HematologyPub Date : 2026-12-31Epub Date: 2026-05-03DOI: 10.1080/16078454.2026.2662818
Sihui Chen, Zhenqi Lou, Jieyang Zhu, Jun Xue, Biao Huang, Zinan Lin, Yun Li, Hongjuan Yang
{"title":"Lactylation and liquid-liquid phase separation related genes influence prognosis and immune characteristics of diffuse large B-cell lymphoma patients.","authors":"Sihui Chen, Zhenqi Lou, Jieyang Zhu, Jun Xue, Biao Huang, Zinan Lin, Yun Li, Hongjuan Yang","doi":"10.1080/16078454.2026.2662818","DOIUrl":"10.1080/16078454.2026.2662818","url":null,"abstract":"<p><strong>Objectives: </strong>Lactylation and liquid-liquid phase separation related genes have been reported to be associated with tumor prognosis and immunity, but their specific influence on the prognosis and immune characteristics of diffuse large B-cell lymphoma (DLBCL) remains unclear.</p><p><strong>Methods: </strong>GSE56315 (33 control samples and 55 DLBCL patient samples) has been used to screen for lactylation and liquid‒liquid phase separation related differentially expressed genes (LLRDEGs). Based on the optimal cutoff, LLRDEGs associated with DLBCL prognosis (overall survival) was further screened through LASSO and univariate Cox regression. Then, based on the GSE10846 dataset (414 DLBCL samples), a prognostic model was constructed based on the LLRDEGs. The Kaplan-Meier curve is used to evaluate the prognostic value (median risk score). And validate the prognostic model on an independent external validation dataset (GSE181063 (1310 DLBCL samples)). Finally, further analysis was conducted on consensus clustering, biological pathways, the immune microenvironment and drug sensitivity.</p><p><strong>Results: </strong>4 LLRDEGs were ultimately identified as genes associated with the DLBCL' prognosis. A prognostic model was constructed based on the 4 LLRDEGs. An independent external validation dataset confirmed the prognostic model's prognostic value. 4 LLRDEGs were significantly correlated with various immune cells.</p><p><strong>Discussion: </strong>This study screened four LLRDEGs significantly associated with prognosis and constructed a prognostic risk stratification model. The Kaplan-Meier curve confirms the prognostic value of the prognostic risk stratification model. This prognostic model is related to the clinical characteristics and immune microenvironment of DLBCL patients.</p><p><strong>Conclusion: </strong>This study elucidates the role of LLRDEGs in the prognosis and immune features of DLBCL, providing insights into potential therapeutic targets.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2662818"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147814484","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}
HematologyPub Date : 2026-12-31Epub Date: 2026-04-01DOI: 10.1080/16078454.2026.2653275
Anna Junkins, Wanqing Wen, Loren Lipworth, Xijing Han, Heather Munro, Michael T Mumma, Martha J Shrubsole, Wei Zheng, Eden Biltibo, Staci Sudenga
{"title":"Social determinants of health associated with multiple myeloma incidence and survival among a low-income cohort in the Southeastern U.S.","authors":"Anna Junkins, Wanqing Wen, Loren Lipworth, Xijing Han, Heather Munro, Michael T Mumma, Martha J Shrubsole, Wei Zheng, Eden Biltibo, Staci Sudenga","doi":"10.1080/16078454.2026.2653275","DOIUrl":"10.1080/16078454.2026.2653275","url":null,"abstract":"<p><strong>Objectives: </strong>Multiple myeloma (MM) is the second most common hematologic malignancy in the U.S.; however, the etiology is poorly understood. We investigated social determinants of health (SDoH) associated with MM incidence and survival among low-income Black and White participants in the Southern Community Cohort Study (SCCS).</p><p><strong>Methods: </strong>The SCCS enrolled participants aged 40-79 years from 12 Southeastern states. We examined associations between SDoH (residential racial segregation, neighborhood deprivation, population density, persistent poverty, and rurality) geocoded to zip code, with MM incidence and all-cause mortality using multivariable Cox regression analyses. Additional stratified analyses examined MM incidence by obesity status.