International Journal of Hematology最新文献

筛选
英文 中文
Recent progress in clonal hematopoiesis: expanding the concept. 克隆造血的最新进展:概念的拓展。
IF 1.8 4区 医学
International Journal of Hematology Pub Date : 2025-07-31 DOI: 10.1007/s12185-025-04034-9
Shigeru Chiba
{"title":"Recent progress in clonal hematopoiesis: expanding the concept.","authors":"Shigeru Chiba","doi":"10.1007/s12185-025-04034-9","DOIUrl":"https://doi.org/10.1007/s12185-025-04034-9","url":null,"abstract":"<p><p>Clonal hematopoiesis (CH) has emerged as a common age-related phenomenon and a central concept linking somatic mutations in hematopoietic stem cells to both malignant and non-malignant diseases. While initially recognized in the context of hematologic neoplasms, CH is now known to contribute to increased all-cause mortality, particularly through heightened risk of cardiovascular and inflammatory diseases. Frequent mutations in genes such as DNMT3A, TET2, and ASXL1 alter epigenetic regulation and immune signaling, thereby promoting clonal expansion and systemic consequences. Longitudinal studies have illuminated the dynamics of clonal growth and revealed how germline variants influence somatic selection. VEXAS syndrome, driven by UBA1-mutated CH, exemplifies the broader clinical reach of clonal expansion beyond malignancy. CH occupies an intermediate biological state with far-reaching implications. In this Progress in Hematology series, contributors explore the natural history, genetic underpinnings, and inflammatory manifestations of CH, offering insights into its role as both a biomarker and a potential therapeutic target in aging populations.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144760049","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}
引用次数: 0
Treatment of post-transplant lymphoproliferative disorders in kidney transplant recipients: a single-center retrospective analysis. 肾移植受者移植后淋巴增生性疾病的治疗:单中心回顾性分析。
IF 1.8 4区 医学
International Journal of Hematology Pub Date : 2025-07-28 DOI: 10.1007/s12185-025-04039-4
Futoshi Yoshino, Daisuke Kaji, Otoya Watanabe, Kyosuke Yamaguchi, Kosei Kageyama, Yuki Taya, Aya Nishida, Kazuya Ishiwata, Shinsuke Takagi, Hisashi Yamamoto, Yuki Asano-Mori, Naoyuki Uchida, Atsushi Wake, Go Yamamoto
{"title":"Treatment of post-transplant lymphoproliferative disorders in kidney transplant recipients: a single-center retrospective analysis.","authors":"Futoshi Yoshino, Daisuke Kaji, Otoya Watanabe, Kyosuke Yamaguchi, Kosei Kageyama, Yuki Taya, Aya Nishida, Kazuya Ishiwata, Shinsuke Takagi, Hisashi Yamamoto, Yuki Asano-Mori, Naoyuki Uchida, Atsushi Wake, Go Yamamoto","doi":"10.1007/s12185-025-04039-4","DOIUrl":"https://doi.org/10.1007/s12185-025-04039-4","url":null,"abstract":"<p><p>Post-transplant lymphoproliferative disorder (PTLD) is a significant complication of immunosuppression after kidney transplantation and has no established standard therapy. Achieving favorable treatment outcomes and preserving renal function in patients with PTLD remains challenging, particularly when the central nervous system (CNS) is involved. Here we describe our experience with 8 patients who developed PTLD after kidney transplantation at our institution. Diffuse large B-cell lymphoma was the most common histological subtype, observed in 5 patients (62.5%). Epstein-Barr Virus infections were observed in 7 patients (87.5%), CNS involvement in 5 patients (62.5%) and gastrointestinal tract involvement in 3 patients (37.5%). With a median follow-up of 55.3 months, the 4-year overall survival rate was 72.9%. Seven patients (87.5%) achieved complete remission (CR), and 5 maintained CR as of the last follow-up visit. In the CNS involvement group, patients treated with aggressive chemotherapy died of sepsis, and those treated with a combination of rituximab, whole-brain radiation therapy, and reduction of immunosuppression achieved long-term progression-free survival without reinstitution of dialysis or neurological toxicity. The clinical courses of these 8 kidney transplant recipients who developed PTLD, most of whom had CNS involvement, suggest that long-term remission may be achievable without systemic chemotherapy.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144730309","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}
引用次数: 0
