Expert Review of Hematology最新文献

{"title":"Association between sickle cell disease and autoimmune diseases in Saudi population: a single center study.","authors":"Rehab Y Al-Ansari, Alexander Woodman, Hana Ibrahim Al Daajani, Mohammad Ibrahim Aljamily, Tawasoul Fadoul, Elham Hamid Alfallaj, Khaled Abdullah Hassan, Nada Rajab Al Zahrani, Zechariah Jebakumar Arulanantham, Mohammed Al Ghamdi","doi":"10.1080/17474086.2025.2534713","DOIUrl":"https://doi.org/10.1080/17474086.2025.2534713","url":null,"abstract":"<p><strong>Background: </strong>The association between sickle cell disease (SCD) and autoimmune diseases (AID) is not well understood. This retrospective study aims to investigate the frequency of coexistence of AIDS with SCD.</p><p><strong>Research design and methods: </strong>This study was conducted in a tertiary care hospital. Data of <i>n</i> = 168 SCD patients between January 2016 and March 2023 were extracted and analyzed. Data included demographics, medications, hospitalization, phenotypes, presence of AIDs, and laboratory data.</p><p><strong>Results: </strong>The mean age of <i>n</i> = 168 SCD patients was 30.66 (SD ± 11.27), with 54.2% of cases being female. 88.56% of patients had HGBSS phenotyping. The incidence of SCD cases with negative AID was 84.52%, while incidence of SCD cases with positive AID was 15.47% (<i>p</i> = 0.00001). Systemic lupus erythematosus (SLE, 4.2%), hypothyroidism (3.6%), and antiphospholipid syndrome (APS, 3.6%) were the most common SCD-associated AIDs. In addition, 69.2% of AIDs-positive patients used hydroxyurea. A strong correlation was found between hospitalization rate and a positive AID (<i>p</i> = 0.03).</p><p><strong>Conclusions: </strong>The result of this study represents the significant rate of AID and SCD coexistence. The findings require further study to substantiate the need to develop recommendations for screening and early detection of the SCD-associated AIDs.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144625715","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":"A systematic review of histological characteristics in arterial and venous thrombi.","authors":"Gurtej Singh, Shiffoni Sukhlal, Isha Joshi, Shang Lee, Nicholas Sikalas, Jose A Diaz, Nicos Labropoulos","doi":"10.1080/17474086.2025.2533237","DOIUrl":"https://doi.org/10.1080/17474086.2025.2533237","url":null,"abstract":"<p><strong>Introduction: </strong>Despite being the leading causes of morbidity and mortality worldwide, very little is known about the similarities and differences between venous and arterial thrombi. This review is focused on comparing their structural, molecular, and temporal characteristics.</p><p><strong>Methods: </strong>A systematic review following PRISMA guidelines and focusing on arterial and venous thrombi was conducted. Only studies having histological images <i>in vivo</i> from animal studies and humans were included. Data on structural components and temporal changes of thrombi were collected and analyzed.</p><p><strong>Results: </strong>There were 76 articles found eligible from the full-text review. Only two studies had simultaneous temporal and spatial comparisons and did not attempt to compare the two types of thrombi: arterial and venous. Therefore, comparative insights were additionally drawn from studies analyzing one thrombus type in isolation. Comparisons were made for components commonly studied in both types of thrombi. Four common factors were identified: red blood cells (RBCs), white blood cells (WBCs), platelets, and fibrin. Platelet concentration was higher in arterial thrombi, while more red blood cells (RBCs) were found in the venous thrombi. The number of WBCs and fibrin concentration varied over time on either type of thrombi. Neovascularization and lysis were identified in both types of thrombi, with the latter being more common in venous thrombosis. Several factors, such as blood flow velocity, cryptogenic thrombi, and coexistence of red and white thrombi, complicated this delineation. Precise molecular and cellular quantification and a more detailed analysis of both arterial and venous thrombi temporal and spatial profiles, along with a standardized methodology, were not reported.</p><p><strong>Conclusions: </strong>There is a clear lack of direct comparison between arterial and venous thrombi, with limited information on their evaluations. Most available findings are derived from independently conducted analyses. Further work is needed to define these thrombi better and enhance our understanding of their short- and long-term remodeling to improve outcomes.</p><p><strong>Registration: </strong>The protocol was registered on PROSPERO (registration ID: CRD420251003712).