</p><p><strong>Results: </strong>Among 74,294 participants, there were 162 MM cases, 133 self-identified as Black individuals and 29 self-identified as White individuals. Living in the highest vs. lowest deprivation areas was associated with 2-fold increased MM risk (HR:2.24; 95% CI:1.01-4.95). Among MM cases, those living in the least vs. most residentially segregated areas had 2-fold increased mortality (HR:2.21; 95% CI:1.03-4.74). Among participants who were not obese, those who lived in the most densely populated areas had a reduced risk of MM compared to those who lived in the least densely populated areas (HR:0.31; 95% CI: 0.14-0.67); and those who lived in urban vs. rural areas had a reduced risk (HR:0.52; 95% CI: 0.32-0.85).</p><p><strong>Discussion: </strong>SDoH factors including neighborhood deprivation and residential racial segregation could influence MM risk and survival among low-income populations.</p><p><strong>Conclusion: </strong>SDoH factors should be considered when developing strategies to reduce overall MM burden, and disparities among people with lower socioeconomic backgrounds.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2653275"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147591809","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":"Single-cell and bulk transcriptomic integration identifies a high-CNV, dark-zone-enriched malignant cell state associated with adverse prognosis in GCB-DLBCL.","authors":"Jianing Wang, Xiuying Xiang, Xiaomei He, Yun Pan, Zewei Jiang, Jiaxiong Tan","doi":"10.1080/16078454.2026.2740288","DOIUrl":"https://doi.org/10.1080/16078454.2026.2740288","url":null,"abstract":"<p><strong>Background: </strong>Germinal centre B-cell-like diffuse large B-cell lymphoma (GCB-DLBCL) generally has a better prognosis than activated B-cell-like DLBCL, yet a clinically significant subset of patients still develops aggressive disease, suggesting marked malignant cellular heterogeneity within this subtype. This study aimed to characterise malignant cell states in GCB-DLBCL and assess their prognostic relevance.</p><p><strong>Methods: </strong>Single-cell transcriptomic data were integrated with inferred copy-number variation (CNV) analysis to characterise malignant B-cell states. Regulatory programmes, functional features, and genetic associations were investigated using regulon analysis, functional profiling, and scPAGWAS. Single-cell-derived signatures were projected onto three independent bulk transcriptomic cohorts by deconvolution to evaluate clinical relevance. Integrative analyses were then performed to identify candidate molecular features associated with the M-C2 state.</p><p><strong>Results: </strong>Marked malignant-cell heterogeneity was identified. A high-CNV compartment displayed dark-zone-like transcriptional programmes, enrichment of proliferation- and survival-associated features, and reduced antigen-presentation capacity. Subclustering resolved three malignant states, among which M-C2 showed the highest inferred CNV burden and a distinct germinal-centre regulatory programme marked by enhanced BCL6, TCF3, and PAX5 activity. M-C2 was enriched for DLBCL susceptibility-associated signals, and its higher abundance was consistently associated with inferior overall survival across independent bulk cohorts. Integrative prioritisation identified an eight-gene M-C2-associated panel comprising IRF4, AKT1S1, PIEZO1, POLR1A, EML6, TTN, FCRL5, and IGHG2. An exploratory prognostic model integrating molecular and clinical features further supported the potential predictive value of this programme.</p><p><strong>Conclusions: </strong>This study identifies a high-CNV, dark-zone-enriched malignant cell state within GCB-DLBCL, represented by the M-C2 subpopulation, associated with impaired antigen presentation and adverse prognosis. This framework improves understanding of malignant heterogeneity and warrants further validation for risk stratification.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2740288"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149074587","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}
HematologyPub Date : 2026-12-31Epub Date: 2026-09-08DOI: 10.1080/16078454.2026.2720973
Maria Virginia Meza, Sarah Hodgkinson, Christian Stefan Eichinger, Faraz Zaman, Diana Boyko
