Hematology最新文献

{"title":"Polyclonal B-cell lymphocytosis: a rare, benign condition that mimics a chronic lymphoproliferative disorder.","authors":"Emily Parkes, Pedro Martin-Cabrera, Hannah Creasey, David Bloxham, George Follows","doi":"10.1080/16078454.2026.2704266","DOIUrl":"10.1080/16078454.2026.2704266","url":null,"abstract":"<p><strong>Objectives: </strong>We report a case of Persistent polyclonal B-cell lymphocytosis (PPBL), a rare and benign lymphoproliferative disorder, to highlight the importance of recognising its characteristic features in order to avoid unnecessary investigations and inappropriate treatment. We also underscore the potential reversibility of disease features with smoking cessation.</p><p><strong>Results: </strong>The patient presented with a prolonged history of low-level abdominal discomfort and unintentional weight loss on a background of a significant smoking history. Physical examination revealed splenomegaly. The laboratory results revealed a persistent lymphocytosis, prompting a referral to a haematology specialist with a working diagnosis of a malignant lymphoproliferative disorder. Subsequent investigations revealed markedly elevated polyclonal IgM with no monoclonal paraprotein via serum electrophoresis. A peripheral blood smear demonstrated characteristic binucleate lymphocytes, and flow cytometry confirmed polyclonal B-cell expansion with a normal immunophenotype. Molecular and cytogenetic analysis excluded B-cell neoplasia and supported a final diagnosis of PPBL.</p><p><strong>Discussion: </strong>PPBL can closely mimic malignant chronic lymphoproliferative disorders. Recognition of its characteristic laboratory and morphological features, namely polyclonal lymphocytosis, atypical binucleated lymphocytes, and elevated serum immunoglobulin M (IgM) without a monoclonal paraprotein, is essential to avoid unnecessary invasive investigations and inappropriate treatment. PPBL pathogenesis remains elusive but is likely to involve a genetic predisposition and chronic antigen stimulation. Cigarette smoking appears to be the dominant environmental trigger, with cessation often leading to stabilisation or resolution of lymphocytosis. The role of the Epstein‒Barr virus has been proposed given the binucleated lymphocytes and polyclonal B-cell expansion, but evidence remains inconclusive.</p><p><strong>Conclusion: </strong>This case highlights the importance of recognising PPBL to avoid unnecessary invasive investigations and inappropriate treatment and underscores the potential reversibility of disease features with smoking cessation.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2704266"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148630342","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Highly suppressive functional CCR7 ^<b>low</b> Treg subset cells may correlate with poor prognosis of B-ALL.","authors":"Sui Songnan, Jiamian Zheng, Jing Lai, Yupei Zhang, Xu Jiang, Ling Xu, Liye Zhong, Jie Chen, Jie Wu, Xun Zhu, Yangqiu Li, Guocai Wu, Zhanghui Chen","doi":"10.1080/16078454.2026.2702683","DOIUrl":"10.1080/16078454.2026.2702683","url":null,"abstract":"<p><strong>Background: </strong>Regulatory T cells (Tregs) may influence prognosis in leukemia. This study aimed to explore the effects and mechanisms of Treg subclusters on the prognosis in B-cell acute lymphoblastic leukemia (B-ALL) using single-cell RNA sequencing (scRNA-Seq).</p><p><strong>Methods: </strong>scRNA-seq was performed on sorted T cells from adults with newly diagnosed (Dix) B-ALL and healthy donors (HD) to establish the training cohort. Treg phenotypes, functions, and prognostic relevance were evaluated. A scRNA-seq dataset from the GEO database, including Dix and relapsed B-ALL, served as the validation cohort. Key findings were confirmed by flow cytometry. Transcription factor and cell-cell interaction analyses were conducted to clarify the mechanisms linking Treg subsets to prognosis.</p><p><strong>Results: </strong>Tregs were divided into CCR7^high and CCR7^low subclusters. The proportion of CCR7^low Tregs was higher in B-ALL than in HD and in Rel than in Dix. Patients with higher CCR7^low Treg signature scores had a poorer prognosis. Compared with CCR7^high Tregs, CCR7^low Tregs exhibited enhanced activation, proliferation, and immunosuppression. Trajectory analysis suggested differentiation from CCR7^high to CCR7^low Tregs. IKZF2 and JUNB showed consistently increased activity and expression in CCR7^low Tregs across cohorts. CCR7^low Tregs showed increased interactions with terminal effector and exhausted CD8⁺ T cells through the LGALS9-P4HB/HAVCR2/CD44 pathway.