Annals of Hematology最新文献

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Refinement of intermediate-risk Karyotypes according to the IPSS-R in patients with myelodysplastic neoplasms (MDS). 骨髓增生异常肿瘤(MDS)患者中危核型的IPSS-R改进。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-06-09 DOI: 10.1007/s00277-025-06443-6
K Nachtkamp, F Schulz, A Kasprzak, C Strupp, B Hildebrandt, M Pfeilstöcker, P Valent, B Blum, A Giagounidis, K Götze, V Flatten, S Dietrich, G Kobbe, D Haase, N Gattermann, U Germing
{"title":"Refinement of intermediate-risk Karyotypes according to the IPSS-R in patients with myelodysplastic neoplasms (MDS).","authors":"K Nachtkamp, F Schulz, A Kasprzak, C Strupp, B Hildebrandt, M Pfeilstöcker, P Valent, B Blum, A Giagounidis, K Götze, V Flatten, S Dietrich, G Kobbe, D Haase, N Gattermann, U Germing","doi":"10.1007/s00277-025-06443-6","DOIUrl":"10.1007/s00277-025-06443-6","url":null,"abstract":"<p><p>MDS patients show a heterogenous prognosis which can be stratified by the IPSS-R in order to derive therapeutic implications. Based on 618 patients with myelodysplastic neoplasms belonging to the cytogenetic intermediate-risk group according to IPSS-R, we show that this group is heterogeneous in terms of overall survival and cumulative risk of AML. The group can be reorganized into subgroups according to their prognostic impact. A small subgroup of patients with isolated -X or der(1;7) can be regarded as very-low-risk patients with a median survival time of 112 months and a cumulative AML progression rate of 9% after 2 years. A larger group of patients with either diverse aberrations of one chromosome or -Y + one additional aberration shows a benign course of the disease with a median survival time of 46 months and a cumulative AML progression rate of 26% after 2 years. A very large group of patients presenting with either + 8, +19, i(17q), + 21, +mar, del(9q), + 8 plus one other aberration, or del(7q) have a poor prognosis with a median survival time of 26 months and a cumulative AML progression rate of 32% after 2 years. In a very small set of patients with trisomy 11 the course of disease was similar to very-high-risk patients with a median survival time of 17 months only and a cumulative AML progression rate of 100% after 2 years. These findings could lead to a refinement of prognostic scoring systems such as the IPSS-R and the IPSS-M.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3251-3259"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283795/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144246098","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
Impact of ETV6 mutations on clinical outcomes in acute myeloid leukemia: a real-world retrospective cohort study. ETV6突变对急性髓系白血病临床结局的影响:一项真实世界的回顾性队列研究
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-06-21 DOI: 10.1007/s00277-025-06451-6
Xueqian Li, Hong Wang, Haohao Han, Jiaqian Qi, Yifang Yao, Xiaoyan Xu, Tingting Pan, Depei Wu, Yue Han
{"title":"Impact of ETV6 mutations on clinical outcomes in acute myeloid leukemia: a real-world retrospective cohort study.","authors":"Xueqian Li, Hong Wang, Haohao Han, Jiaqian Qi, Yifang Yao, Xiaoyan Xu, Tingting Pan, Depei Wu, Yue Han","doi":"10.1007/s00277-025-06451-6","DOIUrl":"10.1007/s00277-025-06451-6","url":null,"abstract":"<p><p>As a member of the ETS family, ETV6 has been demonstrated to be implicated with the molecular etiology of various hematopoietic diseases. However, the clinical impact of ETV6 mutations (ETV6<sup>mut</sup>) in acute myeloid leukemia (AML) remains unclear. Hereon, we included 879 consecutive newly diagnosed AML patients in our institution to elucidate the prognostic impact of ETV6 mutation status. In the overall cohort, ETV6<sup>mut</sup> were found in 24 cases (2.7%) and were associated with lower hemoglobin levels. The predominant common mutation types were missense mutations (15/31, 48.4%) and frameshift mutations (14/31, 45.2%). ETV6 mutations often occurred in conjunction with U2AF1 and ASXL1 mutations. Moreover, ETV6<sup>mut</sup> was associated with a lower complete remission (CR) rate (45.8% vs. 69.1%, P = 0.015) and shorter OS (P = 0.048) compared to the ETV6 wild-type (ETV6<sup>wt</sup>) group. Notably, the achievement of CR did not contribute to survival benefit in AML with ETV6<sup>mut</sup>. In multivariate analysis, ETV6 mutation was shown to be an independent adverse factor for OS in AML patients (HR: 1.72, 95% CI: 1.03-2.89; P = 0.040). Taken together, our study shows a mutational profile of ETV6 in AML and suggests that ETV6 mutations are associated with poor prognosis in AML patients.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3271-3279"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283831/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144339854","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
