Journal of Blood Medicine最新文献

{"title":"Aztreonam-Avibactam Successfully Treated Severe <i>Stenotrophomonas maltophilia</i> Infection After Allogeneic Transplantation: A Case Report and Literature Reviews.","authors":"Honglei Wang, Jia Song, Hui Liu, Shanfeng Hao, Chongjie Pang, Rong Fu","doi":"10.2147/JBM.S618181","DOIUrl":"10.2147/JBM.S618181","url":null,"abstract":"<p><strong>Background: </strong><i>Stenotrophomonas maltophilia</i> infection after allogeneic hematopoietic stem cell transplantation is rare and dangerous, especially bloodstream infection, with an extremely high mortality rate.</p><p><strong>Case presentation: </strong>In this study, we present a case of acute T-lymphoblastic leukemia developed St<i>enotrophomonas maltophilia</i> sepsis on the 8th day after allogeneic hematopoietic stem cell transplantation, accompanied by severe infections of the skin and soft tissues, pharynx, and gastrointestinal tract. After anti-infective treatment with aztreonam-avibactam, the patient's infection was effectively controlled. Neutrophil engraftment occurred on day 19 and platelet engraftment on day 30 post-transplantation. The patient was smoothly transferred from the transplant ward to a general ward.</p><p><strong>Conclusion: </strong>Aztreonam-avibactam may have a very good clinical effect on patients with severe and multi-site <i>Stenotrophomonas maltophilia</i> infections.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"618181"},"PeriodicalIF":2.1,"publicationDate":"2026-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13537200/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148880086","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}

{"title":"Longstanding Microcytosis with a Thalassaemia-Like Phenotype and Inconclusive Molecular Testing: A Case Report.","authors":"Fahd A Kuriri","doi":"10.2147/JBM.S627239","DOIUrl":"10.2147/JBM.S627239","url":null,"abstract":"<p><strong>Background: </strong>Persistent microcytosis in adults with normal or only mildly reduced haemoglobin usually prompts investigation for iron deficiency or thalassaemia trait. In some patients, however, the cause remains uncertain despite haemoglobin fractionation and targeted molecular testing.</p><p><strong>Case presentation: </strong>An asymptomatic adult male had longstanding microcytosis, with haemoglobin of 13.6 g/dL, a red blood cell count of 5.67 × 10⁶/µL, and a mean corpuscular volume of 72 fL. Serum iron was low, but ferritin remained within the reference range at 100.5 ng/mL. Haemoglobin fractionation showed HbA₂ of 2.4% and HbF below 1%, without an abnormal fraction. Multiplex PCR, sequencing of HBA2, HBA1, and HBB, and MLPA of the alpha-globin gene cluster identified no causative deletion, sequence variant, or copy-number change within the scope of testing. Rare or untested mechanisms remained possible. Empirical iron treatment was not initiated.</p><p><strong>Interpretation: </strong>The longstanding microcytosis, relatively preserved red blood cell count, normal HbA₂ and HbF, and preserved ferritin were consistent with a thalassaemia-like phenotype. Classical raised-HbA₂ beta-thalassaemia trait was considered less likely. An inherited alpha-globin abnormality or another disorder of haemoglobin synthesis remained possible, although it could not be confirmed. The preserved ferritin made absolute iron deficiency less likely, but iron-restricted erythropoiesis could not be excluded because additional iron studies were unavailable.</p><p><strong>Conclusion: </strong>This case highlights the difficulty of interpreting a stable microcytic phenotype when molecular testing does not establish a causative abnormality. Molecular findings should be considered alongside the longitudinal haematological pattern and interpreted within the analytical scope of the methods used. Iron treatment should be reserved for patients in whom iron deficiency has been demonstrated by appropriate testing.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"627239"},"PeriodicalIF":2.1,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13532884/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148874278","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}

