Annals of Hematology最新文献

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Correction to: Modern and multidisciplinary care in polycythemia vera 真性红细胞增多症的现代多学科治疗。
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-25 DOI: 10.1007/s00277-026-07207-6
Francesca Palandri, Chiara Sartor, Alessandro Pileri, Olga Addimanda, Christian Gagliardi, Michelangelo Sartori, Monica Benni, Maddalena Giannella, Federica Lo Dato, Elena Sabattini, Lorenzo Fiorino, Alessandra Dedola, Filippo Branzanti, Maria B. Rondinelli, Massimo Reta, Nazzareno Galiè, Pierluigi Viale, Benilde Cosmi, Michelangelo La Placa, Maria C. Morelli, Pier Luigi Zinzani
{"title":"Correction to: Modern and multidisciplinary care in polycythemia vera","authors":"Francesca Palandri, Chiara Sartor, Alessandro Pileri, Olga Addimanda, Christian Gagliardi, Michelangelo Sartori, Monica Benni, Maddalena Giannella, Federica Lo Dato, Elena Sabattini, Lorenzo Fiorino, Alessandra Dedola, Filippo Branzanti, Maria B. Rondinelli, Massimo Reta, Nazzareno Galiè, Pierluigi Viale, Benilde Cosmi, Michelangelo La Placa, Maria C. Morelli, Pier Luigi Zinzani","doi":"10.1007/s00277-026-07207-6","DOIUrl":"10.1007/s00277-026-07207-6","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 8","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13401565/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148590136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Integrated transcriptomics reveals a TNFRSF-metabolism axis and a context-dependent BCMA signaling state in myeloma progression. 整合转录组学揭示了骨髓瘤进展中tnfrsf代谢轴和上下文依赖的BCMA信号状态。
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 DOI: 10.1007/s00277-026-07192-w
Wei-Han Huang, Mohammed Thangameeran Shaik Ismail, Chih-Wen Peng
{"title":"Integrated transcriptomics reveals a TNFRSF-metabolism axis and a context-dependent BCMA signaling state in myeloma progression.","authors":"Wei-Han Huang, Mohammed Thangameeran Shaik Ismail, Chih-Wen Peng","doi":"10.1007/s00277-026-07192-w","DOIUrl":"https://doi.org/10.1007/s00277-026-07192-w","url":null,"abstract":"<p><p>Multiple myeloma (MM) evolves from monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM) with extensive metabolic reprogramming and immune remodeling. We investigated whether coordinated activation of the tumor necrosis factor receptor superfamily (TNFRSF) aligns with metabolic state and prognosis across this continuum. We integrated bone-marrow single-cell RNA sequencing from healthy donors, MGUS, SMM, and MM with bulk RNA sequencing from the Multiple Myeloma Research Foundation (MMRF) CoMMpass cohort. At single-cell resolution, per-cell metabolic activity scores increased with disease stage and were highest in malignant plasma cells. TNF/TNFRSF signaling was strongest in MM, and expression of TNFRSF17 (B-cell maturation antigen, BCMA) in plasma cells correlated with metabolic activity (Spearman ρ = 0.56 in MM). In the clinical cohort, a pooled TNFRSF score stratified overall survival (log-rank p < 0.0001), with individual associations for TNFRSF17 and TNFRSF1A/1B. Joint stratification by TNFRSF activation and metabolic activity revealed the poorest outcomes in tumors with low TNFRSF activation but high metabolic activity, indicating that metabolic state contributes to adverse risk in a receptor-context-dependent manner, while TNFRSF captured additional risk not explained by metabolism alone. When focusing specifically on BCMA, the prognostic ordering across BCMA x metabolism strata was not identical to the pooled TNFRSF model, underscoring that metabolic risk is modulated by the receptor state rather than acting as a universal driver. These findings define an integrated TNFRSF-metabolism axis and a context-dependent BCMA state in advanced MM, clinically relevant but adjustment-sensitive survival associations that warrant further validation and combination strategies that explicitly consider metabolic status.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149226906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Long-term hydroxyurea therapy alters bone marrow megakaryocyte morphology and associates with prognosis in myeloproliferative neoplasms. 长期羟基脲治疗可改变骨髓巨核细胞形态并影响骨髓增生性肿瘤的预后。
