American Journal of Hematology最新文献

{"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":"10.1002/ajh.27714","url":null,"abstract":"<p>In the United States, 22 000 splenectomies are performed annually [<span>1</span>] for various conditions. Splenectomy has been linked to overwhelming sepsis and more recently to thromboembolic events (TEE) [<span>2-5</span>]. Danish national registry studies documented the increased occurrence of TEE postsplenectomy; however, earlier studies were restricted to males and later ones, including two Danish studies, did not discriminate TEE in women from those in men [<span>2-4</span>]. TEE risk differs between men and women in several ways [<span>5</span>], so sex-specific assessments are critical. Premenopausal women have a predisposition to TEE for several reasons including hormonal and autoimmune ones; however, TEE risk remains unclear in postmenopausal women. We therefore conducted a prospective cohort study within the Nurses' Health Study (NHS) using biennial questionnaires with medical record confirmation of thromboses to robustly assess association between splenectomy and TEE in postmenopausal women.</p><p>The NHS was established in 1976 and enrolled 121 700 female registered nurses between 30 and 55 years old. Splenectomy history was included on the 2004 questionnaire and 90 853 postmenopausal women (at that time between ages of 58 and 83 years) were included in the current analysis. The biennial questionnaires asked participants whether they had stroke, myocardial infarction (MI), deep vein thrombosis (DVT), or pulmonary emboli (PE) since cohort inception. Participants self-reporting TEE received letters requesting confirmatory medical records. Through 2012, questions about DVT and PE were distinct; subsequently, 2012–2016, participants were asked about both as a single outcome.</p><p>The primary outcomes included DVT, PE, stroke and MI, self-reported on biennial questionnaires. Follow-up for this nested cohort study started in 2004 after all splenectomies. End of follow-up was through the 2016 questionnaire, date of death, or diagnosis of outcome, whichever came first. Loss to follow-up in NHS is < 5% every 2-year cycle. Date and cause of death were obtained through linkage with the National Death Index. We conducted a prospective analyses (start from 2004) with Cox Proportional Hazard models to generate hazard ratios and 95% confidence intervals (CIs). We included the following covariates as potential confounders in multivariate models: (a) continuous variables: age, BMI, hypertension, and cholesterol level; and (b) yes/no variables: smoking status, physical activity, diabetes, and menopausal hormone therapy.</p><p>Biennial questionnaires did not include a specific question about reason for splenectomy. Participants had the option to report these diagnoses under “other illnesses.” They were then mapped to ICD-9 and ICD-10 disease codes including categorizing these conditions into definite ITP (codes = 287.31), and possible ITP (codes = 287.30). Thirty-four participants were considered “ITP,” too few to perform a sub-analysis. The re","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 8","pages":"1459-1462"},"PeriodicalIF":10.1,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.27714","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":"OA","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}

{"title":"Efficacy and Safety of Hetrombopag Versus Thrombopoietin in Promoting Platelet Engraftment After Allogeneic Hematopoietic Stem Cell Transplantation: A Prospective, Multicenter, Randomized Controlled Clinical Trial","authors":"Yimei Feng, Linhui Wang, Mingqiang Ren, Dan Wang, Tao Lang, Jianchuan Deng, Shifeng Lou, Hai Yi, Liangliang Ma, Hongyun Xing, Pengcheng He, Xiaoning Wang, Sanbin Wang, Jishi Wang, Han Yao, Ting Chen, Jia Liu, Lu Zhao, Yuqing Liu, Xiaoqi Wang, Li Li, Shuhan Tang, Lidan Zhu, Jia Liu, Shichun Gao, Huanfeng Liu, Lu Wang, Peiyan Kong, Lei Gao, Xi Zhang","doi":"10.1002/ajh.27746","DOIUrl":"https://doi.org/10.1002/ajh.27746","url":null,"abstract":"Allogeneic hematopoietic stem cell transplantation (allo‐HSCT) is pivotal for hematological malignancies but faces challenges in delayed platelet engraftment, poor graft function (PGF), and bleeding risks. Hetrombopag, a thrombopoietin receptor agonist, was evaluated to enhance platelet recovery posttransplant. Patients