{"title":"Nationwide Distribution of ABO and Rh Blood Group Phenotypes Among Mexican Outpatients: A Multicenter Retrospective Cross-Sectional Study.","authors":"José Antonio Magaña-Lizárraga, Abraham García-Gil, Diana Yadira Calva-Espinosa, Yoseli Yoruby Leal-Leyva, Ariadna Patricia Langle-Martínez, Marco Antonio Luna-Ruiz-Esparza, Abraham Campos-Romero, Jonathan Alcántar-Fernández","doi":"10.2147/JBM.S625908","DOIUrl":"10.2147/JBM.S625908","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to determine the phenotypic distribution of ABO and Rh blood groups in a geographically diverse cohort of Mexican outpatients.</p><p><strong>Patients and methods: </strong>A clinic-based, retrospective, cross-sectional study was conducted using anonymized electronic health records (EHRs) from 1,820,793 individuals aged 1 to 100 years who underwent clinically or administratively indicated ABO and Rh blood typing at Salud Digna clinics across all 32 Mexican states between January 2023 and July 2025. All ABO and Rh determinations were performed by forward typing using either the fully automated Galileo Echo immunohematology analyzer or by the conventional slide method using commercial anti-A, anti-B, and anti-D antisera. Demographic and blood group distributions were summarized using descriptive statistical methods, and sex-specific distributions were evaluated.</p><p><strong>Results: </strong>Among the 1,820,793 individuals analyzed (median age: 29 years), 1,193,587 (65.6%) were female and 627,206 (34.4%) were male. Blood group O was the most frequent ABO phenotype (65.99%), whereas group AB was the least common (1.50%). For the Rh system, 3.39% of individuals exhibited the Rh(d) phenotype. The overall phenotypic distribution was as follows: O Rh(D) (64.01%), A Rh(D) (23.24%), B Rh(D) (7.92%), O Rh(d) (1.98%), AB Rh(D) (1.43%), A Rh(d) (1.02%), B Rh(d) (0.32%), and AB Rh(d) (0.07%). Notably, the AB Rh(d) phenotype was not identified in Oaxaca. From a geographical perspective, a clear north-to-south gradient was observed, with increasing frequencies of the O Rh(D) phenotype toward the southern states, accompanied by declining trends observed for the remaining ABO and Rh phenotypes.</p><p><strong>Conclusion: </strong>Blood group O was the most frequent ABO phenotype, whereas the Rh(d) phenotype was relatively uncommon. The overall distribution patterns were largely consistent across the states. These findings provide a valuable resource for health authorities and blood services to support blood inventory management, inform region-specific donor recruitment strategies for less common blood group phenotypes, and optimize planning for blood transfusion services, organ transplantation, and maternal healthcare.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"625908"},"PeriodicalIF":2.1,"publicationDate":"2026-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13528783/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865153","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}

{"title":"Correlation Between Von Willebrand Factor, Thrombin-Antithrombin Complex, and Disease Severity in Sickle Cell Disease: Implications for Thrombosis.","authors":"Akaba Kingsley Onorhide, Ogar Aje Nkarense, Amon Banturaki, Ushie Godwin","doi":"10.2147/JBM.S622341","DOIUrl":"10.2147/JBM.S622341","url":null,"abstract":"<p><strong>Introduction: </strong>Sickle cell disease (SCD) is a hypercoagulable state characterized by elevated procoagulant factors, including Von Willebrand factor (VWF), and enhanced thrombin generation, marked by increased thrombin-antithrombin complex (TAT). Research on VWF and TAT activity during vaso-occlusive crises (VOC) remains scarce in our region.</p><p><strong>Objective: </strong>To determine VWF antigen and TAT complex serum levels in SCD during steady state and VOC, and correlate these with disease severity.</p><p><strong>Methodology: </strong>This cross-sectional study evaluated 30 SCD patients during VOC and re-evaluated the same cohort during their steady state. A control group of 30 HbAA individuals was included. Serum was analyzed for VWF and TAT complex. Analysis of Variance (ANOVA) and Pearson correlation tests were utilized (p < 0.05).