Turkish Journal of Hematology最新文献

{"title":"The Advancing Landscape of Paroxysmal Nocturnal Hemoglobinuria Treatment","authors":"Cameron Perry, Xinyu Von Buttlar, Swapna Thota","doi":"10.4274/tjh.galenos.2025.2025.0054","DOIUrl":"10.4274/tjh.galenos.2025.2025.0054","url":null,"abstract":"<p><p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare chronic bone marrow failure condition characterized by complement-mediated hemolytic anemia and thrombosis. While its initial clinical description occurred in 1882, somatic mutations in <i>PIGA</i> were discovered in the 1990s. With an improved understanding of PNH biology, a focused effort on complement inhibitors led to the discovery of eculizumab, a C5 inhibitor initially approved by the US Food and Drug Administration in 2007. Terminal complement pathway inhibition reduced intravascular hemolysis, anemia, and thrombosis. Further advancements in drug development for PNH have included improved pharmacokinetics with ravulizumab in 2018 and the introduction of proximal complement inhibitors such as pegcetacoplan (2021), iptacopan (2023), danicopan (2024), and crovalimab (2024) to enhance patient outcomes. With these new proximal and distal complement inhibitors in the treatment landscape, it is timely for clinicians to review the evolving landscape of PNH treatments and patient selection.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"74-81"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099479/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144048645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy, Safety, and Tolerability of Ferric Carboxymaltose and Iron Sucrose in Iron-Deficiency Anemia: A Systematic Review and Meta-Analysis of Randomized Controlled Trials","authors":"Lokman Hekim Tanrıverdi, Ahmet Sarıcı","doi":"10.4274/tjh.galenos.2025.2025.0034","DOIUrl":"10.4274/tjh.galenos.2025.2025.0034","url":null,"abstract":"<p><strong>Objective: </strong>This study comprehensively compares the efficacy, safety, and tolerability of two commonly used intravenous iron preparations, ferric carboxymaltose (FCM) and iron sucrose (IS), in adult patients with iron-deficiency anemia (IDA).</p><p><strong>Materials and methods: </strong>A systematic literature search was conducted across the PubMed, Ovid MEDLINE, Web of Science, and Cochrane Library databases up to January 1, 2024, to identify randomized controlled trials directly comparing FCM and IS treatments in adult patients with IDA. The primary outcome of interest was change in hemoglobin (Hb) levels during follow-up. Meta-analyses were conducted with inverse variance random effects models.</p><p><strong>Results: </strong>Fourteen trials were included in the study, with a total of 4757 patients. FCM resulted in a non-significant increase in Hb levels (mean difference [MD]: 0.45 g/dL, 95% confidence interval [CI]: 0.08 to 0.83, p=0.02) and ferritin levels (MD: 37.32 ng/mL, 95% CI: 18.98 to 55.65, p<0.01) compared to IS. FCM was associated with a higher risk of hypersensitivity reactions compared to IS (relative risk [RR]: 2.97, 95% CI: 1.35 to 6.52, p<0.01) but showed no significant difference in severe adverse events (RR: 1.03, 95% CI: 0.88 to 1.21, p=0.70) and had a non-significant increased risk of hypophosphatemia (RR: 2.84, 95% CI: 0.89 to 9.06, p=0.08).</p><p><strong>Conclusion: </strong>Ten studies showed some concerns of risk of bias (RoB) and four studies had a high RoB for the change in Hb levels during follow-up. The lack of standardized definitions for hypersensitivity reactions and variability in dosing protocols and follow-up durations across studies may affect the generalizability of our safety findings.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"119-135"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099480/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143650809","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Rare Case of Non-Hodgkin Lymphoma Presenting as a Penile Mass","authors":"Ahmet Halil Sevinç, İlker Teke, Özge Hürdoğan, Ali Altay, Zeynep Cantürk, Murat Dursun, Ateş Kadıoğlu","doi":"10.4274/tjh.galenos.2025.2024.0374","DOIUrl":"10.4274/tjh.galenos.2025.2024.0374","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"153-155"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099473/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143587201","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute Myeloid