undergoing allo‐HSCT were randomized on Day 3 Post‐Infusion into low‐dose (2.5 mg/day), high‐dose (5 mg/day) hetrombopag groups, or a control group receiving thrombopoietin (300 U/kg/day). Endpoints included platelet/neutrophil engraftment time, PGF incidence, transfusion needs, and safety. Among 212 analyzed patients, platelet engraftment was faster in hetrombopag groups (median 13 days) versus controls (15 days; <jats:italic>p</jats:italic> = 0.001), with no dose‐dependent difference (<jats:italic>p</jats:italic> = 0.821). Neutrophil engraftment was also accelerated (13 vs. 15 days; <jats:italic>p</jats:italic> = 0.002). PGF incidence was lower in hetrombopag groups (5.04%) versus controls (13.7%; <jats:italic>p</jats:italic> = 0.042). The experimental group required fewer platelet transfusions (<jats:italic>p</jats:italic> = 0.021), had reduced gastrointestinal bleeding, and lower costs. Secondary platelet recovery failure showed no intergroup differences. Hetrombopag safely accelerates platelet and neutrophil engraftment, reduces PGF risk, and decreases transfusion dependency post‐allo‐HSCT. Low‐dose hetrombopag demonstrated efficacy equivalent to high‐dose, offering a cost‐effective strategy to improve transplant outcomes.Trial Registration: ChiCTR2200057764.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"8 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144319318","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":"MRD and NK‐Cell Chimerism Predict Transplant Outcomes in Allogeneic Hematopoietic Stem Cell Transplantation Recipients","authors":"Xing Chen, Huihong Huang, Weihao Chen, Yeqian Zhao, Chuanhe Jiang, Luxiang Wang, Zengkai Pan, Jiayu Huang, Zilu Zhang, Haiyang Lu, Yan Sheng, Xiangqin Weng, Yanmin Zhao, Yang Cao, Xiaoxia Hu","doi":"10.1002/ajh.27750","DOIUrl":"https://doi.org/10.1002/ajh.27750","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"42 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144319750","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":"Comparison of Frontline Therapies in Older Adults Age ≥ 80 Years With Chronic Lymphocytic Leukemia (CLL): A Mayo Clinic and Danish Nation-Wide Study","authors":"Mazie Tsang, Paul J. Hampel, Kari G. Rabe, Christian Brieghel, Wei Ding, Jose F. Leis, Talal Hilal, Saad S. Kenderian, Yucai Wang, Eli Muchtar, Amber B. Koehler, Daniel L. Van Dyke, Curtis A. Hanson, Min Shi, Susan L. Slager, Neil E. Kay, Henrik Hjalgrim, Carsten U. Niemann, Sameer A. Parikh, Emelie C. Rotbain","doi":"10.1002/ajh.27747","DOIUrl":"https://doi.org/10.1002/ajh.27747","url":null,"abstract":"&lt;p&gt;People with chronic lymphocytic leukemia (CLL) have a median age at diagnosis of around 70 years [&lt;span&gt;1&lt;/span&gt;]. With an average time to first treatment of 4–5 years from diagnosis, it is estimated that a third of all people with CLL will be ≥ 80 years old when they become symptomatic and receive frontline therapy [&lt;span&gt;1&lt;/span&gt;]. People ≥ 80 years old often have comorbidities and unfavorable disease characteristics (like unmutated IGHV and 17p deletion) associated with lower survival [&lt;span&gt;2&lt;/span&gt;]. Despite efforts to include advanced age in the eligibility criteria, landmark CLL clinical trials that led to the approval of ibrutinib and venetoclax primarily included participants &lt; 75 years old, making it challenging to apply their findings to older patients. To help close this knowledge gap, we conducted a multi-center retrospective cohort study with data spanning nearly three decades to describe treatment outcomes for patients treated for CLL in the frontline setting at ages ≥ 80 years.&lt;/p&gt;\u0000&lt;p&gt;Using the Mayo Clinic CLL Database and the Danish Lymphoid-lineage Cancer Research (DALY-CARE) data (Danish Cohort), we conducted a retrospective cohort study of patients who were ≥ 80 years old at the time of first CLL treatment between January 1995 and November 2022 (Mayo Clinic) and January 2008 and March 2022 (Denmark). The Mayo Clinic CLL Database contains records of consenting patients diagnosed with CLL who were seen at Mayo Clinic in Rochester, MN. The Danish Cohort includes all Danish citizens diagnosed with CLL, with data available from time of birth or immigration, enabled by linkage of personal-level data across national administrative and clinical quality registers using a unique personal