Annals of Hematology最新文献

{"title":"Exploring thromboembolic risk factors in polycythemia vera: from current evidence to PROSPERO study design.","authors":"Valerio De Stefano, Francesco Passamonti, Francesca Palandri, Francesco Ramundo, Elena Rossi, Silvia Betti, Lorenzo Fagiolo, Paola Guglielmelli, Davide Pio Abagnale, Novella Pugliese, Daniele Cattaneo, Alessandra Iurlo, Filippo Branzanti, Alessandra Dedola, Hillary Catellani, Alessia Tieghi, Marco Basso, Elisabetta Calistri, Elena Maria Elli, Elena Masselli, Erika Morsia, Giulia Benevolo, Massimo Breccia, Vincenza de Fazio, Maria Di Perna, Monia Marchetti, Marco Santoro, Agostino Tafuri, Chiara Castiglioni, Chiara Rotella, Sergio Siragusa, Alessandro Maria Vannucchi","doi":"10.1007/s00277-025-06466-z","DOIUrl":"https://doi.org/10.1007/s00277-025-06466-z","url":null,"abstract":"<p><p>Polycythemia vera (PV) is a chronic myeloproliferative neoplasm with a substantial risk of thromboembolic events (TEs), which contribute to morbidity and mortality. Traditional thrombotic risk stratification primarily considers age and thrombosis history, yet these parameters alone do not capture the complexity of thrombotic risk. Growing evidence highlights the role of additional factors influencing the risk of TEs, underscoring the need for a more comprehensive approach to patient stratification. This paper reviews the current understanding of thromboembolic risk factors in PV and provides the rationale, methodology, and expected contributions of the PROSPERO study, a prospective, multicenter study designed to improve thrombotic risk assessment in patients with high-risk PV. By examining established (advanced age, prior TEs, cardiovascular comorbidities) and emerging thromboembolic risk factors, including specific hematologic parameters, the study aims to emphasize their impact and potential synergistic interactions on thrombotic risk. This review also evaluates the efficacy and limitations of current therapies, such as hydroxyurea (HU), interferons, and ruxolitinib, in preventing TEs, and further underscores the need for comprehensive predictive models to guide individualized management strategies. The PROSPERO study focuses on high-risk PV patients who experienced at least one prior TE and receive either HU or ruxolitinib, aiming to identify predictive factors for TEs and their individual and combined contributions to thrombotic risk by collecting longitudinal data on clinical, laboratory, and treatment-related parameters. PROSPERO aims to identify and validate new variables that can inform the development of precise, integrated prediction models. The findings are expected to enable tailored treatment approaches, ultimately reducing TE recurrence in high-risk PV populations.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144658188","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}

{"title":"Treatment burden in patients with paroxysmal nocturnal hemoglobinuria: an in-depth interview survey.","authors":"Yasutaka Ueda, Naoshi Obara, Shikiko Ueno, Masatoshi Sakurai, Kohei Hosokawa, Tatsunori Murata, Yuta Fukuoka, Nozomi Hayama, Masami Yamashita, Yoshiaki Ogawa, Kensuke Usuki, Takayuki Ikezoe, Tatsuya Kawaguchi, Naoki Hosen, Yuzuru Kanakura, Akihiko Gotoh, Jun-Ichi Nishimura","doi":"10.1007/s00277-025-06486-9","DOIUrl":"https://doi.org/10.1007/s00277-025-06486-9","url":null,"abstract":"<p><p>Paroxysmal nocturnal hemoglobinuria (PNH) is a lifelong, clonal hematologic disease posing life-threatening risks if untreated. The prognosis for PNH has improved with the advent of C5 inhibitors, which are now the standard of care where available. As treatment options continue to expand, healthcare providers can better address both PNH management and the impact of treatment on patients' daily lives. To investigate the burden of disease and key factors taken into consideration when patients with PNH choose their preferred treatment, we conducted an in-depth patient interview survey. Of survey participants (N = 30), 70.0% were receiving intravenous C5 inhibitors. Notably, 56.7% of all patients reported needing ≥ 1 hospital visit per month, and 46.7% required a day off from work or school for visits (33.3% among those receiving intravenous C5 inhibitors). A frequently reported burden of PNH treatment was financial concern, including the cost of treatment, hospital visits, and the negative impact on income. Additionally, burden related to waiting times and distance from the hospital and the overall time spent on outpatient PNH care were identified with similar results for patients receiving intravenous C5 inhibitors. These results suggest that the time and effort to get to treatment centers and the time required to receive treatment for PNH were critical unmet needs in PNH care. Our study indicates that persistent burdens associated with current PNH care should be taken into account alongside therapeutic effectiveness when making treatment decisions. Further analysis with a larger sample size is required to confirm these findings.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144658189","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}