Humoral immunity after hematopoietic stem cell transplantation: evaluation by B-cell receptor repertoire analysis. 造血干细胞移植后体液免疫:用b细胞受体库分析评价。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-25 DOI: 10.1007/s12185-025-04042-9
Sakuya Matsumoto, Yohei Funakoshi, Kimikazu Yakushijin, Takaji Matsutani, Yuri Okazoe-Hirakawa, Goh Ohji, Taiji Koyama, Yoshiaki Nagatani, Keiji Kurata, Shiro Kimbara, Naomi Kiyota, Hironobu Minami
{"title":"Humoral immunity after hematopoietic stem cell transplantation: evaluation by B-cell receptor repertoire analysis.","authors":"Sakuya Matsumoto, Yohei Funakoshi, Kimikazu Yakushijin, Takaji Matsutani, Yuri Okazoe-Hirakawa, Goh Ohji, Taiji Koyama, Yoshiaki Nagatani, Keiji Kurata, Shiro Kimbara, Naomi Kiyota, Hironobu Minami","doi":"10.1007/s12185-025-04042-9","DOIUrl":"https://doi.org/10.1007/s12185-025-04042-9","url":null,"abstract":"<p><p>Immunity acquired before hematopoietic stem cell transplantation (HSCT) may decrease or disappear following HSCT, and it is unclear whether the first vaccination after HSCT in patients with an antigen exposure history before HSCT elicits a primary or secondary immune response. The Quantification of Antigen-Specific Antibody Sequence (QASAS) method enables real-time assessment of responses to SARS-CoV-2 antigen exposure through B-cell receptor (BCR) repertoire analysis. Using this method, we evaluated the disappearance of immunological memory after HSCT. First, in individuals without hematologic disorders, primary SARS-CoV-2 antigen exposure elicited no immune response at 7 days post-exposure but demonstrated activation between 14 to 21 days. In contrast, repeated exposure elicited early responses (secondary immune responses) at 7 days post-exposure. We then enrolled HSCT patients with pre-HSCT SARS-CoV-2 antigen exposure history and collected samples before and after vaccination. Despite prior exposure history, patients receiving their first vaccination after HSCT showed no response around 7 days post-exposure but responded at 14 days. In conclusion, even with pre-HSCT antigen exposure, the first vaccination after HSCT induced a primary immune response. This demonstrates that first vaccination after HSCT should be considered to induce a primary immune response, regardless of previous infection or vaccination history.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144707432","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}
引用次数: 0
Expression profiling of Epstein-Barr virus-derived microRNA in systemic chronic active EBV disease. eb病毒衍生的microRNA在系统性慢性活动性EBV疾病中的表达谱
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-25 DOI: 10.1007/s12185-025-04040-x
Mayumi Yoshimori, Miwako Nishio, Ayaka Ohashi, Yuri Maekawa, Runa Shimomaki, Morito Kurata, Kotaro Yoshioka, Takanori Yokota, Ryusuke Nabeshima, Ayako Arai
{"title":"Expression profiling of Epstein-Barr virus-derived microRNA in systemic chronic active EBV disease.","authors":"Mayumi Yoshimori, Miwako Nishio, Ayaka Ohashi, Yuri Maekawa, Runa Shimomaki, Morito Kurata, Kotaro Yoshioka, Takanori Yokota, Ryusuke Nabeshima, Ayako Arai","doi":"10.1007/s12185-025-04040-x","DOIUrl":"https://doi.org/10.1007/s12185-025-04040-x","url":null,"abstract":"<p><p>Systemic chronic active Epstein-Barr virus disease (sCAEBV) is an intractable disorder characterized by clonal proliferation of EBV-infected T- and NK-cells, leading to persistent systemic inflammation and progression to hemophagocytic lymphohistiocytosis (HLH). The EBV genome encodes 40 mature microRNAs known as miR-BARTs. The expression of miR-BARTs has been reported in other EBV-positive diseases and is associated with tumorigenesis. In this study, we investigated the expression of miR-BARTs in sCAEBV. Expression of miR-BARTs was highly abundant in 4 sCAEBV-derived EBV-positive T- or NK-cell lines and in EBV-infected T- or NK-cells from 23 sCAEBV patients. The highest expression levels were observed for miR-BART7-3p. Sequence analysis revealed no deletions in the EBV genome encoding miR-BARTs. Inhibition of miR-BART7-3p altered the expression of immune-related genes in sCAEBV-derived cell lines. Abundant miR-BART expression was also observed in patients' plasma, with miR-BART7-3p showing the highest levels. Notably, miR-BART7-3p expression was detected in macrophages within the spleen of an sCAEBV patient with HLH. These findings suggest that miR-BARTs are highly expressed and secreted by EBV-infected cells in sCAEBV. We hypothesize that secreted miR-BARTs may be taken up by monocytes, potentially regulating their functions and contributing to inflammation in sCAEBV. Further studies are needed to elucidate these mechanisms.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144707431","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}
