Mediterranean Journal of Hematology and Infectious Diseases最新文献

{"title":"Late-onset Anicteric Veno-occlusive Disease after Haploidentical HSCT: Diagnostic Challenges in the Setting of CMV Reactivation.","authors":"Thi Tuyet Mai Nguyen, Tuan Tung Nguyen, Van Tuan Ta","doi":"10.4084/MJHID.2026.057","DOIUrl":"10.4084/MJHID.2026.057","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026057"},"PeriodicalIF":2.3,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13372103/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456408","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":"Type B Lactic Acidosis and Acute Kidney Injury Heralding B-Lymphoblastic Leukemia/Lymphoma.","authors":"Zhan Su, Qingyun Fan, Yan Jiang","doi":"10.4084/MJHID.2026.053","DOIUrl":"10.4084/MJHID.2026.053","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026053"},"PeriodicalIF":2.3,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13372106/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456340","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":"Multiple Myeloma Diagnosed by CT-Guided Rib Biopsy after a Hemodilute Bone Marrow Aspirate, in a Patient with a Biclonal Pattern on Serum Protein Electrophoresis.","authors":"Arjeta Cyrbja, Liri Seraj, Shaqir Qerama","doi":"10.4084/MJHID.2026.061","DOIUrl":"10.4084/MJHID.2026.061","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026061"},"PeriodicalIF":2.3,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13372099/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456356","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":"Comprehensive Review of Genetic and Epigenetic Regulation of Fetal Hemoglobin in β-Hemoglobinopathies: From Molecular Mechanisms to Clinical Applications.","authors":"Yousef Saeed Mohammad Abu Za'ror, Joseph Bagi Suleiman, Fatima Azzahra Delmani, Jehad F Alhmoud, Amer Mohammad Ayasreh, Sarah Ihsan Al-Wendawi, Tareq Nayef AlRamadneh, Maryam Azlan","doi":"10.4084/MJHID.2026.046","DOIUrl":"10.4084/MJHID.2026.046","url":null,"abstract":"<p><p>Reactivating fetal hemoglobin (HbF) has become a key therapeutic strategy for β-hemoglobinopathies. However, the regulatory networks controlling HbF are complex and have only recently been uncovered. This review integrates current knowledge of the genetic and epigenetic factors that influence HbF expression, including BCL11A, HBS1L-MYB, KLF1, and variants associated with HPFH, and shows how these pathways work together to regulate γ-globin levels. It also highlights recent advances in HbF-targeted treatments, including gene-editing technologies such as CRISPR-Cas9-based BCL11A enhancer disruption, promoter editing to mimic hereditary persistence of fetal hemoglobin (HPFH), and advanced tools like base and prime editing. By combining mechanistic understanding with therapeutic development, this review highlights how improvements in HbF regulation have transformed efforts to find cures for sickle cell disease and β-thalassemia, while also revealing new opportunities for targeted HbF induction across different patient groups.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026046"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13170808/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147942115","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":"Early Predictive Value of Peripheral Inflammatory Index Combined with High-Sensitivity Troponin T for Sepsis-Induced Cardiomyopathy.","authors":"ChunYan Zong, FeiFeng Wu, Xin Chu, FenFen Chen, JiaoJiao Yang, Hong Zhou, BaoFeng Zhu","doi":"10.4084/MJHID.2026.045","DOIUrl":"10.4084/MJHID.2026.045","url":null,"abstract":"<p><strong>Background: </strong>Sepsis-induced cardiomyopathy (SICM) is a common and serious complication of sepsis, and early identification remains challenging. Inflammatory and immune markers may provide complementary information to myocardial injury biomarkers.</p><p><strong>Methods: </strong>A total of 319 adult patients with a first diagnosis of sepsis between June 2022 and January 2025 were enrolled. Complete blood counts and high-sensitivity cardiac troponin T (hs-cTnT) were collected within 0-6 hours after diagnosis. SICM was determined based on clinical and imaging data. Peripheral blood inflammatory indices (PBIIs), including NLR, SII, SIRI, PLR, and PIV, were calculated from blood counts. Variable robustness was assessed using LASSO logistic regression combined with bootstrap resampling. Univariate and multivariate logistic regression analyses were then performed to construct predictive models, and model performance was evaluated using ROC curves, calibration curves, and decision curve analysis.</p><p><strong>Results: </strong>Among 319 patients with sepsis, 115 (36.1%) developed SICM. Compared with the non-SICM group, patients with SICM had significantly higher hs-cTnT levels, indicating more severe myocardial injury. Peripheral inflammatory indices were also higher overall, with the largest between-group differences observed for SII and NLR. SIRI and PLR were also elevated, whereas PIV showed a smaller difference (all P<0.05). The baseline model including hs-cTnT achieved an AUC of 0.825; adding SII or NLR further improved discrimination, with AUCs of 0.856 and 0.860, respectively. Calibration and decision curve analyses showed consistent model performance.