{"title":"Frontline therapies for adult patients with newly diagnosed Philadelphia chromosome-negative B-cell acute lymphoblastic leukemia: a systematic literature review.","authors":"Maria Virginia Meza, Sarah Hodgkinson, Christian Stefan Eichinger, Faraz Zaman, Diana Boyko","doi":"10.1080/16078454.2026.2720973","DOIUrl":"10.1080/16078454.2026.2720973","url":null,"abstract":"<p><strong>Objectives: </strong>Philadelphia (Ph) chromosome-negative B-cell acute lymphoblastic leukemia (B-ALL) is the most common ALL in adults. Overall survival (OS) with frontline chemotherapy remains poor. Blinatumomab is currently the only targeted agent approved for frontline treatment.</p><p><strong>Methods: </strong>We systematically reviewed 96 studies (43 interventional, 53 observational) between 2012-2026 evaluating frontline pharmacologic therapy in adults with Ph- B-ALL.</p><p><strong>Results: </strong>Across chemotherapy studies, nearly half of patients relapsed within 3 years, and only 49%-69% survived beyond 3 years. Blinatumomab demonstrated robust and consistent efficacy in first complete response (CR1), supported by 2 randomized controlled trials (RCT) and 16 single-arm trials (SAT). In one RCT, blinatumomab reduced the risk of death by 59% versus chemotherapy alone (HR 0.41) when added to frontline consolidation in minimal residual disease (MRD) negative patients. Median OS reached 41.2 months in a SAT where blinatumomab monotherapy was administered to patients in MRD-positive CR1. SATs showed consistent efficacy outcomes regardless of age, MRD status, or chemotherapy backbone.</p><p><strong>Discussion: </strong>Additional targeted therapies still under investigation have shown mixed results.</p><p><strong>Conclusion: </strong>Frontline inotuzumab (±blinatumomab) plus chemotherapy showed promise in older populations, while the efficacy benefit of rituximab was inconclusive. No new safety signals were identified in the frontline setting for targeted therapies.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2720973"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148911337","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}
HematologyPub Date : 2026-12-31Epub Date: 2026-10-19DOI: 10.1080/16078454.2026.2749300
Xiazhe Li, Shan Song, Le Wang, Zhiyong Zhong, Yanwei Qi, Yingxin Gong
{"title":"Pacak-Zhuang syndrome with EPAS1 p.Pro531Leu somatic variant: one case report and literature review.","authors":"Xiazhe Li, Shan Song, Le Wang, Zhiyong Zhong, Yanwei Qi, Yingxin Gong","doi":"10.1080/16078454.2026.2749300","DOIUrl":"10.1080/16078454.2026.2749300","url":null,"abstract":"<p><strong>Objective: </strong>To report a case child of Pacak-Zhuang syndrome with <i>EPAS1</i> p.Pro531Leu somatic variant in a child and to summarize the clinical characteristics, diagnosis, treatment methods, and prognosis of this syndrome based on a literature review.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on the clinical data of a child girl with Pacak-Zhuang syndrome admitted to Hebei Children's Hospital in July 2019. Using the search terms 'Pacak-Zhuang syndrome', 'EPAS1', 'HIF-2α', 'HIF-2A', and 'Polycythemia-paraganglioma syndrome', relevant literature was retrieved from PubMed and Web of Science. The clinical manifestations, diagnostic methods, treatment plans, and outcomes of patients with this syndrome were analyzed.</p><p><strong>Results: </strong>A 4-year-old girl had recurrent hypertension, polycythemia and renal hilar masses. Diagnosis was confirmed by imaging, biochemical detection and paraffin tissue genetic testing. She received three operations for paragangliomas; pathology showed CgA(+), Syn(+), NSE(+) and Ki‑67 ≤5%. Follow-up revealed tumor recurrence, but the condition improved with oral temozolomide. Combined with 10 retrieved articles, 18 patients (4 males,14 females, mean age 25 years) were analyzed. Polycythemia was the cardinal manifestation. All patients received surgery, and 10 suffered uncontrolled tumor recurrence.</p><p><strong>Conclusion: </strong>Pacak-Zhuang syndrome is a rare disorder involving the neuroendocrine and hematopoietic systems, often initially presenting with 'polycythemia'. Most cases are sporadic, with no apparent familial inheritance or history of other neuroendocrine tumors. When encountering pediatric patients with unexplained hypertension accompanied by polycythemia symptoms, the possibility of this syndrome should be considered, and further imaging studies and genetic testing are required for definitive diagnosis.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2749300"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223898","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}