{"title":"Clinical features and fusion gene analysis of two Torque Teno Mini virus associated acute promyelocytic leukemia cases.","authors":"Deyan Liu, Jiaqi Chen, Yu Luo, Shu Yan, Hua Nan, Xue Chen, Zhongyuan Lin, Liejun Jiang, Huihe Tang, Hongyan Ma, Yumeng Niu, Jiancheng Fang, Panxiang Cao, Lili Yuan, Xiaoli Ma, Xiaosu Zhou, Fanyong Lv, Yan Dai, Hongxing Liu","doi":"10.1007/s00277-025-06501-z","DOIUrl":"https://doi.org/10.1007/s00277-025-06501-z","url":null,"abstract":"<p><p>Torque Teno Mini Virus (TTMV), a member of the Anelloviridae family, is a commensal component of the human virome. Since the initial identification of the TTMV::RARA fusion gene as a novel driver of acute promyelocytic leukemia (APL), 15 cases have been reported in retrospective studies. With advancements in diagnostic methods and increased awareness, the number of newly diagnosed cases has risen, and the clinical and molecular characteristics of TTMV::RARA-APL are becoming clearer. We systematically identified the clinical characteristics, fusion gene analysis, and treatment protocols of two pediatric APL patients harboring the TTMV::RARA fusion on this basis. While the detection of TTMV::RARA contributes to defining pathogenic fusion gene and MRD monitoring indicators in non-PML::RARA-APL cases, the precise pathogenic mechanisms of this ubiquitous symbiotic virus warrant further investigation.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144658187","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}

{"title":"Consequences and impacts of PEG-IFNα2a shortage: first lessons from a MPN French center.","authors":"Laura Cailly, Wayne-Corentin Lambert, Jean-Richard Eveillard, Brigitte Pan-Petesch, Laura Herbreteau, Laetitia Rio, Lanig Civi, Adrian Tempescul, Florence Dalbies, Hussam Saad, Cristina Bagacean, Marie-Anne Couturier, Gaelle Guillerm, Eric Lippert, Jean-Christophe Ianotto","doi":"10.1007/s00277-025-06510-y","DOIUrl":"https://doi.org/10.1007/s00277-025-06510-y","url":null,"abstract":"<p><p>Between March to December 2024, we observed a PEG-IFNα2a shortage in France for MPN patients. We identified and followed 90 patients from our center and observed 80% of difficulties obtaining the drug, and 61 patients among all (67.8%) did not receive their prescribed drug on time (impacted group). Twenty-one patients need to reduce the dosage of PEG-IFNα2a and 27 stopped the drug. Ten clinical events were identified with nine in the impacted group (p=0.04). No hematological or only partial responses were more frequent in the impacted group (p=0.01). The allelic burden variations were also studied during this period; 17 patients obtained a VAF <10%. Eight patients stopped the drug because of VAF <4% and remained with molecular response. Three-quarters of the patients experimented with psychological and financial impacts of shortage. This study is the first one to explore a shortage of cytoreductive drugs happened in MPN. Much informations will be helpful for clinicians.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144648395","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}