</p><p><strong>Results: </strong>Mean VWF levels were highest during VOC (500.9 ± 175.6 ng/mL), followed by steady state (421.5 ± 130.9 ng/mL) and controls (395.0 ± 115.2 ng/mL) (p=0.014). TAT complex levels were highest during VOC (28.9 ± 10.4 ng/mL) compared to steady state (24.1 ± 7.8 ng/mL) and controls (4.0 ± 4.2 ng/mL) (p<0.001). Steady-state VWF positively correlated with TAT (r = 0.458, p = 0.011), while steady-state TAT positively correlated with disease severity (r = 0.401, p = 0.028).</p><p><strong>Conclusion: </strong>VWF and TAT levels are significantly elevated in SCD patients, with further accentuation during VOC. These elevations strongly correlate with disease severity and specific clinical complications, establishing them as vital markers for evaluating thrombotic risk in SCD patients.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"622341"},"PeriodicalIF":2.1,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13526376/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865074","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}

{"title":"Clinical Outcomes of Rh(D)-Incompatible Allogeneic Haematopoietic Stem Cell Transplantation: A Single-Centre Retrospective Case Series with a Narrative Literature Review.","authors":"Miaomiao Liu, Min Lin, Weijia Fu, Ziwei Wang, Lili Xu, Jie Chen, Jianmin Yang, Haihui Gu, Xiong Ni","doi":"10.2147/JBM.S623234","DOIUrl":"10.2147/JBM.S623234","url":null,"abstract":"<p><p>Rh(D)-negative individuals are relatively rare in the Chinese population, and clinical experience with Rh(D)-incompatible allogeneic haematopoietic stem cell transplantation (allo-HSCT) remains limited. Compared with ABO incompatibility, the immunohaematological consequences of Rh(D) mismatch in allo-HSCT are less well defined. Herein, we report a single-centre retrospective case series of four patients receiving Rh(D)-incompatible allo-HSCT at our institution, including two Rh(D)-negative recipients with Rh(D)-positive donors and two Rh(D)-positive recipients with Rh(D)-negative donors. We systematically evaluated post-transplant transfusion strategies, haematological recovery, anti-D alloantibody seroconversion, haemolysis or graft-versus-host disease (GVHD), and long-term survival outcomes. During a median follow-up of 36 months (range, 24-48 months), none of the patients developed detectable anti-D alloantibodies. Only one patient exhibited laboratory evidence of subclinical haemolysis responsive to low-dose prednisone, and another patient developed grade II acute gastrointestinal GVHD controlled by immunosuppressive agents. Nevertheless, all patients achieved sustained haematological engraftment without transplant-related mortalities. Combined with data from a narrative literature review, our single-centre observations are consistent with prior studies showing low risks of clinically significant haemolysis and anti-D alloimmunisation after Rh(D)-incompatible allo-HSCT. Rh(D) mismatch does not appear to be associated with severe transplant complications in our limited cohort, but these findings cannot be generalised broadly given the extremely small sample size. Rigorous peri-transplant transfusion management and regular post-transplant serological monitoring are still mandatory for Rh(D)-mismatched recipients.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"623234"},"PeriodicalIF":2.1,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13525792/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148857418","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}