</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144625714","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":"Incorporating novel therapies into the treatment of older patients with classic Hodgkin lymphoma.","authors":"Aditya Ravindra, Eric Mou","doi":"10.1080/17474086.2025.2526685","DOIUrl":"10.1080/17474086.2025.2526685","url":null,"abstract":"<p><strong>Introduction: </strong>Outcomes of older patients with classic Hodgkin lymphoma (cHL) are inferior to those of younger patients secondary to their distinct clinical presentation, unique disease biology, increased burden of medical comorbidity, and poorer tolerance to conventional therapies. Greater attention to the objective parameters underlying patient fitness has led to the recognition of comprehensive geriatric assessments (CGA) as an important method to optimize selection for appropriate therapy intensity. Given the magnified risk associated with traditional chemotherapy in older patients with cHL, the incorporation of the novel agents brentuximab vedotin (BV) and immune checkpoint inhibitors (ICIs) into modern treatment paradigms offers promise for improved outcomes in this population.</p><p><strong>Areas covered: </strong>We describe historical outcomes in older patients with cHL, the underpinnings of traditional treatment strategies, the evolving therapeutic landscape via integration of novel therapies into the frontline and relapsed/refractory settings, and the importance of contextualizing therapy selection via formal CGAs.</p><p><strong>Expert opinion: </strong>Novel therapies have broadened the array of therapeutic options for older patients with cHL eligible for either curative or palliative intent therapy. Further investigation into rational combinations of these drugs, together with ongoing efforts to validate cHL-specific CGAs, aim to improve outcomes for older patients across the spectrum of fitness.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":"1-17"},"PeriodicalIF":2.3,"publicationDate":"2025-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144505312","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":"t(4;14), and revised myeloma comorbidity index as good predictors of survival for multiple myeloma.","authors":"Aline G Ramirez-Alvarado, Susana Gabriela Gonzalez-Prieto, Yael Solis-Aranda, Ana Paulina Rivera-Espinoza, Jaime Garcia-Chavez, Laura Arcelia Montiel-Cervantes, Jorge Vela-Ojeda","doi":"10.1080/17474086.2025.2534706","DOIUrl":"https://doi.org/10.1080/17474086.2025.2534706","url":null,"abstract":"<p><strong>Background: </strong>Cytogenetic tests are essential prognostic indicators for patients diagnosed with multiple myeloma (MM). The study aimed to evaluate the prevalence of cytogenetic abnormalities and their prognostic significance in patients with newly diagnosed MM.</p><p><strong>Research design and methods: </strong>A cohort study involving 88 cases. Malignant plasma cells were isolated, and interphase fluorescent in situ hybridization was performed on bone marrow samples.</p><p><strong>Results: </strong>The gain of 1q was observed in 17 (19%) patients, followed by t(4;14) in 10 (11.5%), and the 17p or P53 mutation was found in only 6 (7%) patients. Three patients (3.5%) exhibited t(14;16). Amplification of 1q was not detected in any of the samples. The presence of t(4;14), anemia, renal disease, a revised myeloma comorbidity index (R-MCI) of 7-9, and a lack of treatment response were associated with poor progression-free survival. Additionally, t(4;14), anemia, elevated LDH, an R-MCI of 7-9, and absence of maintenance treatment correlated with decreased overall survival. In the Cox regression analysis, the presence of t(4;14) and an R-MCI of 7-9 were the most significant factors predicting worse outcomes.</p><p><strong>Conclusions: </strong>The t(4;14) and RMCI are reliable predictors of poor survival in newly diagnosed MM patients.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144616861","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":"Factor first! Emergency department management of persons with hemophilia: a single-center study.","authors":"Kelsey Uminski, Natalia Rydz, Dawn Goodyear","doi":"10.1080/17474086.2025.2534704","DOIUrl":"https://doi.org/10.1080/17474086.2025.2534704","url":null,"abstract":"<p><strong>Background: </strong>Timely hemostatic therapy is essential when persons with hemophilia (PwH) present to the emergency department (ED), with guidelines recommending treatment at the time a bleed is suspected ('Factor First' strategy).</p><p><strong>Research design and methods: </strong>This study examined ED management of PwH in Southern Alberta from 2014 to 2022, focusing on adherence to guidelines.</p><p><strong>Results: </strong>A total of 393 ED visits from 191 adults with hemophilia A or B were identified. Most visits (86%) required emergent or urgent care. Median times from ED registration to ordering and administering hemostatic therapy were 2.9 and 4.2 hours, respectively, with treatment given within 2 hours in a minority of cases. Laboratory tests were ordered in nearly half of visits, with a median ordering time of 65 minutes. Use of individualized emergency management protocols increased over time, reaching 91.4% in 2022. Median ED stay was 3.4 hours, and approximately 25% of visits had a hemophilia-related admission diagnosis.</p><p><strong>Conclusions: </strong>These findings highlight persistent delays and nonadherence to best practice recommendations, emphasizing the need for targeted quality improvement interventions to ensure timely, guideline-concordant care for PwH in emergency settings.