HematologyPub Date : 2026-12-31Epub Date: 2026-06-11DOI: 10.1080/16078454.2026.2682009
Carlos Vallejo, Carmen González, Anna Gaya, Silvia de la Iglesia, Mar Tormo, Carmen Benet, Esperanza Lavilla, Ana Fernández, David Benéitez, Santiago Bonanad, Fernando Carnicero, Bianca Díaz, Ataúlfo González, Ana Pilar González-Rodríguez, Inés Hernández, Jaime Martí, Virginia Melero, Marta Morado, Eduardo Salido, Piva Sánchez, Williana Torres, Míriam Vara, Julia Vidán, On Behalf Of Pethema Spain
{"title":"Real-world pegcetacoplan treatment of paroxysmal nocturnal hemoglobinuria patients with an unsatisfactory response to prior therapies.","authors":"Carlos Vallejo, Carmen González, Anna Gaya, Silvia de la Iglesia, Mar Tormo, Carmen Benet, Esperanza Lavilla, Ana Fernández, David Benéitez, Santiago Bonanad, Fernando Carnicero, Bianca Díaz, Ataúlfo González, Ana Pilar González-Rodríguez, Inés Hernández, Jaime Martí, Virginia Melero, Marta Morado, Eduardo Salido, Piva Sánchez, Williana Torres, Míriam Vara, Julia Vidán, On Behalf Of Pethema Spain","doi":"10.1080/16078454.2026.2682009","DOIUrl":"10.1080/16078454.2026.2682009","url":null,"abstract":"<p><strong>Objectives: </strong>Terminal complement inhibitors can control intravascular hemolysis in Paroxysmal Nocturnal Hemoglobinuria (PNH) patients, although the hematological response achieved may not persist, and it may become unsatisfactory. The proximal (C3) complement inhibitor Pegcetacoplan (PEG) improves this situation in clinical trials, yet real-world data on its efficacy and safety are scarce.</p><p><strong>Methods: </strong>This non-interventional, retrospective, multicenter study presents clinical and hematological data from the first PNH patients in Spain treated with PEG (<i>n</i> = 32, median 13 months, range 4-61).</p><p><strong>Results: </strong>PEG rapidly increases the patient's hemoglobin (median increase of 2.65 g/dL in 4 weeks, > 2 g/dL increase in 56.3% of patients), reaching > 8, 10 and 12 g/dL in 93.7%, 87.5% and 46.9% of patients, respectively. Packed red blood cell transfusion requirements dropped from 62.5% to 15.6% of patients treated with PEG, and there was a fall in breakthrough hemolysis from 34.4% to 9.4% after 6 months of treatment. No thrombosis and only mild adverse events (<i>n</i> = 11 patients) were described with PEG therapy. Finally, physicians perceived important enhancements in patients' health-related quality of life.</p><p><strong>Discussion: </strong>This real-world clinical data confirms that PEG produces significant and rapid improvements in hemoglobin and hemolytic parameters, with a favorable safety profile. The data extends that of earlier clinical trials and small real-world studies by offering a longitudinal (as opposed to cross-sectional) assessment of the patient responses across a large number of centers, following a variety of clinical practices..</p><p><strong>Conclusion: </strong>PEG treatment of patients with PNH produces significant and rapid improvements in hemoglobin and hemolytic parameters, with a favorable safety profile.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2682009"},"PeriodicalIF":1.6,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148224843","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}
HematologyPub Date : 2026-12-31Epub Date: 2026-04-03DOI: 10.1080/16078454.2026.2653404
Liling Lu, Shenglan Lin, Yating Chen, Yiqun Huang
