Turkish journal of haematology : official journal of Turkish Society of Haematology最新文献

{"title":"Ethical Discourse of Transplant Team Members on Adult Allogeneic Hematopoietic Stem Cell Donors: A Multicenter Qualitative Study by the Donor Research Team (DART) of the Turkish Society of Hematology.","authors":"Şükrü Keleş, Hasan Fatih Çakmaklı, İbrahim Eker, Gülay Sezgin, Mustafa Baydar, İlknur Pamuk, Zübeyde Nur Özkurt, Meltem Kurt Yüksel, Elif İnce, Can Boğa","doi":"10.4274/tjh.galenos.2026.81542","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.81542","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to analyze the views and experiences of transplant team members working at adult allogeneic hematopoietic stem cell transplantation centers regarding related and unrelated donors. This donor-centered study is expected to contribute to a deeper understanding of the ethical issues encountered during the transplantation process.</p><p><strong>Materials and methods: </strong>This study used a qualitative research method. As part of the study, individual interviews were conducted with 28 transplant team members (13 physicians and 15 transplant team members with various responsibilities in the transplantation process) working at seven transplantation centers in six cities across Türkiye. The data obtained were evaluated using thematic content analysis.</p><p><strong>Results: </strong>Based on individual interviews with transplant team members, the research data were clustered into the following themes: (1) ethical principles and core values in the transplantation process; (2) obtaining informed consent from donors; (3) donor safety; (4) ethical issues and proposed solutions in the transplantation process; and (5) the scope and limitations of clinical ethics consultation in the transplantation process.</p><p><strong>Conclusion: </strong>Three key implications emerged from this study. The first concerns the importance of establishing and maintaining a trust-based relationship between the transplant team and the donor, even after the transplantation process. Another point relates to donor advocacy. Transplant team members have diverse duties and responsibilities throughout the transplant process. The final point concerns the need for clinical ethics consultation to support transplant team members when they encounter ethical dilemmas.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149027479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"From Answers to Agents: What Must Change Before Generative and Agentic AI Become Clinical Infrastructure in Hematology.","authors":"Hilmi Erdem Gözden","doi":"10.4274/tjh.galenos.2026.82621","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.82621","url":null,"abstract":"<p><p>Generative artificial intelligence in hematology is entering a new phase. The dominant question, whether large language models are accurate enough for clinical decision support, is being overtaken by a harder one, as systems shift from answering questions to acting: extracting structured cases, routing them, classifying variants, and grounding recommendations in guidelines and case memory. In 2026, a hematology agent achieved roughly 83% concordance with tumor-board decisions in a prospective silent trial, with hallucinations in 0.3%, suggesting that for well-structured tasks the binding constraint is shifting from accuracy to governability. Moving from experimental support to reliable infrastructure requires four changes: validating trajectories rather than answers; allocating autonomy inversely to case complexity; treating deployed models as regulated instruments under continuous surveillance; and engineering against automation bias, with data-privacy and consent safeguards throughout. For hematologists, this reframes AI adoption as a governance and human-factors program rather than a search for a better model.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149013193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"First Experience of TCRαβ⁺ Depleted Haploidentical Hematopoietic Stem Cell Transplantation in a Child with a <i>TINF2</i>-Related Telomere Biology Disorder.","authors":"Merve Tellioğlu Sarıaslan, Ebru Yılmaz, Veysel Gök, Alper Özcan, Ekrem Ünal, İbrahim Boğa, Musa Karakükçü","doi":"10.4274/tjh.galenos.2026.29939","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.29939","url":null,"abstract":"","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148993550","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Characteristics and Surgical Outcomes of Retroperitoneal Unicentric Castleman Disease: A Single-Center Retrospective Study.","authors":"Mengchao Wei, Siyuan Li, Lu Zhang, Jie Dong","doi":"10.4274/tjh.galenos.2026.79353","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.79353","url":null,"abstract":"<p><strong>Objective: </strong>We aimed to characterize the inflammatory and complication profiles of patients with retroperitoneal unicentric Castleman disease (UCD), compare the clinical features of UCD with an MCD-like inflammatory state (UCD-MIS) and non-MIS cases, and describe treatment patterns and follow-up outcomes.</p><p><strong>Materials and methods: </strong>We retrospectively analyzed 75 patients with retroperitoneal UCD treated at our institution between 2000 and 2025, focusing on inflammatory phenotypes, severe complications, surgical extent, and follow-up outcomes.