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 DOI: 10.1007/s00277-026-07202-x
Zhikang Zheng, Yingqing Xu, Tian Zeng, Rumeng Li, Bingbing Wen, Qingmei Han, Xiaojing Bao, Jian Huang
{"title":"Long-term hydroxyurea therapy alters bone marrow megakaryocyte morphology and associates with prognosis in myeloproliferative neoplasms.","authors":"Zhikang Zheng, Yingqing Xu, Tian Zeng, Rumeng Li, Bingbing Wen, Qingmei Han, Xiaojing Bao, Jian Huang","doi":"10.1007/s00277-026-07202-x","DOIUrl":"https://doi.org/10.1007/s00277-026-07202-x","url":null,"abstract":"<p><p>Hydroxyurea (HU) is a first-line treatment for myeloproliferative neoplasms (MPNs), but a quantitative, standardized framework for assessing its impact on megakaryocyte (MK) morphology and prognosis remains lacking. This retrospective, two-center study analyzed 22,097 MKs from 280 patients (118 polycythemia vera [PV], 162 essential thrombocythemia [ET]), classified into 12 subtypes using the 5th edition WHO classification in conjunction with objectively derived thresholds (minimum P-value approach). Our primary contribution is to establish a quantifiable scoring framework that translates descriptive WHO categories into reproducible prognostic metrics. Using this framework, HU significantly reduced normal MK proportion in JAK2-ET (P = 0.0189), TN-ET (P = 0.0233), and JAK2-PV (P = 0.0427), while increasing abnormal subtypes (bulbous, separated nuclear MKs); conversely, IFN was associated with preserved normal MKs and reduced abnormal forms. Multivariate Cox regression, applied to our empirically derived cutoffs, identified protective thresholds (normal MK ≥ 58% in ET, ≥ 53% in PV; emperipolesis ≥ 2% in ET) and adverse cutoffs (Naked nuclei ≥ 13% in ET, ≥ 7% in PV; staghorn like ≥ 6% in ET; separated nuclear ≥ 3% in ET) for myelofibrosis progression (all P < 0.05). Our quantitative findings corroborate prior qualitative observations that long-term HU is associated with adverse MK remodeling, while IFN correlates with lower progression risk as captured by our threshold-based system. This standardized scoring approach-not the identification of new prognostic factors-provides a reproducible tool for monitoring treatment-related morphological changes and may inform risk-adapted therapy in MPN patients.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149226927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
A rare case of acute basophilic leukemia transformed from high risk myelodysplastic syndrome. 急性嗜碱性白血病由高危骨髓增生异常综合征转化为一例。
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 DOI: 10.1007/s00277-026-07206-7
Christian Aledia Gallardo, Yu Yue Hew, Wei-Ying Jen, Shanmugam Hemalatha, Xiao Wei Ang, Pik Wan Erica Chiang, Kian Guan Eric Lim, Jia En Iris Leow, Shir Ying Lee, Bingwen Eugene Fan
{"title":"A rare case of acute basophilic leukemia transformed from high risk myelodysplastic syndrome.","authors":"Christian Aledia Gallardo, Yu Yue Hew, Wei-Ying Jen, Shanmugam Hemalatha, Xiao Wei Ang, Pik Wan Erica Chiang, Kian Guan Eric Lim, Jia En Iris Leow, Shir Ying Lee, Bingwen Eugene Fan","doi":"10.1007/s00277-026-07206-7","DOIUrl":"https://doi.org/10.1007/s00277-026-07206-7","url":null,"abstract":"<p><p>Acute basophilic leukemia (ABL) transformed from myelodysplastic syndrome is exceedingly rare. We report the case of a 73-year-old man with ABL which transformed from IPSS-M High risk MDS. The patient had a history of metastatic castration-sensitive prostate cancer. Further investigations demonstrated circulating blasts on peripheral blood smear. Upon bone marrow examination, a diagnosis of ABL arising from underlying MDS was established, characterized by cytogenetic analysis demonstrating trisomy 8 (+ 8), with the additional copy of chromosome 8 present in the form of a ring chromosome, and a WT1 frameshift mutation. The patient was treated with azacitidine and venetoclax, followed by best supportive care after disease progression. This case highlights the rare occurrence of ABL secondary to MDS and provides insight into the diagnostic challenges, clonal evolution, and therapeutic limitations associated with this aggressive entity.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Ferritin and LDH predict thrombotic risk beyond conventional scoring systems in DLBCL. 铁蛋白和LDH在DLBCL中预测血栓形成风险超出常规评分系统。