</p><p><strong>Conclusion: </strong>An increased proportion of CCR7^low Tregs correlated with poor prognosis in B-ALL, potentially due to enhanced activation, proliferation, and immunosuppression. IKZF2 and JUNB upregulation may promote CCR7^low Treg differentiation and function. Moreover, CCR7^low Tregs may impair CD8⁺ T cell immunity through the LGALS9-P4HB/HAVCR2/CD44 signaling.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2702683"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148602494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical factors and relative risk stratification for repeat platelet transfusion in thrombocytopenia.","authors":"Yueyan Fu, Chuanbiao Ding, Fan Wang, Xiaodong Ye, Xiang Weng","doi":"10.1080/16078454.2026.2718680","DOIUrl":"https://doi.org/10.1080/16078454.2026.2718680","url":null,"abstract":"<p><strong>Objective: </strong>To identify clinical factors associated with repeat platelet (PLT) transfusion among thrombocytopenic patients and develop an exploratory relative-risk stratification framework for repeat transfusion during hospitalization.</p><p><strong>Methods: </strong>This retrospective case-control study included thrombocytopenic inpatients who received at least one PLT transfusion. Patients with repeat transfusion (≥2 transfusions during the same hospitalization, n=145) were compared with those receiving a single transfusion (n = 145). Multivariable logistic regression using clinically prespecified predictors was performed, with ten-fold cross-validated LASSO used to assess variable stability. Model performance was evaluated by AUC, calibration measures, Brier score, and 1,000-resample bootstrap optimism correction. A simplified integer-based score was developed.</p><p><strong>Results: </strong>Multivariable analysis identified hematologic malignancy, active bleeding, splenomegaly, lower PLT and hemoglobin levels, absence of thrombopoietic-agent use before the first transfusion, and transfusion timing as factors associated with repeat PLT transfusion. The primary model demonstrated an apparent AUC of 0.805 (95% CI, 0.756-0.855), with a bootstrap-corrected C-index of 0.777. The corrected Brier score was 0.196, and the bootstrap-corrected calibration intercept and slope were 0.001 and 0.860, respectively. The simplified score (0-12 points) stratified patients into low-, medium-, and high-score groups, with observed repeat transfusion proportions of 20.0%, 50.7%, and 83.8%, respectively (trend χ² = 59.904, <i>P</i> < 0.001).</p><p><strong>Conclusion: </strong>Several clinical factors were associated with repeat PLT transfusion. The proposed score may support exploratory relative-risk stratification within the development sample; external validation is required before estimating absolute risk or clinical implementation.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2718680"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148792418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An individualized nomogram for predicting progression-free survival in systemic anaplastic large cell lymphoma: a multicenter, retrospective, and internally validated study.","authors":"Mei Zhou, Cui-Zhen Liu, Zhong-Qiong Wei, Sheng-Sheng Zhou, Kai-Teng Cai, Ji-Hao Zhou, Li-E Lin, Ting-Bo Liu, Wen-Yu Li, Hai-Yan Yang, Man Wang, Zhi-Gang Peng, Jie Ma","doi":"10.1080/16078454.2026.2715239","DOIUrl":"10.1080/16078454.2026.2715239","url":null,"abstract":"<p><strong>Objectives: </strong>To develop an individualized nomogram for predicting disease progression risk in systemic anaplastic large cell lymphoma (sALCL).</p><p><strong>Methods: </strong>Independent predictors of progression-free survival (PFS) were identified using Cox regression in a multicenter retrospective cohort of 109 sALCL patients (2010-2022). These were incorporated into a three-factor nomogram, evaluated via bootstrapped internal validation (1000 resamples), ROC analysis, C-index, decision curve analysis (DCA), and clinical impact curve (CIC).</p><p><strong>Results: </strong>A total of 29 PFS events occurred during a median follow-up of 31 months. Multivariable modelling selected serum β2-microglobulin elevation, extranodal disease, and front-line chemotherapy choice (CHOP versus CHOPE or BV+CHP) as autonomous progression drivers. Upon internal bootstrap validation, the nomogram yielded strong prognostic accuracy, achieving AUCs of 0.81, 0.85 and 0.87 for 1-, 3- and 5-year progression-free survival, alongside a corrected C-index of 0.779 (95% CI: 0.699 - 0.861). Calibration plots showed close agreement between predicted and observed outcomes, while DCA confirmed superior net clinical benefit versus conventional IPI or Ann Arbor stratification across multiple decision thresholds.</p><p><strong>Conclusion: </strong>This first sALCL-specific nomogram integrates clinical and treatment variables to provide personalized PFS risk estimation. While internally validated, this exploratory, observation-based tool requires external validation and recalibration in prospective cohorts before clinical implementation.