identification number. We extracted information about clinical characteristics, patient demographics, and all lines of treatment (Table S1). We calculated overall survival (OS) from the date of first treatment to the date of death or date last known to be alive. OS was displayed using the Kaplan–Meier method. Time to next treatment (TTNT) was calculated from the date of first treatment to earliest date of (a) date of second treatment, (b) date of death, or (c) date last known to be alive. TTNT was visualized using the cumulative incidence method with competing risk of death. Univariable Cox proportional hazards models were used to analyze the association between type of treatment and time to events (i.e., OS or TTNT); results were reported as hazard ratios (HR) and 95% confidence intervals (CI). Analyses were conducted using statistical software SAS 9.4 (SAS Institute Inc., Cary, NC, USA) and R statistical software versions 4.2.0 and 4.3.2. The Mayo Clinic Institutional Review Board and the Danish Health Data Authority and National Ethics Committee (approvals P-2020-561 and 1804410, respectively) approved this study.&lt;/p&gt;\u0000&lt;p&gt;We identified a total of 653 patients aged ≥ 80 years at the time of frontline CLL treatment (216 from Mayo Clinic and 437 fr","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"35 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144311848","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":"Hereditary Elliptocytosis Resulting From Heterozygosity for β Spectrin Tandil.","authors":"María-Angustias Molina-Arrebola,Barbara J Bain","doi":"10.1002/ajh.27744","DOIUrl":"https://doi.org/10.1002/ajh.27744","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"232 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144311575","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":"Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): 2025 Update on Diagnosis, Pathophysiology, Risk Assessment, and Management","authors":"Shai Shimony,&nbsp;Marlise R. Luskin,&nbsp;Naseema Gangat,&nbsp;Nicole R. LeBoeuf,&nbsp;Angela M. Feraco,&nbsp;Andrew A. Lane","doi":"10.1002/ajh.27737","DOIUrl":"10.1002/ajh.27737","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Overview</h3>\u0000 \u0000 <p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic neoplasm that involves multiple organs, including the skin, bone marrow and blood, lymph nodes, and central nervous system. Violaceous tumors or plaques appearing in sun-exposed areas are characteristic and present in most patients. The disease typically affects adult men aged 60–70 years, although children and younger adults may also be affected.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Diagnosis</h3>\u0000 \u0000 <p>Diagnosis is based on biopsy of an involved site and is typically based on the identification of blastoid cells displaying the classical immunophenotypes CD123, CD4, and CD56 in addition to specific pDC markers.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Prognosis</h3>\u0000 \u0000 <p>The median overall survival is 18–24 months. Potential risk factors for shortened survival include older age, overt bone marrow involvement, and genetic abnormalities.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Therapy and Management</h3>\u0000 \u0000 <p>Either chemotherapy-based regimens or tagraxofusp, a CD123-directed interleukin 3 conjugated with diphtheria toxin, may be used for upfront therapy. Eligible patients are recommended to be consolidated with hematopoietic allogeneic stem cell transplantation. Assessment and treatment of the central nervous system is mandatory, and careful monitoring of skin disease in conjunction with dermatology is essential.</p>\u0000 </section>\u0000 </div>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 8","pages":"1408-1422"},"PeriodicalIF":10.1,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.27737","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144305171","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Digital Necrosis as Presenting Symptom of Polycythemia Vera","authors":"Charly Edmiston, Brett J. Carroll, Jason A. Freed","doi":"10.1002/ajh.27742","DOIUrl":"https://doi.org/10.1002/ajh.27742","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"12 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144290049","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}