</p><p><strong>Conclusion: </strong>SII and NLR, as readily available peripheral inflammatory markers, were associated with improved early prediction of SICM when combined with hs-cTnT. This combined strategy may help refine early risk stratification in patients with sepsis.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026045"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13170807/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147942099","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":"Aging with Thalassemia and Sickle Cell Disease: A Gerontological Model of Accelerated Multimorbidity and Function-Centered Care Beyond Midlife.","authors":"Sophia Delicou, Constantina Aggeli, Iliana Mani, Christos Savvidis, Myrto Palkopoulou, Theodoros Aforozis, Irene Kouroukli, Panagiota Giannou, Alexandra Mika, Aikaterini Xydaki, Maria Moraki, Elena Papatheodorou, Athanasia Kapota, Ioannis Ilias, John Koskinas","doi":"10.4084/MJHID.2026.039","DOIUrl":"10.4084/MJHID.2026.039","url":null,"abstract":"<p><strong>Background: </strong>Thalassemia and sickle cell disease are now increasingly present as lifelong chronic conditions in high-income countries, with growing numbers of patients reaching their 50s and 60s. This demographic shift transforms hemoglobinopathies from childhood-threatening disorders into chronic, multisystem conditions with cumulative morbidity. However, data specifically focused on later-life hemoglobinopathy populations remain limited, fragmented, and often extrapolated from younger cohorts, leaving hematologists and internists relatively unprepared for the functional decline, vulnerability, and geriatric syndromes that can characterize later life in these populations.</p><p><strong>Content: </strong>This expert opinion narrative review synthesizes available evidence on the intersection of disease-driven pathology (anemia, hemolysis, vasculopathy), long-term treatment burden (transfusion-related iron overload, chelation toxicities), and aging biology (declining physiologic reserve, sarcopenia, cognitive vulnerability) in adults beyond midlife. Given the historical survival patterns in hemoglobinopathies and the inconsistent definition of \"older adult\" across studies, particularly in sickle cell disease, we use a pragmatic age threshold of >=50 years for the main gerontological framing, while incorporating evidence from cohorts beginning at 40-49 years when that is how the literature defines older hemoglobinopathy populations. We distinguish disease-specific priorities: thalassemia faces myocardial and hepatic iron deposition and endocrine failure, while sickle cell disease confronts cerebrovascular disease, chronic pain, and cardiopulmonary complications. Critically, care targets in later life must extend beyond survival and organ-specific metrics to functional endpoints, disability prevention, cognitive health, and quality of life. A conceptual mapping links mechanisms of hemoglobinopathy to established gerontology constructs, including inflammaging, cellular senescence, and vascular aging, while acknowledging that direct mechanistic evidence in older hemoglobinopathy cohorts remains incomplete.</p><p><strong>Conclusions: </strong>Three adjustments are necessary in adults beyond midlife: monitoring should prioritize early detection of treatable complications and emerging functional impairment rather than only documenting cumulative organ damage; therapeutic decisions should weigh treatment benefit, treatment burden, comorbidity burden, and goals of care rather than defaulting to pediatric-era protocols; and care systems should embed shared decision-making, palliative principles, and multidisciplinary coordination within primary care networks, with specialist hemoglobinopathy centers functioning as disease-specific hubs rather than stand-alone primary care providers.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026039"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13170809/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147942126","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 and Safety of Thalidomidein Patients with Transfusion-Dependent B-Thalassemia: A Systematic Review, Meta-Analysis and GRADE Evaluation.","authors":"Jialian Li, Ping Ji, Qinyue Zhong, Lin Dong, Hai Yi, Baiyu Chen, Xu Zhang, Tingting Duan","doi":"10.4084/MJHID.2026.033","DOIUrl":"10.4084/MJHID.2026.033","url":null,"abstract":"<p><strong>Background: </strong>The immunomodulatory drug thalidomide is a promising alternative therapy for transfusion-dependent β-thalassemia (TDT) due to its potent induction of fetal hemoglobin.</p><p><strong>Objective: </strong>To identify the efficacy and safety of thalidomide in TDT patients.</p><p><strong>Methods: </strong>A comprehensive search was conducted across MEDLINE, EMBASE, CENTRAL, Web of Science, CBM, CNKI, VIP, Wangfang Data, and OpenGrey, up to January 24, 2026. We included RCTs and observational studies involving more than 10 TDT patients treated with thalidomide for at least 3 months. Risk of bias was assessed using RoB 2 and MINORS tools. The certainty of evidence was evaluated with the GRADE approach.