American Journal of Hematology最新文献

{"title":"Normalization of Cerebral Hemodynamics After Gene Therapy in Adults With Sickle Cell Disease.","authors":"Akshay Sharma, Jane S Hankins, Jaap-Jan Boelens, Maria Cancio, Radhika Peddinti, Lawrence Rispoli, Sarah Sloan, Sarah DiDominick Costa, Sharon Peled, Andrea Wiethoff, Ranganatha Sitaram, James L LaBelle","doi":"10.1002/ajh.27757","DOIUrl":"https://doi.org/10.1002/ajh.27757","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":10.1,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144504515","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}

{"title":"Pirtobrutinib is an Effective Salvage Treatment After Ibrutinib in Bing–Neel Syndrome","authors":"Anna Maria Frustaci, Arianna Zappaterra, Gloria Turri, Valentina Rossi, Marina Deodato, Carlo Gambacorti‐Passerini, Roberto Cairoli, Romano Danesi, Alessandra Tedeschi","doi":"10.1002/ajh.27759","DOIUrl":"https://doi.org/10.1002/ajh.27759","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"45 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144503476","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}

{"title":"Pirtobrutinib is an Effective Salvage Treatment After Ibrutinib in Bing-Neel Syndrome.","authors":"Anna Maria Frustaci, Arianna Zappaterra, Gloria Turri, Valentina Rossi, Marina Deodato, Carlo Gambacorti-Passerini, Roberto Cairoli, Romano Danesi, Alessandra Tedeschi","doi":"10.1002/ajh.27759","DOIUrl":"https://doi.org/10.1002/ajh.27759","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":10.1,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144504516","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}

{"title":"Normalization of Cerebral Hemodynamics After Gene Therapy in Adults With Sickle Cell Disease","authors":"Akshay Sharma, Jane S. Hankins, Jaap‐Jan Boelens, Maria Cancio, Radhika Peddinti, Lawrence Rispoli, Sarah Sloan, Sarah DiDominick Costa, Sharon Peled, Andrea Wiethoff, Ranganatha Sitaram, James L. LaBelle","doi":"10.1002/ajh.27757","DOIUrl":"https://doi.org/10.1002/ajh.27757","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"270 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144503473","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}

{"title":"Loss of Function SPTA1 Variants Causes Neonatal Liver Failure and Fetal Anemia.","authors":"John Brewin,Barnaby Clark,Frances Smith,Nicholas Parkin,Amina Nardo-Marino,Adam Gassas,Maesha Deheragoda,Jonathon Hind,Sue Height,Subarna Chakravorty,Anil Dhawan,David Rees","doi":"10.1002/ajh.27751","DOIUrl":"https://doi.org/10.1002/ajh.27751","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"26 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144488109","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}

{"title":"Frailty, Genetic Risk, and Long-Term Risk of Venous Thromboembolism: Insight From a UK Biobank Study.","authors":"Jiatang Xu,Qiushi Ren,Yangfan Su,Liling Lin,Runnan Shen,Kai Huang","doi":"10.1002/ajh.27753","DOIUrl":"https://doi.org/10.1002/ajh.27753","url":null,"abstract":"Frailty is more prevalent among venous thromboembolism (VTE) patients. However, the relationship between long-term VTE risk and frailty remained unexplored. This study included 315 523 participants recruited from UK Biobank between 2006 and 2010. Frailty status of participants at recruitment was evaluated by physical frailty and frailty index. The association between frailty and long-term risk of VTE was assessed through Cox proportional models. After excluded participants without genetic data, we further explored the interaction and joint effect between frailty and genetic risk in 258 461 participants. Mediation analyses were performed to investigate whether frailty mediates the association between cancer, obesity, and VTE. Results revealed significant associations between frailty and increased risk of VTE (physical frailty: HR, 1.63; 95% CI, 1.49-1.78; frailty index: HR, 1.66; 95% CI, 1.53-1.78). Participants with high genetic risk and frailty exhibited the highest VTE risk (physical frailty: HR, 3.83; 95% CI, 3.22-4.55; frailty index: HR, 3.96; 95% CI, 3.41-4.62). Frailty and genetic risk exhibited a significant additive interaction, with a relative excess risk due to interaction (RERI) of 0.76 in the physical frailty cohort, and a RERI of 1.11 in the frailty index cohort. Frailty mediated approximately 14% of the association between cancer and VTE, and 9% of the association between obesity and VTE. In conclusion, frailty is significantly associated with an increased long-term risk of VTE in the general population. Combining frailty and genetic risk can assist in the identification of high-risk VTE populations. Also, frailty significantly mediates the association between cancer, obesity, and VTE.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"101 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144370200","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}