Efficacy and safety of rabbit ATLG and ATG in allogeneic hematopoietic stem cell transplantation for children with acquired severe aplastic anemia. 兔ATLG和ATG在儿童获得性重度再生障碍性贫血异基因造血干细胞移植中的疗效和安全性。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-06-19 DOI: 10.1007/s00277-025-06461-4
Senlin Zhang, Qi Ji, Li Gao, Qingwei Wang, Kai Cui, Minyuan Liu, Bohan Li, Yixin Hu, Yongping Zhang, Yuanyuan Tian, Shengqin Cheng, Jun Lu, Shaoyan Hu
{"title":"Efficacy and safety of rabbit ATLG and ATG in allogeneic hematopoietic stem cell transplantation for children with acquired severe aplastic anemia.","authors":"Senlin Zhang, Qi Ji, Li Gao, Qingwei Wang, Kai Cui, Minyuan Liu, Bohan Li, Yixin Hu, Yongping Zhang, Yuanyuan Tian, Shengqin Cheng, Jun Lu, Shaoyan Hu","doi":"10.1007/s00277-025-06461-4","DOIUrl":"10.1007/s00277-025-06461-4","url":null,"abstract":"<p><p>Severe aplastic anemia (SAA) is a life-threatening bone marrow failure syndrome characterized by bone marrow hypoplasia and peripheral blood cytopenia. Without timely treatment, it frequently proves fatal. Rabbit anti-thymocyte globulin (ATG) and anti-human T lymphocyte globulin (ATLG) are widely used for graft-versus-host disease (GVHD) prophylaxis. However, their comparative efficacy in pediatric SAA remains undetermined. This study involved a single-center retrospective analysis of two ATG preparations in pediatric patients undergoing allo-HSCT. The primary endpoint was the incidence of GVHD and viral reactivation following HSCT. Secondary endpoints included overall survival (OS), GVHD-free and failure-free survival (GFFS), neutrophil engraftment, platelet engraftment, hemorrhagic cystitis (HC), tolerability, and toxicities within each group. A total of 124 pediatric SAA patients who underwent their first allo-HSCT between January 2019 and March 2024 were enrolled, with 35 receiving ATLG and 89 receiving ATG. OS, GFFS, GVHD, and HC incidence were comparable between the ATLG and ATG groups (OS: 95.2% vs. 92.9%, P = 0.617). ATLG significantly reduced the incidence of 180-day CMV (45.7% vs. 74.2%, P = 0.0062) and EBV reactivation (29.8% vs. 52.8%, P = 0.025). Additionally, ATLG was associated with fewer adverse events (AEs), including fever (P = 0.009) and rash (P = 0.018). ATLG demonstrated comparable efficacy to ATG in preventing GVHD and achieving OS in pediatric SAA patients undergoing allo-HSCT, while significantly reducing viral reactivation and AEs. These findings support ATLG as a safer alternative, warranting further prospective studies.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3153-3163"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283839/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324352","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
Inborn errors of immunity presenting with lymphoproliferation: lessons from a case series. 以淋巴细胞增生为表现的先天性免疫错误:来自一系列病例的经验教训。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-06-24 DOI: 10.1007/s00277-025-06456-1
Giorgio Costagliola, Emanuela De Marco, Filippo Consonni, Valeria Rocchi, Annalisa Legitimo, Mariacristina Menconi, Eleonora Gambineri, Gabriella Casazza, Rita Consolini
{"title":"Inborn errors of immunity presenting with lymphoproliferation: lessons from a case series.","authors":"Giorgio Costagliola, Emanuela De Marco, Filippo Consonni, Valeria Rocchi, Annalisa Legitimo, Mariacristina Menconi, Eleonora Gambineri, Gabriella Casazza, Rita Consolini","doi":"10.1007/s00277-025-06456-1","DOIUrl":"10.1007/s00277-025-06456-1","url":null,"abstract":"<p><p>Lymphoproliferation can represent the first or leading disease sign in different inborn errors of immunity (IEI), which include autoimmune lymphoproliferative syndrome (ALPS), common variable immunodeficiency (CVID), combined immunodeficiencies, activated phosphoinositide 3-kinase δ syndrome (APDS), Epstein-Barr (EBV)-related disorders, and others. The genetic and molecular background and the clinical implications of IEI presenting with lymphoproliferation are partly unexplored. Therefore, the diagnosis and clinical management of this category of patients is particularly challenging. As treatments targeting the specific genetic defect are available for some of the IEIs associated with lymphoproliferation, identifying the molecular diagnosis is of great clinical relevance and could significantly improve the patient's long-term outcome. To this purpose, a first-level immunological assessment is strongly recommended in patients with persistent or recurrent unexplained lymphoproliferation, and specific second-level analysis can orient towards the correct clinical suspicion. The mechanisms responsible for polyclonal, benign lymphoproliferation in IEI also cause an increased susceptibility to clonal, malignant lymphoproliferation. Therefore, a careful follow-up is recommended in all the patients with IEI and polyclonal lymphoproliferation, to promptly identify the development of clonality. Moreover, as malignant lymphoproliferation can be the first disease sign of some IEI, there is increasing interest in the possibility of recognizing IEIs in patients presenting with lymphoid malignancies. This work, by describing some relevant case studies, reviews the role of lymphoproliferative features in three of the most paradigmatic conditions, particularly ALPS, CVID, and APDS, and provides an updated discussion on the diagnosis, treatment, and follow-up of patients with IEI and lymphoproliferative features.