{"title":"Prognostic significance of angiogenesis-associated molecules and Immunologic characteristic in elderly patients with acute myeloid leukemia.","authors":"Can Chen, Yongfen Huang, Lingling Wang, Linlin Zhang, Jinbo Lu, Yuexin Cheng, Yuqing Miao","doi":"10.1007/s00277-025-06454-3","DOIUrl":"https://doi.org/10.1007/s00277-025-06454-3","url":null,"abstract":"<p><strong>Background: </strong> Neovascularization mechanisms are hyperactivated in tumors, leading to vascular dysfunction and contributing to tumor metastasis and growth. This study aims to comprehensively analyze angiogenesis-associated genes in relation to the prognosis of elderly patients with acute myeloid leukemia (AML).</p><p><strong>Methods: </strong>Leukemia gene expression data were obtained from the GSE37642 (training set) and TCGA_LAML (validation set) datasets. Angiogenesis-associated genes were identified using the GeneCards database. Univariate Cox regression and LASSO analyses were employed to identify angiogenesis-associated genes linked to AML prognosis. A prognostic signature was constructed based on the selected genes, and its biological functions were analyzed. Finally, we predicted AML drug sensitivity and evaluated differences in drug activity based on the prognostic signature.</p><p><strong>Results: </strong>Five angiogenesis-related genes associated with AML prognosis were identified: ECM1, EGLN1, FKBP5, FOXP1, and SIRT2. Kaplan-Meier analyses confirmed their prognostic value. A prognostic signature based on these genes demonstrated commendable efficacy in predicting patient outcomes. This signature was found to be an independent risk factor for AML and revealed distinct immune profiles. Furthermore, the signature was implicated in the tumor immune microenvironment, with high-risk patients exhibiting elevated levels of immune cell infiltration. Drug sensitivity analysis revealed negative correlations between FOXP1 and Daporinad, ABT737, and BI.2536, while SIRT2 showed positive correlations with ABT737, BI.2536, and ULK1_4989.</p><p><strong>Conclusion: </strong>We have constructed an angiogenesis-related gene prognostic signature that enriches the prognostic assessment system for AML and provides novel therapeutic directions for this disease.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144648310","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}

{"title":"Rethinking bone marrow biopsy: is PET/CT enough for staging low-grade follicular lymphomas?","authors":"Yan-Yan Chen, Ji-Jin Wang, Wen-Huan Zhong, Jiang-Rui Guo, Yan-Yan Qiu, Tian-Xiu Liu, Hao Zheng, Si-Lin Chen, Si-Qin Liao, Rui-Zhi Zhao, Gui-Qing Shi, Tian-Lan Tang, Yu-Ping Lin, Cheng Huang, Jin-Hua Chen, Ting-Bo Liu, Yu-Jing Zhang, Yong Yang","doi":"10.1007/s00277-025-06487-8","DOIUrl":"https://doi.org/10.1007/s00277-025-06487-8","url":null,"abstract":"<p><p>This study evaluates the diagnostic performance of staging 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) for detecting bone marrow involvement (BMI) in low-grade follicular lymphoma (FL) and its impact on the clinical necessity of bone marrow biopsy (BMB). We retrospectively analyzed patients newly diagnosed with low-grade (grade 1-2) FL who underwent both ¹⁸F-FDG PET/CT and BMB from 2010 to 2022 at two Chinese institutions. PET/CT's diagnostic accuracy for BMI was assessed using BMB as gold standard. Clinical and imaging data were analyzed to identify risk factors for BMI. Among 171 patients, 27 had positive BMB results. PET/CT demonstrated 86.5% accuracy in detecting BMI. In patients with PET/CT-based stage I-II disease, all BMB results were negative. In contrast, among patients with PET/CT-based stage III-IV disease, BMB upstaged 13 to stage IV. In patients with advanced-stage disease as determined by PET/CT, four risk factors were significantly linked to BMB positivity: female sex, Eastern Cooperative Oncology Group performance score > 1, elevated beta2-microglobulin levels, and involvement of > 4 lymph node regions. Patients were stratified into low-, intermediate-, and high-risk groups with corresponding BMB-positive rates of 5.6%, 40.7%, and 68.8%, respectively. No survival differences were observed between BMB-positive and BMB-negative patients. PET/CT can safely and effectively substitute for BMB in the staging of early-stage, low-grade FL. However, in advanced-stage disease, BMB remains valuable for diagnosis. The rate of BMB positivity correlates with tumor burden -related risk factors.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144648311","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}

{"title":"Validation of the simplified geriatric prognostic index in a Hellenic database of older patients (≥ 70 years old) with diffuse large B-cell lymphoma and similar disorders.","authors":"Theodoros P Vassilakopoulos, Fotios Panitsas, Maria Panagiota Arapaki, Amalia Anastasopoulou, Christina Apostolopoulou, Evgenia Verrou, Christina H Kalpadakis, Alexia Piperidou, Athanasios Liaskas, Sotirios Sachanas, Theodora Triantafyllou, Aikaterini Bitsani, Maria Dimou, Maria Anastasia Ioakeim, Panagiotis Tsirigotis, Marina P Siakantaris, Vassiliki Pappa, Panayiotis Panayiotidis, Gerassimos A Pangalis, Eirini Katodritou, Maria K Angelopoulou, Sotirios G Papageorgiou","doi":"10.1007/s00277-025-06446-3","DOIUrl":"https://doi.org/10.1007/s00277-025-06446-3","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144648312","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}