{"title":"Temporal trends in second primary malignancies among long-term survivors of multiple myeloma across treatment eras: a population-based analysis of the SEER database.","authors":"Liling Lu, Shenglan Lin, Yating Chen, Yiqun Huang","doi":"10.1080/16078454.2026.2653404","DOIUrl":"10.1080/16078454.2026.2653404","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate temporal trends in second primary malignancy (SPM) risk among long-term multiple myeloma (MM) survivors across treatment eras.</p><p><strong>Methods: </strong>This retrospective cohort study utilized the SEER 17 registries database (1975-2022). Patients with MM as first primary malignancy surviving ≥5 years were stratified into four treatment eras: Era 1 (≤2000, pre-novel agents), Era 2 (2001-2010, proteasome inhibitor/IMiD introduction), Era 3 (2011-2015, new-generation IMiDs), and Era 4 (2016+, daratumumab era). SPM was defined as any malignancy occurring ≥5 years post-diagnosis. Cumulative incidence was estimated using competing risk analysis.</p><p><strong>Results: </strong>Among 52,497 MM patients, 15,402 (29.34%) achieved 5-year survival. Overall, 1,408 patients (9.14%) developed SPM over 72,951.70 person-years. SPM rates declined significantly across eras: 11.43% (Era 1), 10.45% (Era 2), 7.78% (Era 3), and 2.45% (Era 4) (P<i>trend</i> < 0.001). The most common SPMs were prostate (13.81%), lung (10.17%), and breast cancer (7.42%). Acute myeloid leukemia (6.78%) remained notable among hematologic malignancies. Sensitivity analysis among 10-year survivors confirmed the declining trend.</p><p><strong>Conclusions: </strong>SPM rates progressively declined from Era 1 through Era 3, with encouraging early signals in Era 4 requiring longer follow-up. The shift from alkylating agents to targeted therapies may contribute to reduced SPM occurrence, providing preliminary evidence for improved long-term safety of contemporary MM treatments.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2653404"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147607592","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":"Impact of defibrotide on survival in sinusoidal obstruction syndrome / veno-occlusive disease: a retrospective cohort study comparing two sequential treatment eras.","authors":"Kengo Katsuki, Takayoshi Tachibana, Akihiko Izumi, Jun Nukui, Shota Arai, Natsuki Hirose, Hiroaki Konishi, Takaaki Takeda, Shuku Sato, Yotaro Tamai, Katsumichi Fujimaki, Koh Yamamoto, Ryuji Ishii, Tetsuya Fukuda, Tetsuzo Tauchi, Masatsugu Tanaka, Hideaki Nakajima","doi":"10.1080/16078454.2026.2721241","DOIUrl":"https://doi.org/10.1080/16078454.2026.2721241","url":null,"abstract":"<p><strong>Background: </strong>Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a serious endothelial injury syndrome following allogeneic hematopoietic stem cell transplantation (allo-HSCT) requiring early diagnosis and treatment. We retrospectively evaluated clinical characteristics, treatments, and outcomes of patients who developed SOS/VOD after allo-HSCT.</p><p><strong>Methods: </strong>We included all consecutive patients who developed SOS/VOD after allo-HSCT between January 2010 and October 2023. SOS/VOD was diagnosed according to Seattle, Baltimore, or EBMT criteria, and severity was classified using the EBMT criteria. Patients treated with recombinant thrombomodulin (rTM) between January 2010 and August 2019 were compared with those treated with defibrotide between September 2019 and October 2023.</p><p><strong>Results: </strong>Among 755 cases, 48 developed SOS/VOD: 15 treated with rTM and 33 with defibrotide. Median ages were 50 years in rTM group and 55 years in defibrotide group, and median days to onset were 13 and 17, respectively. There was no significant difference in disease stage at transplantation or SOS/VOD severity at diagnosis. At 100 days post-onset, overall survival (OS), non-relapse mortality, and SOS/VOD mortality were 20.0% vs 46.9% (<i>p</i> = 0.17), 66.7% vs 54.5% (<i>p</i> = 0.609), and 46.7% vs 31.2% (<i>p</i> = 0.364) in the rTM and defibrotide groups, respectively. In multivariate analysis adjusted for age and conditioning intensity, defibrotide treatment was associated with improved OS (hazard ratio, 0.41; 95% confidence interval, 0.18-0.92, <i>p</i> = 0.032). Review of most severe cases suggested an association between inflammatory status and the clinical course of SOS/VOD.</p><p><strong>Conclusion: </strong>Defibrotide treatment was associated with improved outcome in SOS/VOD. Inflammatory status appeared to be related to disease onset and resolution.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2721241"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148919126","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}
HematologyPub Date : 2026-12-31Epub Date: 2026-07-08DOI: 10.1080/16078454.2026.2700818
Sufei Kuang, Qixin Liu
