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

{"title":"Paroxysmal Nocturnal Hemoglobinuria with a Common Variable Immunodeficiency Phenotype: Noncirrhotic Portal Hypertension Without Demonstrable Splanchnic Thrombosis.","authors":"Ragıp Fatih Kural, Gürsel Kavlak, Nigar Abdullayeva, Reyhan Gümüşburun, Ömür Ardeniz, Nur Soyer","doi":"10.4274/tjh.galenos.2026.10336","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.10336","url":null,"abstract":"","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149225791","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":"Invisible Enemy: CMV Retinitis Despite Persistent Blood PCR Negativity in Pediatric AML.","authors":"Meral Bülbül, Seval Özen, Selin Yıldız Sametoğlu, Gökçen Tuğçe Özsüt, Ayça Koca Yozgat, Dilek Gürlek Gökçebay, Pınar Nalçacıoğlu, Saliha Kanık Yüksek","doi":"10.4274/tjh.galenos.2026.61818","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.61818","url":null,"abstract":"","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149225802","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":"Autologous Hematopoietic Stem Cell Transplantation Without Cryopreservation: A Practical Option in Resource-Limited Settings.","authors":"Zakaria El Kodmiri, Mehdi El Agal, Zineb Guessous, Lina El Mekkoudi, Maryame Ahnach","doi":"10.4274/tjh.galenos.2026.27880","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.27880","url":null,"abstract":"","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149130237","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":"Acid Sphingomyelinase Deficiency in an Adult with Unexplained Splenomegaly Despite Negative <i>SMPD1</i> Sequencing: A Case Report.","authors":"Tahir Alper Cinli, Zehra Akman İlik","doi":"10.4274/tjh.galenos.2026.20599","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.20599","url":null,"abstract":"","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149042862","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 Outcomes of Pediatric Idiopathic Hypereosinophilic Syndrome: A 22-Year Single-Center Case Series.","authors":"Sultan Okur Acar, Çağrı Berhan Kurdu, Tuba Yurdusev, Özlem Tüfekçi Gürocak, Şebnem Yılmaz, Hale Ören","doi":"10.4274/tjh.galenos.2026.33604","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2026.33604","url":null,"abstract":"<p><strong>Objective: </strong>Idiopathic hypereosinophilic syndrome (HES) is a rare and heterogeneous disorder in childhood, and data regarding its clinical course and treatment outcomes are limited.</p><p><strong>Materials and methods: </strong>We aimed to evaluate the clinical characteristics, organ involvement patterns, treatment approaches, and follow-up outcomes of pediatric patients diagnosed with idiopathic HES. This retrospective study included pediatric patients diagnosed with idiopathic HES between 2003 and 2025 at a tertiary pediatric hematology center. Diagnosis was based on persistent peripheral eosinophilia (≥1,500 /mm³), evidence of organ involvement attributable to eosinophilia, and exclusion of secondary and clonal causes. Demographic features, laboratory findings, organ involvement, treatment responses, relapse frequency, and outcomes were analyzed descriptively.</p><p><strong>Results: </strong>Seven patients (2 females, 5 males) were included. The median age at diagnosis was 5 years (range, 3 months-9 years). The median absolute eosinophil count at diagnosis was 16,000 /mm³ (range: 5,600-112,000). Organ involvement was heterogeneous, with hepatic involvement being the most frequent (6/7, 85.7%), followed by cardiac (3/7, 42.9%), cutaneous (2/7, 28.6%), and pulmonary and central nervous system involvement (1/7 each, 14.3%). All patients received corticosteroids as first-line therapy, with generally rapid initial hematologic and clinical responses. However, recurrent relapses occurred in several patients. One patient with frequent relapses and progressive pulmonary involvement died despite multiple additional therapies.</p><p><strong>Conclusion: </strong>Pediatric idiopathic HES demonstrates variable clinical behavior. Although corticosteroids are generally effective as initial therapy, relapse risk and potential for severe organ involvement necessitate close monitoring and individualized management strategies.</p>","PeriodicalId":520821,"journal":{"name":"Turkish journal of haematology : official journal of Turkish Society of Haematology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149043031","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":"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}