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 DOI: 10.1007/s00277-026-07201-y
Buğra Sağlam, Murat Albayrak, Abdulkerim Yıldız, Pınar Tığlıoğlu, Mesut Tığlıoğlu, Merih Reis Aras, Fatma Yılmaz, Hatice Berna Afacan Öztürk
{"title":"Ferritin and LDH predict thrombotic risk beyond conventional scoring systems in DLBCL.","authors":"Buğra Sağlam, Murat Albayrak, Abdulkerim Yıldız, Pınar Tığlıoğlu, Mesut Tığlıoğlu, Merih Reis Aras, Fatma Yılmaz, Hatice Berna Afacan Öztürk","doi":"10.1007/s00277-026-07201-y","DOIUrl":"https://doi.org/10.1007/s00277-026-07201-y","url":null,"abstract":"<p><p>Thrombotic events are clinically significant complications in diffuse large B-cell lymphoma (DLBCL) that adversely affect treatment continuity and outcomes. Despite their importance, no standardized predictive approach exists, and traditional cancer-associated thrombosis models perform suboptimally in hematological malignancies. We retrospectively analyzed 133 adult patients with DLBCL treated at a single center between 2009 and 2021. Thrombotic events required objective radiological confirmation. Baseline demographic, clinical, and laboratory parameters were compared between patients with and without thrombosis. ROC analysis determined the optimal ferritin cutoff, and Cox proportional hazards and Fine-Gray competing-risk models (with death as a competing event) identified independent predictors. Thrombosis occurred in 16 patients (12.0%), all venous. Baseline clinical characteristics and the Khorana score did not differ between groups. Patients with thrombosis had significantly higher ferritin (median 331.5 vs. 113 ng/mL; p = 0.026) and LDH (median 459.5 vs. 256 U/L; p = 0.022). ROC analysis identified a ferritin cutoff of ≥ 227 ng/mL (AUC 0.686, 95% CI 0.532-0.840), associated with a 6.1-fold increased risk (OR 6.10, 95% CI 1.75-21.28). In multivariate Cox regression, ferritin ≥ 227 ng/mL (HR 3.77, 95% CI 1.13-12.60, p = 0.031) was an independent predictor and remained significant in competing-risk analysis (sHR 4.68); LDH was significant in Cox analysis (HR 1.002 per unit increase, p = 0.034) but was attenuated in the competing-risk model. In DLBCL, where no standardized thrombosis risk-stratification approach exists, elevated ferritin at diagnosis independently predicted thrombotic events, while LDH was significant in univariate analysis. These readily available parameters may contribute to lymphoma-specific thrombosis scoring systems and warrant prospective validation.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Defining and managing high-risk acute myeloid leukemia (AML) in 2026 2026年高危急性髓性白血病(AML)的定义和管理。
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 Epub Date: 2026-09-14 DOI: 10.1007/s00277-026-07142-6
Mohamed Nazem Alibrahim, Mariem Ahmad Elsayed, Abdullah Nassir Alqahtani, Fahad Saeed Alshehri
{"title":"Defining and managing high-risk acute myeloid leukemia (AML) in 2026","authors":"Mohamed Nazem Alibrahim,&nbsp;Mariem Ahmad Elsayed,&nbsp;Abdullah Nassir Alqahtani,&nbsp;Fahad Saeed Alshehri","doi":"10.1007/s00277-026-07142-6","DOIUrl":"10.1007/s00277-026-07142-6","url":null,"abstract":"<div><p>Acute myeloid leukemia (AML) remains a highly heterogeneous malignancy in which outcomes are particularly poor for patients classified as having high-risk disease. Traditionally, high-risk AML has been defined by adverse baseline genetic features, including complex cytogenetics, TP53 alterations, and mutations associated with secondary or therapy-related disease. However, this static, genetics-centered definition is increasingly insufficient in the modern therapeutic era. Emerging evidence supports a more dynamic and context-dependent model in which risk is shaped not only by molecular architecture but also by treatment intensity, patient fitness, measurable residual disease (MRD), and evolving resistance mechanisms. Advances in genomic profiling have refined risk stratification frameworks, including ELN 2022 for intensively treated patients and the ELN 2024 classification for those receiving less-intensive therapies. In parallel, MRD