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2715239"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148724441","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incorporation of selinexor into conditioning regimens prior to allogeneic hematopoietic stem cell transplantation: a single-center clinical study.","authors":"Bingjie Wang, Qian Wang, Jialin Zhu, Zeyin Liang, Yue Yin, Yuan Li, Jinping Ou, Hanyun Ren, Yujun Dong","doi":"10.1080/16078454.2026.2724615","DOIUrl":"https://doi.org/10.1080/16078454.2026.2724615","url":null,"abstract":"<p><strong>Objective: </strong>Clinical data on incorporating selinexor into conditioning regimens before allogeneic hematopoietic stem cell transplantation (allo-HSCT) remain limited. We evaluated the feasibility and safety of selinexor-containing conditioning in patients with high-risk myeloid malignancies.</p><p><strong>Methods: </strong>This retrospective single-center case series included 12 consecutive patients receiving selinexor-containing conditioning before allo-HSCT. Toxicities, engraftment, graft-versus-host disease (GVHD), relapse, non-relapse mortality (NRM), and survival were descriptively evaluated. PFS and OS were estimated using Kaplan-Meier methods; competing-risk methods were used for relapse, NRM, and GVHD; and follow-up was estimated using reverse Kaplan-Meier.</p><p><strong>Results: </strong>All patients completed planned selinexor administration, with manageable toxicity and no unexpected organ toxicity. Neutrophil engraftment was achieved in all evaluable patients and platelet engraftment in the majority. Median follow-up was 42 months (95% CI, 25-42 months). Median PFS and OS were 9.0 months (95% CI, 2.0-25 months) and 10.0 months (95% CI, 2-30 months), respectively. At 12 months, the cumulative incidences of relapse and NRM were both 33.3% (95% CI, 10.3%-58.8%). Day + 100 overall aGVHD incidence was 41.7% (95% CI, 15.2%-66.5%), and 12-month overall cGVHD incidence was 25.0% (95% CI, 6.0%-50.5%).</p><p><strong>Conclusion: </strong>In this single-center retrospective experience, incorporation of selinexor into conditioning regimens prior to allo-HSCT was feasible and associated with manageable toxicity in in selected patients with high-risk myeloid malignancies. Given the small sample size and absence of a comparator cohort, these findings should be interpreted cautiously. Prospective studies are warranted to further define the role of selinexor in the transplant setting.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2724615"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Human platelet 5-hydroxyindoleacetic acid concentrations are meager and unlikely to be physiologically active or play a role in inflammation.","authors":"George M Anderson, Martijn Van Faassen, Ido P Kema","doi":"10.1080/16078454.2026.2733376","DOIUrl":"https://doi.org/10.1080/16078454.2026.2733376","url":null,"abstract":"<p><strong>Objectives: </strong>A crucial role for platelet 5-hydroxyindoleacetic acid (5-HIAA, the principal serotonin metabolite) in human immune response and inflammation has been claimed. We have attempted to assess the likelihood of such a role for platelet 5-HAA. To assess the concentration of 5-HIAA in human platelets, we have reviewed prior reports of platelet 5-HIAA concentrations and to measure 5-HIAA concentrations in human platelet-poor plasma (PPP) and platelet-rich plasma (PRP) samples.</p><p><strong>Methods: </strong>A liquid chromatographic-mass spectrometric method (LC-MS) was used to measure 5-HIAA levels in simultaneously obtained PPP and PRP samples (<i>n</i> = 68).</p><p><strong>Results: </strong>Measured median PPP and PRP 5-HIAA concentrations of 9.7 ng/mL (IQR: 7.69-10.6), and 8.42 ng/mL (IQR: 6.57-10.3), respectively, were similar. Levels of PPP and PRP 5-HIAA were highly correlated (Spearman's R² = .872. Measured levels were consistent with most prior reports.</p><p><strong>Discussion: </strong>Based on the observed PRP and PPP 5-HIAA concentrations, platelet 5-HIAA concentrations appear to be less than 1 ng/billion platelets. This compares to a typical mean concentration of human platelet serotonin of 600 ng/billion platelets.</p><p><strong>Conclusion: </strong>It appears that the platelet has only negligible amounts of 5-HIAA and that platelet 5-HIAA is unlikely to play a role in human immune response, in inflammation, or in any other aspect of human physiology.