{"title":"The Effect of MEG3 Promoter Methylation on MEG3 Expression in Acute Myeloid Leukemia: Assessment by High-Resolution Melting (HRM) Analysis.","authors":"Ali Asgharzadeh, Alireza Moradabadi, Ali Noroozi-Aghideh","doi":"10.2147/JBM.S601460","DOIUrl":"10.2147/JBM.S601460","url":null,"abstract":"<p><strong>Introduction: </strong>Epigenetic mechanisms play a crucial role in regulating genome expression and are subject to dysregulation in leukemia, hence exerting a significant impact on the pathogenesis of this malignancy. Maternally expressed gene 3 (MEG3), a long non-coding RNA, has been identified as a tumor suppressor in many hematologic malignancies due to its frequent down-regulation. This study aimed to examine the expression and methylation status of MEG3 in patients diagnosed with acute myeloid leukemia by employing the High-Resolution Melting (HRM) method.</p><p><strong>Materials and methods: </strong>The study employed quantitative RT-PCR to assess the expression of MEG3 in peripheral blood mononuclear cells (PBMCs) derived from a cohort of 62 individuals diagnosed with AML and 32 individuals as healthy controls. The study used the methylation-sensitive high-resolution melting (MS-HRM) method to assess the methylation status of the MEG3 promoter region.</p><p><strong>Results: </strong>The MEG3 expression was significantly downregulated (P <0.0001) in patients with AML compared to healthy controls. Furthermore, the methylation level of the MEG3 promoter was significantly different between patients and controls (P<0.001). The average methylation rate of the MEG3 gene promoter in patients was 70.3%. A statistically significant negative association (r = -0.32, p = 0.03) was observed between MEG3 methylation and its expression.</p><p><strong>Discussion: </strong>The expression of the MEG3 gene exhibited a significant decrease, while the promoter methylation level was elevated in patients. These findings suggest that MEG3 may function as a tumor suppressor gene and may have potential as a prognostic biomarker in AML. These findings suggest that targeting aberrant MEG3 promoter methylation could represent a potential therapeutic strategy in AML, though further studies are warranted to confirm this hypothesis.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"601460"},"PeriodicalIF":2.1,"publicationDate":"2026-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13524113/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148850753","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}

{"title":"The Association Between Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratios and Anemia in Adults with Hypertension in Southwestern Uganda: A Cross-Sectional Study.","authors":"Enoch Muwanguzi, Jazira Tumusiime, Rogers Kalyetsi, Charles Nkubi Bagenda, Elizabeth A John, Benson Okongo","doi":"10.2147/JBM.S622343","DOIUrl":"10.2147/JBM.S622343","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to determine the association of neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) with anemia among hypertensive patients in Southwestern Uganda.</p><p><strong>Patients and methods: </strong>We conducted a secondary analysis of a cross-sectional study involving 386 consecutively enrolled hypertensive patients at Bwizibwera Health Centre IV, Mbarara District, Uganda. Anemia was defined as hemoglobin <12.0 g/dL in women and <13.0 g/dL in men. Data were analyzed using STATA. Categorical variables were compared using chi-square or Fisher's exact tests, continuous variables with the Mann-Whitney <i>U</i>-test. Logistic regression examined associations of NLR, PLR, and monocyte-to-lymphocyte ratio (MLR) with anemia. Predictive performance was assessed using receiver operating characteristic (ROC) curves.</p><p><strong>Results: </strong>Overall anemia prevalence was 17.1% (95% CI: 13.7-21.2). Prevalence was significantly higher among participants with uncontrolled versus controlled blood pressure (20.8% vs 12.6%; p=0.035). Anemic participants had significantly higher NLR (1.49 vs 1.14; p<0.001), PLR (156.06 vs 118.45; p<0.001), and MLR (0.23 vs 0.20; p=0.006). High NLR and PLR were independently associated with anemia. Participants in the top tertile of NLR had over twice the odds of anemia compared to those in the bottom tertile (aOR=2.29; 95% CI: 1.04-5.07; p=0.040). Similarly, those in the top tertile of PLR had higher odds of anemia (aOR=2.28; 95% CI: 1.05-4.97; p=0.038). Both ratios showed significant predictive ability to distinguish anemic from non-anemic participants: NLR AUC=0.63 (95% CI: 0.56-0.71); PLR AUC=0.64 (95% CI: 0.56-0.72).</p><p><strong>Conclusion: </strong>In hypertensive patients, higher NLR and PLR are independently associated with anemia. These inexpensive, readily available inflammatory hematological ratios may help identify individuals at elevated anemia risk.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"622343"},"PeriodicalIF":2.1,"publicationDate":"2026-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13520959/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840135","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}