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144616860","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":"Mendelian randomization combined with transcriptomic data reveals LINC00652 as a protective factor against the risk of acute lymphoblastic leukemia.","authors":"Haiyan Qi, Jianping Lan, Wensong Wang, Xiaogang Wang","doi":"10.1080/17474086.2025.2528886","DOIUrl":"10.1080/17474086.2025.2528886","url":null,"abstract":"<p><strong>Background: </strong>LINC00652 plays pivotal roles in acute lymphoblastic leukemia (ALL) a heterogeneous hematological malignancy. Here, we investigated the potential regulatory mechanisms of LINC00652 in ALL.</p><p><strong>Research design and methods: </strong>Genome-wide association study data for LINC00652 were obtained for Mendelian randomization and integrated with RNA sequencing data, Bioinformatics analyses, including weighted gene co-expression network analysis (WGCNA), Spearman or Pearson correlation analysis, function, immune microenvironment, subcellular localization, and competing endogenous RNA (ceRNA) network construction, were employed explore the potential mechanisms of LINC00652 in ALL.</p><p><strong>Results: </strong>LINC00652 exhibited no significant causal relationship with ALL but was identified as a protective factor (<i>p</i> > 0.05). LINC00652 levels were significantly decreased in patients with ALL, while WGCNA demonstrated the significance of black and blue modules for ALL. Seventy-nine messenger RNA (mRNAs) (SYT7) significantly correlated with LINC00652 (<i>p</i> > 0.05) and were enriched in the Janus kinase/signal transducer and activator of transcription (STAT), interleukin 2-STAT5, apoptosis, and adipogenesis pathways. Twenty-eight immune cells infiltrated the ALL, with immature B-cells and gamma delta T-cells significantly associated with LINC00652 expression (<i>p</i> < 0.05). LINC00652 was predominantly cytoplasmic, and a ceRNA network involving LINC00652, 36 microRNAs, and 18 mRNAs were proposed.</p><p><strong>Conclusions: </strong>LINC00652 May protect against ALL and influence its pathogenesis through ceRNA mechanisms.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":"1-14"},"PeriodicalIF":2.3,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144539710","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":"How will genomic testing impact a clinician's choice for managing chronic myeloid leukemia?","authors":"Jessica Sia, Naranie Shanmuganathan","doi":"10.1080/17474086.2025.2533235","DOIUrl":"https://doi.org/10.1080/17474086.2025.2533235","url":null,"abstract":"<p><strong>Introduction: </strong>Chronic myeloid leukemia (CML) is a disease characterized by a single diagnostic molecular abnormality and epitomizes a genetically based diagnosis and management. Targeted therapy with tyrosine kinase inhibitors has revolutionized treatment and prognosis of this disease; however, a proportion of patients will experience resistance to therapy and progress to blast phase, the terminal phase of this disease. Emerging genomic profiling in CML reveals a genetically heterogenous landscape which may permit further risk stratification, personalization of treatment, and the potential for drug development.This review encompasses original literature exploring the genomic profile of CML and the potential impact of additional genomic abnormalities on prognosis and treatment response.</p><p><strong>Expert opinion: </strong>Somatic variants in cancer-associated genes, particularly <i>ASXL1</i>, are of prognostic and potential therapeutic significance in CML. Up-front next-generation sequencing is therefore useful when available in all patients with newly diagnosed CML and repeat testing should be considered at timepoints of suboptimal treatment response, TKI resistance, and progression. Gene rearrangements and fusions are an emerging class of mutation that may confer adverse prognostic risk and hence use of a robust testing method that enables detection of such abnormalities is desirable.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144599892","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":"Von Willebrand factor is a multifaceted player in hemostasis requiring a diverse array of analytical and diagnostic approaches.","authors":"François Depasse, Nikolaus B Binder, Mariona Bono, Matthias Germer, Michael Heins, Martina Leplatoni, Jürgen Patzke, Stephan Schwers, Michael Spannagl, Peter L Turecek","doi":"10.1080/17474086.2025.2525458","DOIUrl":"10.1080/17474086.2025.2525458","url":null,"abstract":"<p><strong>Introduction: </strong>Von Willebrand factor (VWF) plays a crucial role in hemostasis: its interactions with endothelial matrices, platelets, and factor VIII make it a key player in both primary hemostasis and coagulation. Pathology associated with VWF spans mild to severe bleeding manifestations in the case of inherited von Willebrand disease (VWD), the most common congenital bleeding disorder, or acquired von Willebrand syndrome (AVWS). Conversely, VWF can be associated with thrombotic manifestations in situations related to inflammation, infection, or inherited or acquired ADAMTS13 defects.