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2733376"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149001720","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"In-hospital and short-term outcomes in patients with atrial fibrillation undergoing chimeric antigen receptor-T cell therapy.","authors":"Nan Zhao, Wen Li, Qian Chen, Qi Zhang, Qiuyan Yu, Na Liu, Xunxun Zhu, Zixun Yin, Hui Wang, Kui Zhang, Chi Zhou, Min Fang, Hongjing Dong","doi":"10.1080/16078454.2026.2662692","DOIUrl":"10.1080/16078454.2026.2662692","url":null,"abstract":"<p><strong>Background: </strong>Chimeric antigen receptor T-cell (CAR-T) therapy is an innovative immunotherapy for hematologic malignancies. Cardiovascular complications associated with CAR-T therapy have also received increasing attention. However, the impact of atrial fibrillation (AF) on outcomes in patients undergoing CAR-T therapy remains poorly understood.</p><p><strong>Methods: </strong>We conducted a retrospective study using the National Readmission Database (NRD) to identify adult patients who underwent CAR-T therapy between 2017 and 2020. We divided the patients into two groups based on the presence of AF to study in-hospital and 30-day outcomes. Propensity score matching was performed, and outcomes were analyzed using multivariable logistic regression and Cox proportional hazards models. The primary outcomes included in-hospital mortality, stroke, sepsis, and 30-day readmission.</p><p><strong>Results: </strong>A total of 3,003 hospitalizations were identified, incluing 162 (5.39%) patients with AF. Patients in the AF group were older and more likely to be male. Compared with patients without AF, those with AF had higher rates of in-hospital mortality (7.4% vs 3.2%; <i>P</i> = 0.007), sepsis (18.5% vs 8.8%; <i>P</i> < 0.001), and stroke (8.0% vs 3.2%; <i>P</i> < 0.001). No significant differences were observed in neurotoxicity or acute kidney injury. In 30-day readmissions, there were no differences in all-cause readmission or in readmissions due to cancer- or treatment-related events, sepsis or infection, and neurologic events.</p><p><strong>Conclusion: </strong>Among patients undergoing CAR-T cell therapy, AF was associated with a higher risk of in-hospital mortality, stroke and sepsis. However, the 30-day all-cause readmission rate showed no difference between the AF group and the non-AF group.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2662692"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147769982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Threshold effect of serum 25-hydroxyvitamin D on Anemia risk in U.S. adults aged 45 years and older.","authors":"Xuekong Li, Ruixia Qi, Ruimin Li, Yuying Ren, Zekang Li, Lizhen Zhao, Keyu Liu","doi":"10.1080/16078454.2026.2746132","DOIUrl":"https://doi.org/10.1080/16078454.2026.2746132","url":null,"abstract":"<p><strong>Background: </strong>While previous studies have reported conflicting linear or U-shaped associations between vitamin D and anemia, evidence regarding the specific dose-response relationship in middle-aged and older adults remains limited. This study aimed to identify the threshold effect of serum 25-hydroxyvitamin D [25(OH)D] on anemia risk in U.S. adults aged ≥ 45 years.</p><p><strong>Methods: </strong>We conducted a cross-sectional analysis using data from the National Health and Nutrition Examination Survey (NHANES) 2007-2018. A total of 9,306 participants were included. Anemia was defined according to WHO hemoglobin thresholds. Survey-weighted multivariate logistic regression and restricted cubic spline (RCS) models, together with segmented regression, were used to assess the association and identify inflection points.</p><p><strong>Results: </strong>The prevalence of anemia was 13.3%. In the fully adjusted survey-weighted model, higher serum 25(OH)D levels were independently associated with a lower risk of anemia. RCS analysis revealed a significant non-linear association (<i>P</i> for non-linearity < 0.001), identifying a threshold of 64.57 nmol/L (bootstrap 95% CI: 64.45-64.69). Below this threshold, each 1 nmol/L increment in serum 25(OH)D was associated with approximately 2% lower odds of anemia (OR = 0.98, 95% CI: 0.97-0.99, <i>P</i> < 0.001); at or above this threshold, the association plateaued, with no significant further effect on anemia risk (OR = 1.00, 95% CI: 1.00-1.01, <i>P</i> = 0.64).</p><p><strong>Conclusion: </strong>In U.S. adults aged ≥ 45 years, the protective association between serum 25(OH)D and anemia appears to exhibit a threshold around 64.57 nmol/L, below which lower levels were associated with progressively higher anemia risk. These findings are hypothesis-generating and merit confirmation in prospective and interventional studies.