{"title":"Evaluation of Determinants of Intraoperative Blood Loss During Craniotomy for Brain Tumors: A Retrospective Single-Center Cohort Study.","authors":"Riza Zahara, Bintang Pramodana, Riyadh Firdaus, Tesha Islami Monika, Radea Renoza","doi":"10.2147/JBM.S630851","DOIUrl":"10.2147/JBM.S630851","url":null,"abstract":"<p><strong>Background: </strong>Inefficient blood product management in craniotomy for tumor removal poses clinical and economic burdens. This study evaluates predictive factors for significant intraoperative blood loss to establish a robust perioperative risk stratification model.</p><p><strong>Materials and methods: </strong>This retrospective cohort study examined adults undergoing elective brain tumor resections between 2020 and 2024 at a national tertiary referral center. Our primary outcome was significant blood loss, defined as an estimated loss of at least 20% of the patient's total blood volume (EBV). Independent predictors were evaluated using multivariate logistic regression, specifically backward-elimination methods.</p><p><strong>Results: </strong>Out of 378 patients, 35% of patients experienced significant intraoperative blood loss. Bivariate analysis showed female gender, meningioma pathology, cerebral sinus involvement, tumor size ≥ 5 cm, and operation time ≥ 300 minutes associated with higher blood loss (p < 0.05). In the final multivariate model, tumor size ≥ 5 cm was the primary independent predictor (adjusted Odds Ratio [aOR] = 2.736; 95% Confidence Interval [CI]: 1.746-4.285; p < 0.001), followed by meningioma pathology (aOR = 2.104; 95% CI: 1.292-3.426; p = 0.003). Operation time ≥ 300 minutes in the final model (aOR = 1.703) was retained due to its clinical relevance in the operating room.</p><p><strong>Conclusion: </strong>Tumor size ≥ 5 cm and meningioma pathology were independently associated with significant intraoperative blood loss. Utilizing these factors as a preoperative screening tool allows clinical teams to anticipate significant blood loss, optimize cross-matching, and reduce institutional resource wastage.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"630851"},"PeriodicalIF":2.1,"publicationDate":"2026-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13511440/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148828600","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}

{"title":"Immunopathogenesis of Sickle Cell Disease: Mechanisms of Immune Dysregulation and Clinical Consequences, a Narrative Review.","authors":"Simeon Ikechukwu Egba, Virginus Agozie Umeh, Prince Ogochukwu Alaebo, Uchenna Valentine Chukwuma","doi":"10.2147/JBM.S636992","DOIUrl":"https://doi.org/10.2147/JBM.S636992","url":null,"abstract":"<p><p>Sickle cell disease (SCD) is a hereditary haemoglobin abnormality due to a point mutation in the β-globin gene resulting in the production of haemoglobin S. Polymerization of deoxygenated hemoglobin S results in red cell sickling, hemolysis, endothelial injury, ischemia-reperfusion damage, vaso-occlusion, and, in addition to being a hemolytic anaemia and vaso-occlusive condition, SCD is now considered a chronic inflammatory disease with significant immunological abnormalities. This review explores the pathophysiological and immunological basis of SCD, with particular emphasis on how hemolysis, inflammation, innate and adaptive immunity, vulnerability to infection and their implications for current and emerging therapeutics. SCD is characterised by sustained innate and adaptive immune