has emerged as a powerful biomarker that reclassifies patients during treatment, identifying those with persistent, therapy-resistant disease despite morphologic remission. Biologically, high-risk AML is driven by the interplay of clonal evolution, epigenetic plasticity, leukemic stem cell persistence, and protective microenvironmental and immune interactions, all of which contribute to relapse. Therapeutically, the landscape has expanded to include targeted agents, venetoclax-based combinations, and transplantation strategies, yet outcomes remain limited in key high-risk subsets, particularly TP53-mutated disease and post-venetoclax relapse. Accordingly, current strategies emphasize rational combination therapies, MRD-guided treatment adaptation, and approaches targeting both leukemic cells and their supportive niches. In 2026, high-risk AML is best understood as a dynamic, treatment-context-dependent state. Improving outcomes will require integration of precision diagnostics, biologically informed therapy, and adaptive strategies designed to anticipate and overcome resistance.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13574891/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148966258","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Analysis of the diagnostic efficacy of hemoglobin capillary electrophoresis for thalassemia in children under 2 years old in Southern Fujian (Minnan) Region, China. 闽南地区2岁以下儿童血红蛋白毛细管电泳对地中海贫血的诊断效果分析
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 DOI: 10.1007/s00277-026-07186-8
Xiaolong Liu, Qianmei Zhuang, Meizhen Yan, Chunqiang Liu, Geng Wang, Baojia Huang
{"title":"Analysis of the diagnostic efficacy of hemoglobin capillary electrophoresis for thalassemia in children under 2 years old in Southern Fujian (Minnan) Region, China.","authors":"Xiaolong Liu, Qianmei Zhuang, Meizhen Yan, Chunqiang Liu, Geng Wang, Baojia Huang","doi":"10.1007/s00277-026-07186-8","DOIUrl":"https://doi.org/10.1007/s00277-026-07186-8","url":null,"abstract":"<p><p>To establish age-specific diagnostic cut-off values for HbF and HbA<sub>2</sub> in children under 2 years in Southern Fujian, China, across α-thalassemia group(carriers and patients),β-thalassemia group(carriers), and a negative control group, and evaluate their diagnostic efficacy for precise thalassemia screening. A retrospective analysis was conducted on 1,072 children under 2 years old. Participants were divided into α-thalassemia group(carriers and patients,331 samples), β-thalassemia group(carriers,220 samples), α+β-thalassemia group(10 samples), and negative control groups (511 samples), and further stratified into three age groups: 0-6 months (172 samples), 7-12 months (532 samples), and 13-24 months (368 samples). ROC curve analysis determined optimal cut-off values for HbF and HbA<sub>2</sub>, with AUC, sensitivity, and specificity calculated. Normal reference ranges were also analyzed. In the negative control group, mean HbA<sub>2</sub> and HbF varied significantly with age. For β-thalassemia, optimal HbA<sub>2</sub> cut-offs were 2.6% (0-6 months, AUC=0.849, sensitivity 80.6%, specificity 85.9%), 3.15% (7-12 months, AUC=0.979, sensitivity 97.9%, specificity 98.8%), and 3.7% (13-24 months, AUC=0.990, sensitivity 98.9%, specificity 98.7%). Optimal HbF cut-offs were 10.1% (0-6 months, AUC=0.720), 4.75% (7-12 months, AUC=0.920), and 2.35% (13-24 months, AUC=0.931). For α-thalassemia, HbA<sub>2</sub>cut-offs showed limited diagnostic value (AUC ≤0.667). HbF and HbA<sub>2</sub> levels are significantly influenced by age. Age-stratified cut-off values for HbA<sub>2</sub>demonstrate excellent diagnostic efficacy for β-thalassemia and can serve as primary screening indicators. For α-thalassemia, HbA<sub>2</sub> measurement alone is insufficient, highlighting the need for complementary testing methods to improve diagnostic accuracy.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149226957","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Addition of thiotepa to total body irradiation-based conditioning in allogeneic hematopoietic stem cell transplantation for relapsed/refractory acute leukemia and lymphoma: a propensity score-matched retrospective study. 在复发/难治性急性白血病和淋巴瘤的异基因造血干细胞移植中,将硫替帕加入到基于全身照射的调节中:一项倾向评分匹配的回顾性研究