引用次数: 0
Cardiovascular toxicity from tyrosine kinase inhibitors in chronic myeloid leukemia with severe dilated cardiomyopathy. 酪氨酸激酶抑制剂对慢性髓系白血病合并严重扩张型心肌病的心血管毒性。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-22 DOI: 10.1007/s12185-025-04041-w
Kenta Hayashino, Naoya Mochizuki, Akihiro Kawatsuki, Yuki Fujiwara, Hiroki Kobayashi, Tomoko Yamasaki, Takeru Asano, Shiro Kubonishi, Yasushi Hiramatsu
{"title":"Cardiovascular toxicity from tyrosine kinase inhibitors in chronic myeloid leukemia with severe dilated cardiomyopathy.","authors":"Kenta Hayashino, Naoya Mochizuki, Akihiro Kawatsuki, Yuki Fujiwara, Hiroki Kobayashi, Tomoko Yamasaki, Takeru Asano, Shiro Kubonishi, Yasushi Hiramatsu","doi":"10.1007/s12185-025-04041-w","DOIUrl":"https://doi.org/10.1007/s12185-025-04041-w","url":null,"abstract":"<p><p>Tyrosine kinase inhibitors (TKIs) have significantly improved the prognosis of patients with chronic myeloid leukemia (CML). However, few patients maintain remission after discontinuing TKIs, and most require long-term treatment. Prolonged use of TKIs is associated with an increased risk of cardiovascular events (CVEs), particularly in patients with pre-existing cardiovascular comorbidities. We present the case of a 37-year-old man with a decade-long history of severe dilated cardiomyopathy that responded inadequately to standard pharmacologic management, who was under consideration for heart transplantation. Following a diagnosis of CML, the patient experienced various CVEs with TKIs, including dasatinib, bosutinib, imatinib, and nilotinib. These agents were discontinued, and treatment was switched to asciminib, a novel agent that targets the myristoyl pocket of the BCR::ABL1 protein distinct from the ATP-binding site targeted by conventional TKIs. This treatment was well tolerated without any CVEs. Given its minimal off-target activity and lower reported incidence of CVEs, asciminib may offer a viable and safer therapeutic option for CML patients with advanced cardiovascular comorbidities, including those awaiting heart transplantation.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144690134","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}
引用次数: 0
Efficacy and safety of momelotinib in Janus kinase inhibitor-experienced Asian patients with myelofibrosis and anemia. 莫美洛替尼治疗有Janus激酶抑制剂的亚洲骨髓纤维化和贫血患者的疗效和安全性。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-21 DOI: 10.1007/s12185-025-04037-6
Sung-Soo Yoon, Chih Cheng Chen, Sung-Eun Lee, Hung Chang, June-Won Cheong, Hsin-An Hou, Won Sik Lee, Sung-Nam Lim, Joon Ho Moon, Kiat Hoe Ong, Yi Dai, Chang Liu, Jun Kawashima, Yeow Tee Goh
{"title":"Efficacy and safety of momelotinib in Janus kinase inhibitor-experienced Asian patients with myelofibrosis and anemia.","authors":"Sung-Soo Yoon, Chih Cheng Chen, Sung-Eun Lee, Hung Chang, June-Won Cheong, Hsin-An Hou, Won Sik Lee, Sung-Nam Lim, Joon Ho Moon, Kiat Hoe Ong, Yi Dai, Chang Liu, Jun Kawashima, Yeow Tee Goh","doi":"10.1007/s12185-025-04037-6","DOIUrl":"https://doi.org/10.1007/s12185-025-04037-6","url":null,"abstract":"<p><strong>Introduction: </strong>This post hoc analysis investigated the efficacy and safety of momelotinib in the Asian subpopulation of MOMENTUM (NCT04173494).</p><p><strong>Methods: </strong>Patients were randomized 2:1 to momelotinib 200 mg once daily (QD) plus danazol placebo (momelotinib group) or danazol 600 mg QD plus momelotinib placebo (danazol group) for 24 weeks (W), after which they could receive open-label momelotinib or danazol.</p><p><strong>Primary endpoint: </strong>W24 total symptom score (TSS) response rate (≥ 50% reduction from baseline). W24 key secondary endpoints: transfusion independence rate; mean TSS change from baseline; splenic response rate; rate of zero transfusions.