</p><p><strong>Results: </strong>UCD-MIS was identified in 20 patients (26.7%). Paraneoplastic pemphigus (PNP) occurred in 9 patients (12.0%), bronchiolitis obliterans (BO) in 8 (10.7%), and amyloid A (AA) amyloidosis in 2 (2.7%). Patients with UCD-MIS more frequently had PNP (35.0% vs. 3.6%; FDR-adjusted P = 0.004) and BO (25.0% vs. 5.5%; FDR-adjusted P = 0.042) than those without MIS. For treatment, 61 patients underwent surgery, including 57 complete resections. During a median follow-up of 60.8 months (range, 6.1-266.2 months), two deaths occurred. Both patients had PNP and met the criteria for UCD-MIS, and one also had BO. Neither patient had undergone surgical excision of the UCD lesion. The small number of deaths precluded between-group survival comparisons.</p><p><strong>Conclusion: </strong>Comprehensive screening for complications is essential in retroperitoneal UCD, and complete surgical excision remains the cornerstone of therapy when technically feasible.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148979328","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Concordance of Flow Cytometry and Real-Time Quantitative Polymerase Chain Reaction for Measurable Residual Disease Detection in Childhood B-Cell Acute Lymphoblastic Leukemia: A Real-Life Data Analysis.","authors":"Hasan Fatih Çakmaklı, Nübar Mustafayeva, Klara Dalva, Rolf Köhler, Elif Ince, Talia İleri, Beyza Doğanay, Mehmet Ertem","doi":"10.4274/tjh.galenos.2026.98475","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.98475","url":null,"abstract":"<p><strong>Objective: </strong>Measurable residual disease (MRD) is the cornerstone of risk-adapted therapy in childhood B-cell acute lymphoblastic leukemia (B-ALL). In Türkiye, MRD assessment relies on flow cytometry (FCM), whereas molecular MRD is not routinely available. We aimed to evaluate the concordance between an experienced FCM laboratory and an international EuroMRD reference real-time quantitative polymerase chain reaction (RQ-PCR) laboratory and its potential impact on treatment.</p><p><strong>Patients, materials and methods: </strong>In this real-world study, 85 paired bone marrow samples from 46 children with B-ALL were analyzed simultaneously for MRD by FCM and RQ-PCR. MRD positivity was defined as ≥1×10⁻⁴. Qualitative agreement was evaluated by concordance and Cohen's kappa coefficient. Diagnostic performance of FCM was evaluated using RQ-PCR as the reference method.</p><p><strong>Results: </strong>Qualitative concordance between FCM and RQ-PCR was 87.1%, indicating moderate agreement (κ=0.64, 95% CI 0.45-0.83). Concordance was 83% at the end of induction (EOI) (κ=0.58) and 93% at the end of consolidation (EOC) (κ=0.71). FCM showed excellent specificity (96.8%), but modest sensitivity (60.9%). Reliance on FCM alone would have altered MRD-guided treatment allocation in 17% of patients at EOI and 7% at EOC, mainly because of false-negative results.</p><p><strong>Conclusion: </strong>FCM-MRD performed in an experienced laboratory demonstrated moderate agreement with an international EuroMRD reference laboratory but failed to detect some RQ-PCR-MRD positivite cases, potentially affecting treatment decisions. These findings highlight the need for continuous quality assurance, national reference laboratories, dedicated MRD laboratory teams with long-term continuity, and complementary use of molecular MRD techniques alongside FCM to standardize MRD assessment.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963997","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comprehensive Genetic Analysis of Hemophilia A in a Chinese.","authors":"Yuling Xu, Xianli Qin, Yuan Long, Fuyong Zhang, Na Sun, Zhenfang Liu, Peng Cheng","doi":"10.4274/tjh.galenos.2026.45822","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.45822","url":null,"abstract":"<p><strong>Objective: </strong>Hemophilia A is a congenital bleeding disorder caused by <i>F8</i> gene variants, leading to factor Ⅷ deficiency. Genetic testing has become essential for precise diagnosis and personalized treatment strategies. However, the detection of <i>F8</i> gene variants remains challenging due to their heterogeneity. This study aims to investigate the diagnostic significance of next-generation sequencing (NGS) and long-read sequencing (LRS) in hemophilia A patients.</p><p><strong>Methods: </strong>This study included 118 hemophilia A patients. A two-step strategy was adopted: NGS was first used to screen for <i>F8</i> gene variants, and LRS or long-distance PCR (LD-PCR) was subsequently performed in cases with negative NGS results. LRS or LD-PCR was specifically employed to detect complex structural variants.</p><p><strong>Results: </strong>NGS identified pathogenic <i>F8</i> variants in 74 of 118 (62.7%) hemophilia A patients, while the remaining 44 (37.3%) tested negative. Among the 74 positive cases, 53 carried single-nucleotide variants (SNVs), including 7 novel SNVs; 9 had small deletions, 5 had large deletions, and 7 had duplications or insertions. Inversion analysis detected intron 22 inversions in 44 patients, with LD-PCR confirming 38 cases and LRS subclassifying the other 6 as type I. No type II inversions were detected.</p><p><strong>Conclusion: </strong>In summary, this study offers novel strategies for hemophilia A genetic diagnosis, confirming that NGS alone is inadequate and requires complementary methods in specific cases. Our results highlight the utility of LRS in comprehensively identifying various <i>F8</i> gene variants, particularly in precisely subtyping intron 22 inversions.