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 DOI: 10.1007/s00277-026-07198-4
Minglu Li, Shuqin Zhang, Xiaoyong Man, Hongxia Wen, Wei Wei, Yixin Yang, Jingbo Wang
{"title":"Addition of thiotepa to total body irradiation-based conditioning in allogeneic hematopoietic stem cell transplantation for relapsed/refractory acute leukemia and lymphoma: a propensity score-matched retrospective study.","authors":"Minglu Li, Shuqin Zhang, Xiaoyong Man, Hongxia Wen, Wei Wei, Yixin Yang, Jingbo Wang","doi":"10.1007/s00277-026-07198-4","DOIUrl":"https://doi.org/10.1007/s00277-026-07198-4","url":null,"abstract":"<p><strong>Background: </strong>Relapsed/refractory (R/R) acute leukemia (AL) carries a poor prognosis, with allogeneic hematopoietic stem cell transplantation (allo-HSCT) the primary curative option. Total body irradiation (TBI)-based conditioning is a cornerstone, but optimizing intensity remains challenging. Thiotepa, a bifunctional alkylating agent, may enhance disease control when added to TBI, but data in R/R AL are lacking.</p><p><strong>Methods: </strong>This single-center retrospective study (2020-2025) included R/R AL and lymphoma patients receiving TBI-based conditioning with (TBI + TT) or without (TBI) thiotepa. Propensity score matching (1:2, caliper = 0.2) used 9 covariates. Primary endpoints were overall survival (OS) and disease-free survival (DFS). Secondary endpoints included relapse, non-relapse mortality (NRM), engraftment, GVHD, CMV infection, and mucositis. Kaplan-Meier, Fine-Gray competing risk, and inverse probability of treatment weighting (IPTW) analyses were employed.</p><p><strong>Results: </strong>After matching, 48 TBI + TT and 86 TBI patients were analyzed. OS (HR = 0.901, 95% CI 0.569-1.425, P = 0.655) and DFS (HR = 0.809, 95% CI 0.504-1.299, P = 0.380) were comparable. The 1-year relapse cumulative incidence was lower with TBI + TT (6.7% vs. 17.8%, Gray's P = 0.035; multivariable sHR = 0.280, 95% CI 0.083-0.942, P = 0.040), though hypothesis-generating given only 3 relapse events. NRM was comparable (Gray's P = 0.374). Mucositis was significantly higher with TBI + TT (81.2% vs. 64.0%, P = 0.049). IPTW confirmed comparable OS (P = 0.690) and DFS (P = 0.975).</p><p><strong>Conclusion: </strong>Adding thiotepa to TBI-based conditioning for R/R AL is associated with lower relapse incidence without compromising OS, DFS, engraftment, or GVHD, at the cost of increased mucosal toxicity. The relapse finding requires cautious interpretation and prospective validation.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223186","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Therapeutic Advances in Adult B-cell Acute Lymphoblastic Leukemia with KMT2A Rearrangements 成人b细胞急性淋巴细胞白血病伴KMT2A重排的治疗进展。
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-24 Epub Date: 2026-08-13 DOI: 10.1007/s00277-026-07191-x
Shen-Hao Liu, Ai-Ni Deng, Hui-Ying Li, Kai-Wen Tan, Sheng-Li Xue, Hai-Ping Dai
{"title":"Therapeutic Advances in Adult B-cell Acute Lymphoblastic Leukemia with KMT2A Rearrangements","authors":"Shen-Hao Liu,&nbsp;Ai-Ni Deng,&nbsp;Hui-Ying Li,&nbsp;Kai-Wen Tan,&nbsp;Sheng-Li Xue,&nbsp;Hai-Ping Dai","doi":"10.1007/s00277-026-07191-x","DOIUrl":"10.1007/s00277-026-07191-x","url":null,"abstract":"<div><p>B-cell acute lymphoblastic leukemia (ALL) with <i>KMT2A</i> rearrangements (<i>KMT2A</i>r B-ALL) represents a distinct, high-risk subtype in adults, characterized by aggressive disease biology, high relapse rates, and inferior long-term survival. Conventional intensive chemotherapy, even when consolidated with allogeneic hematopoietic stem cell transplantation (allo-HSCT), yields suboptimal outcomes, underscoring the need for novel therapeutic approaches. In recent years, substantial progress has been made with the introduction of antibody-based immunotherapies, cellular immunotherapies, and small molecular inhibitors, reshaping the treatment landscape for this challenging subgroup. This review provides a comprehensive overview of current and emerging therapeutic strategies for adult patients with <i>KMT2A</i>r B-ALL. We summarize outcomes