</p><p><strong>Areas covered: </strong>This review article aims to provide guidance on the use and interpretation of clinical laboratory assays available to measure VWF and other factors related to VWD. Different VWF tests can be used in different clinical settings for efficient diagnosis and patient management. Assay limitations are also addressed.</p><p><strong>Expert opinion: </strong>A myriad of laboratory assays, from first line to esoteric assays, exist to enable adequate diagnosis of VWD and other diseases influenced by VWF. Clinical investigations of VWF are complicated because VWF has multiple functions which variably depend on the patient's pathophysiological status. The right choice of assays is therefore critical to provide adequate diagnosis in due time and with reasonable analytical efforts.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":"1-18"},"PeriodicalIF":2.3,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144483756","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":"Review of laboratory methods used for analysis of von Willebrand factor and for diagnosis of related diseases.","authors":"Jürgen Patzke, Nikolaus B Binder, Mariona Bono, François Depasse, Matthias Germer, Michael Heins, Martina Leplatoni, Stephan Schwers, Michael Spannagl, Peter L Turecek","doi":"10.1080/17474086.2025.2525461","DOIUrl":"https://doi.org/10.1080/17474086.2025.2525461","url":null,"abstract":"<p><strong>Introduction: </strong>Von Willebrand factor (VWF) is a large multimeric protein present in the blood. Its most important and best characterized function is to control bleeding in primary hemostasis, which is triggered by different biophysical mechanisms and protein-receptor interactions involving different domains of VWF. Many different diseases related to VWF, most importantly von Willebrand disease comprising different types and sub-types, require diagnosis, laboratory analysis of concentration, and function of VWF.</p><p><strong>Areas covered: </strong>As several different specific functions of VWF are physiologically relevant, several different assays are needed. The aim of this article is to provide a comprehensive overview of all relevant assays together with a description of how each assay is technically designed. Furthermore, guidance is given for choosing the right and validated assay methods.</p><p><strong>Expert opinion: </strong>This information includes technical requirements, analytical performance data, references to relevant guidelines and other guidance documents, and reference standards. Also, information on availability and the type of the assay is provided, such as automated or manual, in vitro diagnostics or research use only. Relation to the clinical use of these assays, as well as performance, and result interpretation is covered in a second article in the same issue of this journal by the same authors.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":"1-15"},"PeriodicalIF":2.3,"publicationDate":"2025-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559617","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":"Predictive strategies for poor outcomes after allotransplantation in patients with acute myeloid leukemia: what are the options and when should clinicians use them?","authors":"Xiao-Su Zhao, Ying-Jun Chang","doi":"10.1080/17474086.2025.2528887","DOIUrl":"https://doi.org/10.1080/17474086.2025.2528887","url":null,"abstract":"<p><strong>Introduction: </strong>Allotransplantation can provide a curative option for many AML patients. Over the past decade, an expanding array of predictive tools has been effectively used to identify high-risk patients with poor outcomes, enabling personalized treatment strategies. These advances have made precision medicine a reality for AML patients undergoing allogeneic transplantation, ultimately improving survival outcomes. The published articles were searched from Web of science with the keywords of 'stem cell transplantation' or 'bone marrow transplantation' (subject) and 'acute myeloid leukemia.'</p><p><strong>Areas covered: </strong>We first discussed the predictive biomarkers for poor outcomes in AML patients after allotransplantation. We also summarized the clinical utility of biomarkers in guiding optimal donor selection, conditioning regimen optimization, as well as prophylaxis, maintenance, and preemptive therapy for transplantation complications in AML patients, with a primary focus on leukemia relapse and GVHD. we highlighted the challenges and future directions in the field of biomarker-directed transplantation complication prediction and therapy.</p><p><strong>Expert opinion: </strong>Advances in biomarker-based prediction of transplant complications, prophylactic strategies, and preemptive therapy have made precision medicine a reality for AML patients. Future efforts should focus on identifying novel biomarkers to develop new techniques for predicting poor outcomes and guiding personalized prophylaxis and therapy.</p>","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144539711","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}