{"title":"Impact of Duffy Status on Neutrophil Counts and Clinical Outcomes in Pediatric Patients With Sickle Cell Disease","authors":"Yan Zheng, Jeffrey Gossett, Pei‐Lin Chen, Guolian Kang, Clifford M. Takemoto","doi":"10.1002/ajh.27748","DOIUrl":"https://doi.org/10.1002/ajh.27748","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"25 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144334844","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}

{"title":"Thromboembolic Events Are Increased After Splenectomy in Postmenopausal Women","authors":"Qi Feng, Yi Mu, Christopher Kabrhel, Bernard Rosner, Rulla M. Tamimi, James B. Bussel","doi":"10.1002/ajh.27714","DOIUrl":"https://doi.org/10.1002/ajh.27714","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"55 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328531","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}

{"title":"International Consensus Histopathological Criteria for Subtyping Idiopathic Multicentric Castleman Disease Based on Machine Learning Analysis","authors":"Midori Filiz Nishimura, Tomoka Haratake, Yoshito Nishimura, Asami Nishikori, Remi Sumiyoshi, Hideki Ujiie, Yuri Kawahara, Tomohiro Koga, Masao Ueki, Dorottya Laczko, Eric Oksenhendler, David C. Fajgenbaum, Frits van Rhee, Atsushi Kawakami, Yasuharu Sato","doi":"10.1002/ajh.27743","DOIUrl":"https://doi.org/10.1002/ajh.27743","url":null,"abstract":"Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder classified into three recognized clinical subtypes—idiopathic plasmacytic lymphadenopathy (IPL), TAFRO, and NOS. Although clinical criteria are available for subtyping, diagnostically challenging cases with overlapping histopathological features highlight the need for an improved classification system integrating clinical and histopathological findings. We aimed to develop an objective histopathological subtyping system for iMCD that closely correlates with the clinical subtypes. Excisional lymph node specimens from 94 Japanese iMCD patients (54 IPL, 28 TAFRO, 12 NOS) were analyzed for five key histopathological parameters: germinal center (GC) status, plasmacytosis, vascularity, hemosiderin deposition, and “whirlpool” vessel formation in GC. Using hierarchical clustering, we visualized subgroups and developed a machine learning‐based decision tree to differentiate the clinical subtypes and validated it in an external cohort of 12 patients with iMCD. Hierarchical cluster analysis separated the IPL and TAFRO cases into mutually exclusive clusters, whereas the NOS cases were interspersed between them. Decision tree modeling identified plasmacytosis, vascularity, and whirlpool vessel formation as key features distinguishing IPL from TAFRO, achieving 91% and 92% accuracy in the training and test sets, respectively. External validation correctly classified all IPL and TAFRO cases, confirming the reproducibility of the system. Our histopathological classification system closely aligns with the clinical subtypes, offering a more precise approach to iMCD subtyping. It may enhance diagnostic accuracy, guide clinical decision‐making for predicting treatment response in challenging cases, and improve patient selection for future research. Further validation of its versatility and clinical utility is required.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"45 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144334846","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}

{"title":"Luspatercept in Transfusion‐Dependent β‐Thalassemia: The Benefit Is Real, and So Are the Risks","authors":"Khaled M. Musallam","doi":"10.1002/ajh.27745","DOIUrl":"https://doi.org/10.1002/ajh.27745","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"12 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144319752","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}