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2746132"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149147701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Distinct clinical, molecular, and treatment response profiles in primary and secondary myelofibrosis: a single-center retrospective study.","authors":"Wenya Liu, Yue Ren, Yingao Liang, Yumei Liu, Liyan Yang, Xifeng Dong, Limin Xing, Zonghong Shao, Rong Fu, Huaquan Wang","doi":"10.1080/16078454.2026.2682015","DOIUrl":"10.1080/16078454.2026.2682015","url":null,"abstract":"<p><strong>Background: </strong>Myelofibrosis (MF) includes primary myelofibrosis (PMF) and secondary myelofibrosis (SMF) evolving from essential thrombocythemia (ET) or polycythemia vera (PV). The clinical and molecular heterogeneity of MF remains incompletely characterized in real-world cohorts.</p><p><strong>Methods: </strong>We retrospectively analyzed 56 patients with MF treated at a single center, including 33 with PMF and 23 with SMF (ET-MF or PV-MF). Clinical characteristics, laboratory parameters, bone marrow pathology, cytogenetic and molecular findings, treatment strategies, and follow-up outcomes were evaluated.</p><p><strong>Results: </strong>PMF patients demonstrated a predominantly cytopenic phenotype, characterized by more severe anemia and lower platelet counts, whereas SMF patients showed a more proliferative profile with higher leukocyte and platelet counts. Bone marrow examination revealed typical megakaryocytic atypia and reticulin fibrosis in both groups, while PMF exhibited more heterogeneous marrow cellularity. Mutational analysis showed that <i>JAK2</i> was the most frequent driver mutation, followed by <i>CALR</i> and <i>MPL</i>, and approximately one-third of PMF cases were triple-negative. Among additional mutations, <i>ASXL1</i> was the most common, followed by spliceosome-related genes such as <i>SF3B1</i> and <i>U2AF1</i>. Patients harboring <i>JAK2</i> mutations displayed higher hemoglobin levels, red blood cell counts, and neutrophil percentages compared with JAK2-wild type patients. Treatment strategies included supportive care, interferon, hydroxyurea, and ruxolitinib. During follow-up, most patients remained alive, although leukemic transformation occurred in a small proportion of PMF cases.</p><p><strong>Conclusions: </strong>PMF and SMF demonstrate distinct clinical phenotypes despite sharing bone marrow fibrosis as a common endpoint. The mutational landscape highlights the genetic heterogeneity of MF and underscores the importance of integrating clinical, pathological, and molecular information to improve disease characterization and guide individualized management.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2682015"},"PeriodicalIF":1.6,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"OVCA2 acts as an oncogene in pediatric AML by negatively regulating CDKN1A to drive cell cycle progression.","authors":"Wanyan Jiao, Saisai Cheng, Xiao Yang, Haiyan Liu, Xiaodong Tang, Qianlong Li, Ying Li, Bi Zhou","doi":"10.1080/16078454.2026.2665020","DOIUrl":"10.1080/16078454.2026.2665020","url":null,"abstract":"<p><strong>Objectives: </strong>Pediatric acute myeloid leukemia (AML) is biologically distinct from adult AML and carries a poor prognosis. OVCA2, a serine hydrolase with context-dependent roles, has unknown function in AML. We aimed to investigate its role, regulation, and clinical significance in pediatric AML.</p><p><strong>Methods: </strong>OVCA2 was identified as a downstream target of a pediatric AML-specific core transcriptional regulatory circuit using CUT&Tag integrative analysis. Expression and prognostic associations were assessed in TARGET (pediatric) and TCGA (adult) datasets. Functional validation via lentiviral shRNA knockdown was performed in MV4-11 and Kasumi-1 cells using CCK-8, colony formation, and cell cycle assays. RNA-seq and GSEA elucidated mechanisms. Co-knockdown of CDKN1A tested functional rescue.</p><p><strong>Results: </strong>OVCA2 was significantly upregulated in AML and correlated with worse overall survival exclusively in the pediatric cohort. Knockdown impaired proliferation, colony formation, and induced G1 arrest, with reduced C-MYC and CDK2 levels. Transcriptomic analysis revealed activation of 'Negative Regulation of Cell Population Proliferation' pathway, with CDKN1A as a top upregulated gene. Co-knockdown of CDKN1A partially rescued the anti-proliferative effect.</p><p><strong>Discussion: </strong>OVCA2 acts as a novel oncogene in pediatric AML by transcriptionally repressing CDKN1A to drive cell cycle progression. Its age-specific prognostic association and origin from a pediatric AML core regulatory circuit highlight its role in age-related regulatory networks.</p><p><strong>Conclusion: </strong>OVCA2 represents a promising therapeutic target in pediatric AML, with its effects mediated through CDKN1A repression.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2665020"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147770008","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}