</p><p><strong>Results: </strong>Seventeen Asian patients with myelofibrosis were included (momelotinib: n = 11; danazol: n = 6). TSS response rate at W24 was 36.4% with momelotinib and 0% with danazol. Secondary endpoints favored momelotinib and were consistent with the intention-to-treat population. Grade ≥ 3 treatment-emergent adverse events were reported in 36.4 and 66.7% of the momelotinib and danazol groups, respectively, including one grade ≥ 3 anemia in the momelotinib group. Treatment interruption and/or dose reduction occurred in 18.2 and 16.7% of the momelotinib and danazol groups, respectively. Two danazol-treated patients discontinued study treatment.</p><p><strong>Conclusion: </strong>In the Asian subpopulation of MOMENTUM, momelotinib improved myelofibrosis-associated symptoms, anemia measures, and spleen response, with generally favorable safety versus danazol.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682609","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}
引用次数: 0
Intravenous umbilical cord-derived mesenchymal stromal cell therapy may improve overall survival in Japanese patients with idiopathic pneumonia syndrome after hematopoietic stem cell transplantation: a multicenter, single-arm, phase II trial. 静脉输注脐带间充质间质细胞治疗可提高日本特发性肺炎综合征患者造血干细胞移植后的总生存率:一项多中心、单臂、II期试验。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-15 DOI: 10.1007/s12185-025-04024-x
Noriko Doki, Nobuharu Fujii, Shinichi Kako, Emiko Sakaida, Yoshinobu Kanda
{"title":"Intravenous umbilical cord-derived mesenchymal stromal cell therapy may improve overall survival in Japanese patients with idiopathic pneumonia syndrome after hematopoietic stem cell transplantation: a multicenter, single-arm, phase II trial.","authors":"Noriko Doki, Nobuharu Fujii, Shinichi Kako, Emiko Sakaida, Yoshinobu Kanda","doi":"10.1007/s12185-025-04024-x","DOIUrl":"https://doi.org/10.1007/s12185-025-04024-x","url":null,"abstract":"<p><p>Idiopathic pneumonia syndrome (IPS) is a serious complication of allogeneic hematopoietic stem cell transplantation (HSCT) and has a poor prognosis. Although IPS is often treated with steroids, the disease can become resistant to or dependent on steroid treatment, and there is no effective cure for patients with refractory or steroid-dependent IPS. This multicenter, open-label, single-arm, phase II clinical trial investigated the efficacy and safety of HLC-001 (allogeneic umbilical cord-derived mesenchymal stromal cells) in patients with progressive steroid-dependent or refractory IPS after HSCT. Seven male patients (all male; mean age: 43.3 years) received HLC-001 and three completed the trial. The survival rate at day 56 (primary endpoint) was 71.4% (5/7 patients; 95% confidence interval: 29.0%-96.3%) and was sustained at day 100, suggesting that HLC-001 was more effective than previously reported treatment. Three of the five patients with ≥ 100 days of follow-up died. Five patients experienced at least one adverse drug reaction, none of which were serious. These findings indicate that HLC-001 was potentially effective and generally well tolerated in Japanese patients with steroid-dependent or refractory IPS after HSCT. Given there is no effective cure for steroid-dependent or refractory IPS, HLC-001 may be a promising treatment option and further clinical evaluation is warranted.Trial registration: Japan Registry of Clinical Trials identifier: jRCT2063220014.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144642525","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}
引用次数: 0
High-dose thiotepa may induce inappropriate secretion of antidiuretic hormone syndrome (SIADH) during conditioning treatment for allogeneic stem cell transplantation. 大剂量硫替帕可能诱导异基因干细胞移植调理治疗过程中抗利尿激素综合征(SIADH)的不适当分泌。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-14 DOI: 10.1007/s12185-025-04032-x
Maria Assunta Limongiello, Elisabetta Metafuni, Sabrina Giammarco, Eugenio Galli, Federica Sorà, Francesco Autore, Silvia Baroni, Patrizia Chiusolo, Simona Sica
{"title":"High-dose thiotepa may induce inappropriate secretion of antidiuretic hormone syndrome (SIADH) during conditioning treatment for allogeneic stem cell transplantation.","authors":"Maria Assunta Limongiello, Elisabetta Metafuni, Sabrina Giammarco, Eugenio Galli, Federica Sorà, Francesco Autore, Silvia Baroni, Patrizia Chiusolo, Simona Sica","doi":"10.1007/s12185-025-04032-x","DOIUrl":"https://doi.org/10.1007/s12185-025-04032-x","url":null,"abstract":"<p><p>We conducted a retrospective, single-center, observational cohort study in patients with inappropriate secretion of antidiuretic hormone syndrome (SIADH) induced by high-dose thiotepa during conditioning for allogeneic stem cell transplantation (allo-HSCT). Between May 2018 and December 2023, 431 patients underwent allo-HSCT. All patients who developed SIADH had received TBF conditioning, and the incidence of SIADH among TBF-treated patients was 7.3%. Despite its limitations as a retrospective study, this real-life analysis shows that hyponatremia with corresponding serum hypo-osmolality and persistent urinary sodium excretion is compatible with a diagnosis of SIADH in the presence of normovolemia. Fluid balance and electrolyte levels should be closely monitored during conditioning to ensure prompt detection of SIADH promptly and enable immediate treatment.