</p><p><strong>Results: </strong>Twenty-six studies involving 2422 patients were included. No studies had a high risk of bias. The meta-analysis of RCTs demonstrated that, compared with the control group, thalidomide significantly increased the major response rate (MRR; low certainty), the overall response rate (ORR; low certainty), and hemoglobin level (Hb; moderate certainty), as well as fetal hemoglobin level (HbF; moderate certainty). There was no significant between-group difference in changes in serum ferritin (SF) levels (very low certainty). The pre-post meta-analysis showed the following pooled outcomes: MRR 62.91% (very low certainty), ORR 75.07% (very low certainty), a Hb level change of 1.94 g/dL, a HbF level change of 39.21%, and an SF level change of -1432.39 ng/mL. The pooled incidence of adverse drug events (ADEs) was 4.65 per 100 person months, and that of ADEs leading to drug discontinuation was 0.04 per 100 person months. Most reported events were mild and tolerable.</p><p><strong>Conclusion: </strong>Thalidomide may provide limited benefits for TDT patients. In clinical practice, its potential risks must be thoroughly weighed against these benefits. Therefore, future large-scale, globally diverse, and high-quality RCTs are warranted.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026033"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13170816/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147941356","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":"Mapping Three Decades of Research on Southeast Asian Ovalocytosis: A Bibliometric Analysis.","authors":"Muhd Alwi Muhd Helmi, Nor Zamzila Abdullah, Ahmad Marzuki Omar, Nour El Huda Abd Rahim, Norlelawati A Talib","doi":"10.4084/MJHID.2026.036","DOIUrl":"10.4084/MJHID.2026.036","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026036"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13170805/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147941518","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":"Association Between Pre-Admission 48-Hour Fever Burden and Outcomes in Pediatric Mycoplasma Pneumoniae Infection.","authors":"Lijie Ma, Ling Chen","doi":"10.4084/MJHID.2026.034","DOIUrl":"10.4084/MJHID.2026.034","url":null,"abstract":"<p><strong>Background: </strong>Refractory and severe evolution complicate pediatric Mycoplasma pneumoniae pneumonia (MPP), yet early risk stratification still relies largely on single-time admission biomarkers. We tested whether a prespecified 48-hour pre-admission fever-burden index (FBI48) predicts in-hospital outcomes and improves model performance beyond guideline-consistent clinical and laboratory predictors.</p><p><strong>Methods: </strong>We conducted a retrospective single-center cohort study of hospitalized children ≤14 years with laboratory-confirmed MPP. FBI48 was defined as the area under the temperature-time curve above 38.0 °C over -48 to 0 h (°C·h). Thresholds of 38.5 °C and 39.0 °C were evaluated in sensitivity analyses. Prespecified covariates, chosen based on prior RMPP/SMPP studies and pediatric CAP/MPP guidelines, included age, illness days, SpO2, imaging severity, prior macrolide exposure, antipyretic and steroid use, LDH, log2-transformed NLR, CRP, and PCT. Penalized logistic regression generated predicted risks, while unpenalized models provided adjusted odds ratios. Model performance (AUC, Brier score, calibration) and decision-curve analysis (DCA) net benefit were assessed for base (clinical + laboratory) and augmented (base + FBI48) models across 10-30% risk thresholds.</p><p><strong>Results: </strong>Of 720 eligible hospitalizations, 648 were analyzed. The composite endpoint (RMPP and/or incident SMPP) occurred in 176/648 (27.2%; RMPP 24.7%; SMPP 8.0%). FBI48 was independently associated with the composite endpoint (adjusted odds ratio [aOR] 1.45, 95% CI 1.25-1.68 per 1 SD ≈22 °C·h) and with SMPP alone (aOR 1.58, 95% CI 1.21-2.06). Adding FBI48 to the base model improved AUC from 0.78 to 0.83 (ΔAUC 0.05, p<0.001), reduced the Brier score (0.176 to 0.164, p=0.006), and increased net benefit compared with the base model and a treat-none strategy across 10-30% thresholds. Alternative fever thresholds (38.5 °C/39.0 °C) yielded similar effect sizes.</p><p><strong>Conclusions: </strong>A simple 48-hour pre-admission fever-burden metric provides independent and incremental prognostic information on the risk of refractory or severe evolution in pediatric MPP, complementing guideline-based clinical and laboratory predictors and supporting admission-time risk stratification. External validation and prospective evaluation of dynamic, in-hospital updating are warranted.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026034"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13170790/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147942045","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":"Atypical Evolution of a Vaso-Occlusive Presentation in Sickle Cell Disease: Lessons in Diagnostic Vigilance.","authors":"C Giubbilei, E Angeli, F Fossi, A Baffioni, G Giustini, V Carrai","doi":"10.4084/MJHID.2026.042","DOIUrl":"10.4084/MJHID.2026.042","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"18 1","pages":"e2026042"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13170798/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147942077","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}