associated with pediatric-inspired chemotherapy and allo-HSCT, focus on clinical evidence for antibody-based immunotherapy including blinatumomab and inotuzumab ozogamicin, across frontline, consolidation, and relapsed or refractory settings. Advances in cellular immunotherapy, particularly CD19-directed chimeric antigen receptor T-cell therapy, are discussed, with a focus on unique resistance mechanisms such as antigen loss and lineage switch. In addition, we review the biological rationale, efficacy and emerging resistance mechanisms of menin inhibitors, a promising class of agents specifically targeting the epigenetic dependency of <i>KMT2A</i>-rearranged leukemia. Finally, other molecular approaches, including epigenetic modifiers, apoptosis pathway inhibitors, and signaling pathway inhibitors are discussed. Despite these advances, treatment resistance and disease relapse remain major obstacles. These ongoing challenges highlight the urgent need for multi-center, prospective trials to investigate rational combination strategies to improve outcomes for adults with <i>KMT2A</i>r B-ALL.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 9","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07191-x.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148720847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Safety of red blood cell transfusion despite serologic incompatibility in autoimmune hemolytic anemia: A real-world multicenter study. 自身免疫性溶血性贫血血清学不相容时输血红细胞的安全性:一项真实世界的多中心研究
IF 2.3 3区 医学
Annals of Hematology Pub Date : 2026-07-23 DOI: 10.1007/s00277-026-07188-6
Claudia Cammarata, Simona Raso, Marta Mattana, Riccardo Tomasello, Giulia Maria Camarda, Simona D'Angelo, Elisabetta Scirè, Maria Ilaria Dilena, Roberta Fedele, Sergio Siragusa, Mariasanta Napolitano
{"title":"Safety of red blood cell transfusion despite serologic incompatibility in autoimmune hemolytic anemia: A real-world multicenter study.","authors":"Claudia Cammarata, Simona Raso, Marta Mattana, Riccardo Tomasello, Giulia Maria Camarda, Simona D'Angelo, Elisabetta Scirè, Maria Ilaria Dilena, Roberta Fedele, Sergio Siragusa, Mariasanta Napolitano","doi":"10.1007/s00277-026-07188-6","DOIUrl":"10.1007/s00277-026-07188-6","url":null,"abstract":"<p><p>Red blood cell transfusion in Autoimmune Hemolytic Anemia (AIHA) poses significant clinical challenges due to pan-reactive autoantibodies that interfere with cross-matching, often raising concerns regarding the safety of transfusing serologically \"incompatible\" blood. This retrospective multicenter study evaluated the frequency, management criteria, and safety profile of red blood cell transfusions in a cohort of adult patients affected by AIHA with a specific focus on transfusion management in the setting of serologic incompatibility. We analyzed 93 adult patients diagnosed with AIHA at two hematology referral centers in Palermo, Italy, between 2020 and 2025. Transfusion support was required in 31.2% (N = 29) of patients. Decisions were mainly guided by a \"double trigger\" of low hemoglobin (mean 6.3 g/dL) and symptoms of tissue hypoxia, rather than by fixed hemoglobin thresholds. Notably, 61.1% of characterized units were serologically \"incompatible\" due to autoantibodies. Despite this, the adverse reaction rate was extremely low (1.2% per unit), with no hemolytic reactions recorded. This safety profile was likely supported by concomitant immunosuppressive therapy (89.6% of transfused patients), acting as \"biological premedication\". Splenomegaly was identified as a marker for higher transfusion dependence (41.2% vs. 29.5%). Transfusion in AIHA is a safe and effective life-saving measure when managed with appropriate immunohematological oversight. Red blood cell transfusion in AIHA appears safe and effective when clinically indicated and managed with appropriate immunohematologic oversight, even in the presence of persistent serologic incompatibility. Transfusion support should not be delayed solely because of crossmatch incompatibility once clinically significant alloantibodies have been reasonably excluded.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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