Leukemia with <i>NUP98::LNP1</i> Fusion Mimicking Chronic Myeloid Leukemia","authors":"Haiyang Wang, Yu Peng, Zailin Yang","doi":"10.4274/tjh.galenos.2025.2024.0391","DOIUrl":"10.4274/tjh.galenos.2025.2024.0391","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"142-143"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099463/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143650695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"hsa_circRNA_092488 Exacerbates the Progression of Deep Vein Thrombosis Through the NLRP3/NF-κB Signaling Pathway","authors":"Jian Wang, Binghui Du","doi":"10.4274/tjh.galenos.2025.2024.0160","DOIUrl":"10.4274/tjh.galenos.2025.2024.0160","url":null,"abstract":"<p><strong>Objective: </strong>Deep vein thrombosis (DVT) is a vascular disorder with an incidence rate of about 0.1%. Endothelial progenitor cells (EPCs) are precursor cells of endothelial cells and contribute to vascular repair and regeneration. Circular RNA (circRNA) has become a new focus of research as circRNAs are involved in various biological processes including the progression of DVT. This study explored the upregulation of hsa_circRNA_092488 in DVT patients.</p><p><strong>Materials and methods: </strong>The expression of hsa_circRNA_092488 was evaluated in venous blood samples obtained from DVT patients (n=42) and healthy controls (n=42). Gain- and loss-of-function studies of hsa_circRNA_092488 were carried out. The expression levels of related RNAs and proteins were examined by quantitative real-time reverse-transcription polymerase chain reaction, western blotting and immunofluorescence assays. The proliferation, migration, cell cycle progression, and apoptosis of transfected cells were measured by CCK-8 assay, transwell assay, and flow cytometry. The association of hsa_circRNA_092488 and NOD-like receptor protein 3 (NLRP3) in EPCs was revealed using RNA pull-down analysis. Furthermore, the stability of NLRP3 mRNA was examined in transfected EPCs.</p><p><strong>Results: </strong>Upregulation of hsa_circRNA_092488 was detected in blood samples from DVT patients and it had the ability to suppress the proliferation and migration of EPCs, induce cell cycle arrest from the S to the G0/G1 phase, and trigger cellular apoptosis. Furthermore, NLRP3 was identified as the potential downstream target molecule of hsa_circRNA_092488 and it could exert its regulatory functions by activating the NLRP3/nuclear factor (NF)-κB signaling pathway. Overexpression of hsa_circRNA_092488 in cells notably elevated the protein expression of caspase-1, interleukin-1β, P-NF-κB-p65/NF-κB-p65, and P-IκBα/IκBα, while knockdown of hsa_circRNA_092488 significantly reduced the levels of those proteins in EPCs.</p><p><strong>Conclusion: </strong>hsa_circRNA_092488/NLRP3/NF-κB signaling could be a novel therapeutic candidate for the treatment of DVT.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"108-118"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099489/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143567683","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Large and Multi-Nuclei Blasts in Acute Myeloid Leukemia with the Hypotriploid Karyotype and <i>TP53</i> Mutation with P210 <i>BCR::ABL1</i> Transcript","authors":"Yihong Huangchuan, Xueyan Chen","doi":"10.4274/tjh.galenos.2025.2025.0428","DOIUrl":"10.4274/tjh.galenos.2025.2025.0428","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"146-147"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099481/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143984207","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploration of Leucine-Rich Alpha-2 Glycoprotein 1 (LRG1) and Its Association with Proangiogenic Mediators in Sickle Cell Disease: A Potential Player in the Pathogenesis of the Disease","authors":"Oğuzhan Özcan, Murat Kaçmaz, Fatma Hazal Erdoğan, Lütfiye Seçil Deniz Balyen, Hamdi Oğuzman, Hasan Kaya, Abdullah Arpacı","doi":"10.4274/tjh.galenos.2025.2025.0441","DOIUrl":"10.4274/tjh.galenos.2025.2025.0441","url":null,"abstract":"<p><strong>Objective: </strong>Leucine-rich alpha-2-glycoprotein 1 (LRG1) is a novel mediator involved in abnormal angiogenesis. We aimed to investigate circulating LRG1 levels and their relationship with proangiogenic mediators in sickle cell disease (SCD).