responses, including leukocytosis; neutrophil and monocyte activation; changes in cytokine profiles; complement activation; and T- and B-cell dysfunction. Danger-associated molecular patterns released during hemolysis, including activate inflammasomes, oxidative stress, and endothelial dysfunction through toll-like receptors. Repeat vaso-occlusion maintains sterile inflammation and facilitates coagulation, immune regulation, and vascular damage. Also present are functional asplenia and defective humoral immunity, which predisposes to infection, particularly that caused by encapsulated bacteria. These disruptions help cause vaso-occlusive crises, acute chest syndrome, stroke, pulmonary hypertension, leg ulcers, nephropathy and long-term organ damage. Immunological dysregulation lies at the core of the pathophysiology and complications of SCD. A more detailed understanding of the immune landscape can enhance disease management and inform treatment, including drug administration, transfusion, hematopoietic stem cell transplantation, and gene-based therapies.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"636992"},"PeriodicalIF":2.1,"publicationDate":"2026-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13489050/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793608","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}

{"title":"Adolescent and Young Adult versus Older Adult Philadelphia-Negative Myeloproliferative Neoplasms: A Single-Center Experience from the MENA Region.","authors":"Shehab F Mohamed, Awni Alshurafa, Dina Sameh Soliman, Abdulrahman F Al-Mashdali, Mohammed Abdulgayoom, Anas M Babiker, Mahmoud Osman, Abdalla Fadul, Elmustafa Abdalla, Ahmed Abdelghafar Alsayed, Noor A Aweer, Manar E Abdel-Rahman, Omar Mohammad Ismail, Anil Yousaf, Mohamed Yassin","doi":"10.2147/JBM.S612879","DOIUrl":"https://doi.org/10.2147/JBM.S612879","url":null,"abstract":"<p><strong>Background: </strong>Philadelphia-negative myeloproliferative neoplasms (MPNs), including essential thrombocythaemia (ET), polycythaemia vera (PV), and myelofibrosis (MF), usually affect older adults, but a subset occurs in adolescents and young adults (AYA). Regional age-stratified data remain limited.</p><p><strong>Methods: </strong>We retrospectively studied Philadelphia-negative MPN patients managed at the National Center for Cancer Care and Research from 2014 to 2024. Patients were classified as AYA (15-39 years) or older adults (≥40 years). Clinical, laboratory, molecular, thrombotic, bleeding, transformation, and survival outcomes were analyzed by subtype. Risk categories followed institutional disease-specific practice; analyses were descriptive and unadjusted.</p><p><strong>Results: </strong>The cohort included 220 patients: 79 ET, 47 MF, and 94 PV; 76 (34.5%) were AYA. Low-risk disease predominated in AYA ET (p<0.001) and PV (72.4% vs 35.4%; p=0.002), partly reflecting age-based risk definitions. In ET (molecular n=30 vs 49), <i>CALR</i> was 16/30 (53.3%) versus 19/49 (38.8%) and <i>JAK2</i> was 6/30 (20.0%) versus 21/49 (42.9%); <i>MPL</i>, triple-negative, and unavailable categories were 2, 4, and 2 versus 2, 6, and 1, respectively. In MF (n=11 vs 36), <i>JAK2</i> was 4 (36.4%) versus 18 (50.0%), <i>CALR</i> 4 (36.4%) versus 10 (27.8%), <i>MPL</i> 0 versus 2 (5.6%), triple-negative 1 (9.1%) versus 3 (8.3%), and unavailable data 2 (18.2%) versus 3 (8.3%). In PV (n=29 vs 65), <i>JAK2</i> V617F was 28 (96.6%) versus 63 (96.9%) and <i>JAK2</i> exon 12 was 1 (3.4%) versus 2 (3.1%). Overall survival did not differ by age.</p><p><strong>Conclusion: </strong>AYA patients with Philadelphia-negative MPNs demonstrated age-related differences in risk classification, molecular profile, and thrombotic phenotype, while overall survival was comparable to older adults. Larger prospective multicenter studies are warranted.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"612879"},"PeriodicalIF":2.1,"publicationDate":"2026-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475427/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148764914","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}