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144636978","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}
引用次数: 0
Discovering the hidden link: hematological disorders caused by copper deficiency. 发现隐藏的环节:缺铜引起的血液病。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-14 DOI: 10.1007/s12185-025-04036-7
Akiyoshi Takami, Kaori Uchino
{"title":"Discovering the hidden link: hematological disorders caused by copper deficiency.","authors":"Akiyoshi Takami, Kaori Uchino","doi":"10.1007/s12185-025-04036-7","DOIUrl":"https://doi.org/10.1007/s12185-025-04036-7","url":null,"abstract":"<p><p>Copper deficiency can lead to anemia, leukopenia, or thrombocytopenia, often mimicking vitamin B<sub>12</sub> deficiency or myelodysplastic syndrome. We review the crucial role of copper in iron metabolism and hematopoiesis, highlighting how high-dose zinc supplementation, gastrointestinal surgeries, and long-term nutritional support can induce deficiency. The pathophysiology involves compromised ferroxidase activity, which hampers iron utilization, and reduced neutrophil survival due to impairment of cytochrome c oxidase and superoxide dismutase. Timely recognition is vital, as most cases respond to copper repletion and zinc reduction. Institutional data underscore the need for routine copper monitoring, and management strategies aim to optimize patient care by maintaining adequate copper levels.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626255","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}
引用次数: 0
Growth differentiation factor-15 as a non-invasive biomarker of liver fibrosis in sickle cell disease. 生长分化因子-15作为镰状细胞病肝纤维化的非侵入性生物标志物
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-07-13 DOI: 10.1007/s12185-025-04035-8
Konstantinos Manganas, Sophia Delicou, Emilia Hadziyannis, Stavroula Giannouli, John Koskinas
{"title":"Growth differentiation factor-15 as a non-invasive biomarker of liver fibrosis in sickle cell disease.","authors":"Konstantinos Manganas, Sophia Delicou, Emilia Hadziyannis, Stavroula Giannouli, John Koskinas","doi":"10.1007/s12185-025-04035-8","DOIUrl":"https://doi.org/10.1007/s12185-025-04035-8","url":null,"abstract":"<p><p>This study aimed to evaluate the relationship between growth differentiation factor-15 (GDF-15) levels and liver fibrosis in patients with sickle cell disease (SCD) and assess the diagnostic performance of GDF-15 as a non-invasive biomarker. Thirty patients with SCD were categorized into two groups based on fibrosis severity (≥ F2 vs. < F2) determined by Fibroscan, AST to Platelet Ratio Index (APRI) and FIB-4. GDF-15 levels were compared between groups, and independent predictors of GDF-15 were identified by multiple linear regression. Patients with significant liver fibrosis (≥ F2) had significantly higher GDF-15 levels. Multivariate linear regression revealed that GDF-15 concentration was independently associated with liver elastography values (β = 0.619, p = 0.002). The area under the curve for detecting significant fibrosis using GDF-15 as a diagnostic test was 0.835 (95% CI: 0.686-0.983, p = 0.002). A GDF-15 cut-off of 4200 pg/ml had a positive predictive value of 73.6%, a negative predictive value of 81.8%, sensitivity of 87.5% and specificity of 64.3%. In conclusion, GDF-15 is strongly associated with liver fibrosis in SCD and demonstrates high diagnostic accuracy as a non-invasive biomarker. Given its role in inflammation, oxidative stress, and iron metabolism, GDF-15 may serve as a valuable tool for early fibrosis detection and disease monitoring.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144617374","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}
引用次数: 0
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信