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3117-3127"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283836/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144473889","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
Germline mutations predispose a concurrent thymoma and diffuse large B-cell lymphoma. 种系突变易导致并发胸腺瘤和弥漫性大b细胞淋巴瘤。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-05-06 DOI: 10.1007/s00277-025-06322-0
Xiaoyan Fu, Qiao Jiang, Wenbin Mo, Jinjing Zhang, Yan Li, Yuan Miao, Rui Zhang
{"title":"Germline mutations predispose a concurrent thymoma and diffuse large B-cell lymphoma.","authors":"Xiaoyan Fu, Qiao Jiang, Wenbin Mo, Jinjing Zhang, Yan Li, Yuan Miao, Rui Zhang","doi":"10.1007/s00277-025-06322-0","DOIUrl":"10.1007/s00277-025-06322-0","url":null,"abstract":"<p><p>Although thymoma is a rare malignancy, it is usually accompanied by secondary cancers. Non-Hodgkin's B-cell lymphoma is one of the most common secondary neoplasms observed in patients with thymoma, presenting before, concurrently with, or after the diagnosis. However, the underlying molecular mechanism of concomitant thymoma and lymphoma remains unclear. This study aimed to report a case with concurrent extrathymic, diffuse large B-cell lymphoma (DLBCL), and thymoma. Published studies and the SEER database were queried to summarize the features of patients with these concomitant cancers. Whole-exome sequencing (WES) was performed on tumor specimens and buccal swab mucosa. Six germline mutations and several specific somatic alterations were found on each neoplastic tissue, which may elucidate the potential pathogenesis of concurrent cancers. This study was novel in reporting concurrent extrathymic DLBCL and thymoma by applying WES on matched neoplasm-normal samples to explore the pathogenesis of these distinct neoplasms.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3511-3516"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283472/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143952406","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
Intrathecal rituximab in pediatric CD20-positive central nervous system limited lymphoproliferative disease: report of two cases and literature review. 鞘内利妥昔单抗治疗小儿cd20阳性中枢神经系统有限淋巴细胞增生性疾病2例报告并文献复习
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-05-05 DOI: 10.1007/s00277-025-06386-y
Huixia Gao, Ningning Zhang, Chunju Zhou, Ling Jin, Jing Yang, Shuang Huang, Meng Zhang, Nan Li, Tianyou Wang, Yanlong Duan
{"title":"Intrathecal rituximab in pediatric CD20-positive central nervous system limited lymphoproliferative disease: report of two cases and literature review.","authors":"Huixia Gao, Ningning Zhang, Chunju Zhou, Ling Jin, Jing Yang, Shuang Huang, Meng Zhang, Nan Li, Tianyou Wang, Yanlong Duan","doi":"10.1007/s00277-025-06386-y","DOIUrl":"10.1007/s00277-025-06386-y","url":null,"abstract":"<p><p>Isolated central nervous system (CNS) involvement due to posttransplantation proliferative disorder (PTLD) is even rarer, with only a few cases reported in the literature. CNS involvement in patients with mature B-cell non-Hodgkin's (NHL) and PTLD confers a significantly worse prognosis as compared to patients without CNS lymphoma disease. Treatment of CNS lymphoma (CNSL) is challenging due to resistance to conventional cytotoxic and intrathecal chemotherapy. Here, we report the successful use of intrathecal rituximab in two pediatric cases of CD20 + isolated CNSL that had failed to respond to standard chemotherapy, intravenous rituximab and Epstein-Barr virus (EBV)-specific cellular therapy. However, after repeated intrathecal administration of rituximab, both patients' clinical symptoms were alleviated, which has created opportunities for further treatments. We emphasise that intrathecal rituximab may be a safe and promising strategy for the treatment of pediatric patients with CD20 + isolated CNSL. The literature on this topic was also reviewed.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3487-3494"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283754/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143966675","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