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148932307","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transcriptomic Characterization of TCR-NK Cells Reveals Challenges Associated with CD3ζ Overexpression.","authors":"Tolga Sütlü, Elif Çelik, Betül Çakıcı, Serra Özarı, Mertkaya Aras","doi":"10.4274/tjh.galenos.2026.93902","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.93902","url":null,"abstract":"<p><strong>Objective: </strong>TCR-engineered NK cells represent a promising strategy for targeting intracellular tumor antigens while overcoming the risk of TCR alpha/beta chain mispairing inherent to TCR-engineered T cells. Previous work showed that CD3ζ overexpression in TCR-NK cells did not improve antigen-specific cytotoxicity and instead increased non-specific background reactivity. This study aims to investigate the molecular alterations associated with this phenotype by examining the transcriptomic profile of engineered NK-92 cells.</p><p><strong>Materials and methods: </strong>Wild-type and genetically modified NK-92 cell lines expressing a tyrosinase-specific TCR in the presence or absence of CD3ζ overexpression were analyzed. Bulk RNA sequencing was followed by differential gene-expression analysis, hierarchical clustering, multidimensional scaling, and pathway enrichment analysis. CD28 surface expression was assessed by flow cytometry, and activation-associated signaling responses were evaluated by western blotting after CD3/CD28 stimulation.</p><p><strong>Results: </strong>CD3ζ-overexpressing NK-92 cells formed distinct transcriptomic clusters and displayed broader gene expression changes. Expression of CD3δγε alone or in combination with tyrosinase-specific TCR resulted in comparatively limited transcriptional divergence from the empty-vector control, whereas CD3ζ inclusion shifted enriched pathways toward lymphocyte activation, cytotoxicity, and PI3K-AKT-related signaling. CD3ζ- overexpressing cells also exhibited increased CD28 surface expression and altered phosphorylation patterns, most notably involving AKT in response to CD3/CD28 stimulation.</p><p><strong>Conclusion: </strong>CD3ζ overexpression is not a neutral modification in TCR-NK cell engineering. It is associated with broad transcriptional remodeling and activation-associated signaling changes that may provide a molecular context for the previously observed increase in non-specific activity. These findings support TCR-NK engineering strategies that preserve endogenous CD3ζ regulation while introducing only the missing CD3δγε components required for functional TCR expression.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148908656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Revisiting Asciminib Resistance: From Binding Affinity to Allosteric Disruption in <i>BCR::ABL1</i> Leukemias.","authors":"Müge Sayitoğlu, Yücel Erbilgin, İbrahim C Haznedaroğlu","doi":"10.4274/tjh.galenos.2026.86383","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.86383","url":null,"abstract":"<p><p>Tyrosine kinase inhibitors (TKIs) have reshaped the treatment of BCR::ABL1 positive leukemias. The first-in-class STAMP (Specifically Targeting the ABL Myristoyl Pocket) inhibitor, asciminib introduces a distinct therapeutic paradigm by targeting the myristoyl pocket and avoids direct competition with ATP. However, resistance to asciminib has been reported in 4-10% of chronic myeloid leukemia (CML) cases. Asciminib resistance is predominantly driven by impaired allosteric regulations: 1) Expanded spectrum of kinase domain mutations \"pre-activate\" the kinase and confer resistance despite retained drug affinity; 2) Intrinsic primary resistance, from b2(e13)a3 and b3(e14)a3 transcript variants; and 3) Isoform-specific (p190 vs. p210) resistance, with common Ploop mutations exhibit greater resistance in p190 than in p210. This paradigm shift from an \"affinity\" problem to an \"allostery\" problem necessitates a corresponding evolution in clinical and laboratory practice. This includes expanding mutational screening, incorporating pre-treatment screening of the BCR::ABL1 isoforms, and transcript types to appropriately guide therapeutic selection.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148908640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"When Thrombosis and Hemorrhage Coexist: A Case of Pediatric Septic Pulmonary Embolism.","authors":"Aslı İmran Yılmaz, Murat Sel, Müşerref Kasap Cüceoğlu, Ayşe Şimşek, Çelebi Kocaoğlu","doi":"10.4274/tjh.galenos.2026.68812","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.68812","url":null,"abstract":"","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148908626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Thrombopoietin Receptor Agonist Exposure in Pregnancy: Not Always Without Fetal or Obstetric Complications","authors":"Ufuk Demirci, Mine Miskioğlu","doi":"10.4274/tjh.galenos.2026.77178","DOIUrl":"10.4274/tjh.galenos.2026.77178","url":null,"abstract":"","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":"288-290"},"PeriodicalIF":1.3,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13540412/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147795805","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}