</p><p><strong>Materials and methods: </strong>A total of 50 patients with SCD, with 25 in steady-state condition (SCD-SS) and 25 in periods of painful vaso-occlusive crisis (SCD-VOC), and 25 healthy controls were included in the study. Demographical and clinical data were collected from hospital records. Serum LRG1, vascular endothelial growth factor A (VEGFA), and hypoxia-inducible factor 1-alpha (HIF1A) levels were measured by enzyme-linked immunosorbent assay (ELISA), and C-reactive protein (CRP) was measured by the nephelometric method. Routine biochemical parameters were assessed using an autoanalyzer. Multinomial logistic regression was used to analyze ELISA parameters, and receiver operating characteristic (ROC) curves were constructed to determine the optimal cut-off point for HIF1A to predict VOCs in SCD patients.</p><p><strong>Results: </strong>LRG1 and VEGFA levels were significantly higher in SCD patients than controls (p<0.001), with no difference between the SCD-SS and SCD-VOC groups. HIF1A, CRP, and lactate dehydrogenase levels differed significantly across all groups, being highest in the SCD-VOC group (p<0.001). After adjusting for age and sex, LRG1, HIF1A, and VEGFA remained elevated in the SCD groups. HIF1A correlated with CRP (r=0.351, p=0.024), but LRG1 showed no correlation with proangiogenic mediators in the SCD-VOC group. The area under the ROC curve was calculated as 0.694 (95% confidence interval: 0.542-0.845, p=0.021) and the optimal cut-off point was 494.5 pg/mL for HIF1A in predicting vaso-occlusive crises in patients with SCD.</p><p><strong>Conclusion: </strong>Circulating LRG1 levels may reflect neutrophil activation and contribute to the cross-talk between proangiogenic mediators released in SCD.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"100-107"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099478/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144048664","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Basophils in Acute Promyelocytic Leukemia: Clonality or Reactiveness?","authors":"Xue Li, Qingqing Yang, Pengfei Qin, Baodan Yu","doi":"10.4274/tjh.galenos.2025.2024.0433","DOIUrl":"10.4274/tjh.galenos.2025.2024.0433","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"148-149"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099476/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143650923","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hair Re-Pigmentation After Nilotinib","authors":"Öznur Aydın, Mehmet Turgut","doi":"10.4274/tjh.galenos.2025.2025.0102","DOIUrl":"10.4274/tjh.galenos.2025.2025.0102","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"163-164"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099471/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144048354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retrospective Evaluation of Clinical and Follow-Up Outcomes in Primary Cutaneous CD30⁺ Lymphoproliferative Disorders","authors":"Hatice Şanlı, Ahmet Taha Aydemir, İncilay Kalay Yıldızhan, Aylin Heper, Işınsu Kuzu, Ayça Kırmızı, Ayşenur Botsalı, Bengü Nisa Akay","doi":"10.4274/tjh.galenos.2025.2025.0045","DOIUrl":"10.4274/tjh.galenos.2025.2025.0045","url":null,"abstract":"<p><p>This study evaluated the demographic data, clinical characteristics, treatment approaches, and treatment responses of 43 patients with primary cutaneous CD30⁺ lymphoproliferative disorders. Lymphomatoid papulosis (LyP) was characterized by predominantly papular (94.1%) and generalized (70.6%) lesions, while primary cutaneous anaplastic large-cell lymphoma (pcALCL) presented with tumoral (77.8%) and solitary (77.8%) lesions (p<0.001). Common treatments for LyP included methotrexate (response rate: 78.5%), topical corticosteroids (response rate: 83.3%), and phototherapy (response rate: 85.8%), but relapse rates were high. In pcALCL, complete remission was achieved with all treatments, with no relapses after brentuximab vedotin (BV). Secondary malignancies were noted in 20.6% of LyP cases. Both LyP and pcALCL had a 100% 5-year disease-specific survival rate, although two LyP patients (5.9%) died of secondary malignancies. In conclusion, LyP and pcALCL are both indolent lymphomas, with LyP being more prone to relapse. BV is effective for resistant pcALCL. LyP patients need long-term monitoring due to the risk of secondary malignancies.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"136-141"},"PeriodicalIF":1.5,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143567686","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}