The effect of impaired renal function, increased red cell mass and plasma volume on thrombotic risk in PV patients. 肾功能受损、红细胞质量和血浆容量增加对PV患者血栓形成风险的影响。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-05-23 DOI: 10.1007/s00277-025-06348-4
Marko Lucijanic, Danijela Lekovic, Andrija Bogdanovic, Ivan Krecak
{"title":"The effect of impaired renal function, increased red cell mass and plasma volume on thrombotic risk in PV patients.","authors":"Marko Lucijanic, Danijela Lekovic, Andrija Bogdanovic, Ivan Krecak","doi":"10.1007/s00277-025-06348-4","DOIUrl":"10.1007/s00277-025-06348-4","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3527-3529"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283811/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144126168","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
Bleeding prediction scores in patients with venous thromboembolism using direct oral anticoagulants. 静脉血栓栓塞患者直接口服抗凝剂的出血预测评分。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-06-02 DOI: 10.1007/s00277-025-06434-7
Guilan Wu, Jiana Chen, Yunda Chen, Peiguang Niu, Sijie Chang, Fuxin Ma, Chunhua Wang, Xiangsheng Lin, Xiumei Liu, Jun Su, Hengfen Dai, Yuxin Liu, Jinhua Zhang
{"title":"Bleeding prediction scores in patients with venous thromboembolism using direct oral anticoagulants.","authors":"Guilan Wu, Jiana Chen, Yunda Chen, Peiguang Niu, Sijie Chang, Fuxin Ma, Chunhua Wang, Xiangsheng Lin, Xiumei Liu, Jun Su, Hengfen Dai, Yuxin Liu, Jinhua Zhang","doi":"10.1007/s00277-025-06434-7","DOIUrl":"10.1007/s00277-025-06434-7","url":null,"abstract":"<p><p>We aimed to identify factors associated with bleeding in patients with venous thromboembolism (VTE) when using direct oral anticoagulants (DOACs), to construct and externally validate a predictive model for bleeding, and to provide a validated tool for clinical assessment of bleeding. The prediction model in the development cohort was constructed using logistic regression and visualized through a Nomogram. We conducted external validation of the model. The accuracy and calibration were evaluated through the area under the curve (AUC), calibration curves, and the Hosmer-Lemeshow test. This multicenter retrospective study recruited 1121 patients treated with DOACs for VTE. The training set consisted of 806 patients from 11 centers and the external validation set consisted of 315 patients from 9 centers. The Alfalfa-VTE-Major model (AUC = 0.883) is composed of five variables: Age ≥ 65 years, history of bleeding, malignancy, and coadministration of antiplatelet drugs/nonsteroidal antiinflammatory drugs (NSAIDs) were independent risk factors for major bleeding, and coadministration of gastrointestinal protectants was a protective factor. The Alfalfa-VTE-Minor model (AUC = 0.875) was composed of five independent risk factors for minor bleeding variables: Age ≥ 65 years, anemia, history of bleeding, vascular disease, and coadministration of antiplatelet drugs/NSAIDs. We externally validate the currently widely used VTE bleeding model. The predictive power of the three bleeding scores was RIETE (AUC = 0.773), VTE-BLEED (AUC = 0.746), and Hokusai (AUC = 0.558) in descending order. We constructed and externally validated a predictive model for the occurrence of major and minor bleeding in VTE patients using DOACs. Both models have good predictive value and may be effective tools to help reduce the incidence of bleeding in patients with DOACs.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3477-3486"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283757/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144198086","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
Epidemiology and survival of patients with primary lymphoma of bone: a large retrospective cohort study. 原发性骨淋巴瘤患者的流行病学和生存率:一项大型回顾性队列研究。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-05-24 DOI: 10.1007/s00277-025-06413-y
Xiaojie Liang, Weixiang Lu, Tong Li, Baiwei Luo, Yuzhe Wu, Chaoran Lin, Yang Liu, Liang Wang
{"title":"Epidemiology and survival of patients with primary lymphoma of bone: a large retrospective cohort study.","authors":"Xiaojie Liang, Weixiang Lu, Tong Li, Baiwei Luo, Yuzhe Wu, Chaoran Lin, Yang Liu, Liang Wang","doi":"10.1007/s00277-025-06413-y","DOIUrl":"10.1007/s00277-025-06413-y","url":null,"abstract":"<p><p>Primary lymphoma of bone (PLB) is a rare extranodal lymphoma, and its epidemiology and prognosis remain controversial. We conducted a retrospective analysis of 1,222 patients with PLB in the Surveillance, Epidemiology, and End Results (SEER) database to investigate its epidemiology and prognostic factors. The incidence of PLB peaked in 1992 with an average annual percent change of 1.72 after a significant rise from 1975 to 1992, followed by a