{"title":"Integrated multi-omics analysis identifies prognostic risk genes and constructs a predictive signature in diffuse large B-cell lymphoma.","authors":"Sufei Kuang, Qixin Liu","doi":"10.1080/16078454.2026.2700818","DOIUrl":"https://doi.org/10.1080/16078454.2026.2700818","url":null,"abstract":"<p><strong>Background: </strong>Diffuse large B-cell lymphoma (DLBCL) presents a complex etiology and challenging diagnosis. This study aims to investigate potential pathogenic genes.</p><p><strong>Methods: </strong>We identified DLBCL risk genes (DRGs) through expression quantitative trait loci-Mendelian randomization (eQTL-MR). Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) analyses were conducted to explore biological functions. Single-cell RNA sequencing (scRNA-seq) data were analyzed to delineate the subcellular localization. Immune infiltration analyses examined the role of genes in the DLBCL immune microenvironment. Finally, drug sensitivity analyses were performed to predict potentially sensitive drugs.</p><p><strong>Results: </strong>Following eQTL-MR and prognostic analyses, we identified 15 genes associated with both the pathogenesis and prognosis of DLBCL. These genes were successfully integrated into a risk gene model, achieving an Area Under the Curve (AUC) of 0.787. GO and KEGG enrichment analyses of genes localized significant pathways, including NF-κB signal transduction. ScRNA-seq analysis suggested that DRGs may be linked to the immune microenvironment of DLBCL. Further immune infiltration analysis confirmed the pivotal role of immune infiltration in the malignant progression of DLBCL.</p><p><strong>Conclusion: </strong>This study unveils 15 risk genes as potential pathogenic and therapeutic biomarkers for DLBCL. These findings provide novel insights and targets for understanding the pathogenesis, diagnosis, and treatment of DLBCL.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2700818"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148411638","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":"Case report: persistent anemia after eculizumab in paroxysmal nocturnal hemoglobinuria: non-dominantly active intravascular hemolysis.","authors":"Baozhi Fang, Xiao Yu, Yifei Zhou, Qiudan Shen, Muzhi Yuan, Peng Wang, Mingen Lü, Guangsheng He","doi":"10.1080/16078454.2026.2647316","DOIUrl":"10.1080/16078454.2026.2647316","url":null,"abstract":"<p><strong>Objectives: </strong>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare condition characterized by intravascular hemolysis (IVH), thrombosis, and organ damage. Although C5 complement inhibitors such as eculizumab have significantly improved the prognosis of patients with PNH, persistent anemia remains in some patients.</p><p><strong>Methods: </strong>We report a patient with PNH who initially responded to eculizumab but experienced recurrent hemoglobin decrease and became transfusion-dependent after 24 weeks of treatment. Here, we present the clinical features of this patient. In addition, we reviewed the current literature to characterize persistent anemia caused by non-dominant residual IVH following C5 inhibitor therapy in PNH, along with related biomarker assessment and clinical applications of proximal complement inhibitors.</p><p><strong>Results: </strong>Lactate dehydrogenase (LDH) levels did not significantly increase, and the direct antiglobulin test (DAT) with anti-C3d was only weakly positive (titre 1:16). The changes in free hemoglobin, haptoglobin, and type III red blood cells (RBC) (total loss of CD59) indicated subclinical yet active IVH. After the introduction of iptacopan monotherapy for six weeks, the levels of LDH, free hemoglobin, and haptoglobin normalized, and the need for further transfusions was eliminated. The proportion of type III RBC increased to 57.3%.</p><p><strong>Conclusion: </strong>This case suggests that persistent anemia in patients with PNH following C5 inhibitor therapy might be associated with subclinically active IVH, and proximal complement inhibitors, such as iptacopan, may offer effective management of this condition.</p>","PeriodicalId":13161,"journal":{"name":"Hematology","volume":"31 1","pages":"2647316"},"PeriodicalIF":2.0,"publicationDate":"2026-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147485673","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}