{"title":"Filgrastim after autologous stem cell transplantation shortens time to neutrophil recovery and hospital stay.","authors":"K A Oosterink, T J A Dekker, J M L Tjon, W A F Marijt, L C J Te Boome, H Veelken, P A von dem Borne, C J M Halkes","doi":"10.1007/s00277-025-06509-5","DOIUrl":"https://doi.org/10.1007/s00277-025-06509-5","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144641601","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}

{"title":"Advantages of AI-based whole blood film scanning for blast detection in markedly leucopenic blood films.","authors":"Geng Wang, Lin Zheng, Zhejun Fang, Guoju Luo, Qian Chen, Qi Qi Zhang, Bo Shen Wu, Xin Wang, Rongrong Cheng, Ping Deng, Binyao Zhang, Jing Jin, Wei Wu","doi":"10.1007/s00277-025-06473-0","DOIUrl":"https://doi.org/10.1007/s00277-025-06473-0","url":null,"abstract":"<p><p>To evaluate performance of AI-assisted blast detection via whole blood film scanning in cases of severe leukopenia. From August to October 2023, we collected 157 patient specimens with white blood cell (WBC) counts of ≤2.0 × 10⁹/L. We then evaluated and compared the blast detection capabilities of an artificial intelligence-based system (Cygnus instrument) against the CellaVision DI-60 for WBC analysis. Additionally, this study aimed to assess the comparative performance of these two automated blood cell morphology analyzers based on their underlying operational principles. Following manual microscopy confirmation, 17 cases showing ≥ 1 blast cell in peripheral blood smear were identified among 157 specimens with WBC ≤ 2.0 × 10⁹/L. The Cygnus whole-slide scanning mode detected all 17 cases (sensitivity: 100%), whereas the Cygnus 200-cell mode detected only 9 cases (9/17), and the CellaVision DI-60 200-cell mode detected 8 cases (8/17). In leukopenic patients (WBC ≤2.0×10⁹/L), utilizing Cygnus' whole-slide scanning capability substantially improves blast identification rates, potentially enabling earlier detection of hematologic abnormalities.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144636011","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}

{"title":"Successful treatment with mesenchymal stromal cells-Frankfurt am Main in a pediatric patient with steroid-refractory and ruxolitinib-refractory acute gastrointestinal graft-versus-host disease.","authors":"Jana Ernst, Steffen Reinsch, Hans-Joachim Mentzel, Till Milde, Bernd Gruhn","doi":"10.1007/s00277-025-06491-y","DOIUrl":"https://doi.org/10.1007/s00277-025-06491-y","url":null,"abstract":"<p><p>Acute graft-versus-host disease (aGVHD) as complication after allogeneic hematopoetic stem cell transplantation (allo-HSCT) occurs in up to 70% of patients. The survival prognosis depends on the severity of GVHD and response to first-line therapy. In patients who are refractory to both, steroids and ruxolitinib, an unmet medical need exists, especially in severe gastrointestinal forms of aGVHD. We report the case of a 12-year-old male patient who developed severe gastrointestinal aGVHD after allo-HSCT for the treatment of high-risk acute myeloid leukemia. The grade IV aGVHD of the intestine showed no improvement under triple immunosuppression with corticosteroids, cyclosporine A and ruxolitinib. After receiving four weekly infusions of human allogeneic mesenchymal stromal cells DRK-BaWü-He-FFM (MSC-FFM), intestinal inflammation finally improved, as reflected by ameliorated symptoms, normalized intestinal wall thickness (ultrasonography) and decreasing calprotectin levels. Intestinal mucosal healing was further supported by a strict, formula-based modular diet. The present case supports efficacy and safety of MSC-FFM in combination with modular nutrition in complicated courses of aGVHD of the intestine refractory to multiple lines of therapy and introduces intestinal wall thickness and calprotectin levels as valuable markers of disease activity.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144636012","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}