general decline. The risk of death from PLB involves both patient and treatment factors. Survival analysis revealed that age, stage, laterality, chemotherapy, and primary site significantly influence both overall survival and disease-specific survival. We integrated and compared 99 machine learning algorithms, and identified the Random Survival Forest (RSF) model as the most effective for predicting PLB outcomes. Patients were stratified into low- and high-risk groups according to the RSF model score. The incidence of PLB began to decrease after 1992, with variations by age, race, and gender. The factors influencing the prognosis of PLB are multifaceted. And the RSF model showed promising performance, aiding clinicians in early prognosis identification and improving clinical outcomes through revised management strategies and patient care.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3345-3358"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283789/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144135935","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
Efficacy and prognosis of allogeneic hematopoietic stem cell transplantation in aggressive NK-cell leukemia: a meta-analysis. 异体造血干细胞移植治疗侵袭性nk细胞白血病的疗效和预后:一项荟萃分析。
IF 3 3区 医学
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-06-05 DOI: 10.1007/s00277-025-06423-w
Shuya Cao, Chaoxia Cao, Suyun Wang
{"title":"Efficacy and prognosis of allogeneic hematopoietic stem cell transplantation in aggressive NK-cell leukemia: a meta-analysis.","authors":"Shuya Cao, Chaoxia Cao, Suyun Wang","doi":"10.1007/s00277-025-06423-w","DOIUrl":"10.1007/s00277-025-06423-w","url":null,"abstract":"<p><p>Aggressive NK-cell leukemia (ANKL) is a rare and highly malignant lymphoproliferative disorder associated with poor prognosis and high mortality rates. The disease is closely linked to Epstein-Barr virus (EBV) and predominantly affects populations in Asia and Latin America. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has emerged as a promising treatment strategy, yet its efficacy and prognosis in ANKL patients remain to be systematically evaluated. We conducted a systematic review and meta-analysis by searching PubMed, Embase, Web of Science, and the Cochrane Library for studies published up to April 2025. The objective was to evaluate the outcomes of allo-HSCT in patients with ANKL. Six studies met the inclusion criteria. The primary outcomes included overall survival (OS) and progression-free survival (PFS), reported as hazard ratios (HRs), as well as standardized mean differences (SMDs) and median follow-up times for survivors (reported in months with ranges). Depending on the degree of heterogeneity-assessed using the I<sup>2</sup> statistic-random-effects or fixed-effects models were applied. Sensitivity analyses were conducted by sequentially excluding individual studies, and publication bias was assessed using funnel plots. Based on the analysis, a total of six studies comprising 295 ANKL patients who underwent allo-HSCT were included. The SMD for the median follow-up duration among survivors was 1.21 (95% CI: 0.39-2.02), indicating notable variation in follow-up times across studies. Heterogeneity for this outcome was minimal, with an I<sup>2</sup> of 0% and a p-value of 0.6621. Regarding the effect of allo-HSCT on overall survival, the pooled HR was 0.47 (95% CI: 0.32-0.68), demonstrating a statistically significant survival benefit associated with the procedure. Heterogeneity in this analysis was also low (I<sup>2</sup> = 0.0%, p = 0.684), reflecting strong consistency across the included studies. For PFS, the pooled HR was 0.22 (95% CI: 0.12-0.40), again indicating a significant improvement in outcomes. However, moderate heterogeneity was observed in this analysis (I<sup>2</sup> = 68.4%, p = 0.032). Funnel plot analysis revealed no significant evidence of publication bias, suggesting that the included data were robust and not substantially affected by selective reporting. This meta-analysis demonstrates that allo-HSCT significantly improves survival outcomes in patients with ANKL. The pooled hazard ratio of 0.47 indicates a favorable impact on overall survival. Additionally, the median survival time among survivors is notably longer, further supporting the efficacy of allo-HSCT. These results highlight the potential of allo-HSCT as a promising therapeutic strategy for ANKL, emphasizing the need for continued research to refine treatment protocols and enhance patient outcomes.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":"3433-3445"},"PeriodicalIF":3.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283809/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144224091","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}
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