-CFZ combination triggered extensive apoptosis, exacerbating proteotoxic stress and proteasome-associated pathways. BDQ specifically enhanced CFZ's inhibition of the proteasome's chymotrypsin-like activity. Importantly, BDQ synergized with multiple proteasome and ubiquitin-activating enzyme inhibitors, but not with other standard MM agents, underscoring its selective interaction with the UPS pathway. BDQ-CFZ cotreatment markedly reduced MM cell viability and tumor burden in patient-derived cells and zebrafish xenograft models. These findings support the therapeutic repurposing of BDQ to potentiate PI efficacy and overcome resistance in MM and related B-cell malignancies.","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":""},"PeriodicalIF":7.9,"publicationDate":"2026-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147769932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Favorable safety and efficacy profiles of α-1-antitrypsin in steroid- and ruxolitinib-refractory acute graft-versus-host disease of the gastrointestinal tract: a retrospective, single center study. α-1-抗胰蛋白酶在类固醇和鲁索利替尼难治性胃肠道急性移植物抗宿主病中的良好安全性和有效性：一项回顾性单中心研究

IF 10.1 1区医学

Haematologica Pub Date : 2026-04-23 DOI: 10.3324/haematol.2026.300541

Dana Yehudai-Ofir,Hazim Khatib,Ofrat Beyar-Katz,Avraham Frisch,Baher Krayem,Tsila Zuckerman,Israel Henig

引用次数: 0

Hematological theology and the limits of retrospective diagnosis: a critique of the hemophilia hypothesis in Exodus 4:24-26. Comment on: ""Bridegroom of blood" - a biblical reference to hemophilia?" 血液学神学和回顾性诊断的局限性：对出埃及记4:24-26中血友病假说的批判。评论：“血的新郎”——《圣经》中提到的血友病？”

IF 10.1 1区医学

Haematologica Pub Date : 2026-04-23 DOI: 10.3324/haematol.2026.301036

G Shellye Horowitz

引用次数: 0