Annals of Hematology最新文献

{"title":"Flow cytometric analysis of T-cell subsets and platelet surface markers in patients with immune thrombocytopenia: a case-control study","authors":"Merve Kara,&nbsp;Pelin Aytan,&nbsp;Cemil Gülüm,&nbsp;Mehmet Burak Yavuz Çimen","doi":"10.1007/s00277-026-07148-0","DOIUrl":"10.1007/s00277-026-07148-0","url":null,"abstract":"<div><p>Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by increased platelet destruction and impaired platelet production. The role of T-cell subsets and platelet surface markers in ITP pathogenesis remains incompletely understood. To evaluate T-cell subpopulations and platelet surface marker expression in patients with chronic ITP compared with healthy controls using flow cytometry. This prospective case-control study included 26 chronic ITP patients and 25 healthy controls from the Hematology Department of Mersin University Hospital. Flow cytometric analysis was performed to assess CD3+, CD4+, CD8+, CD25+, CD45RA+, CD62L−, and platelet surface markers including CD41, CD42, and CD61. Statistical analyses were performed using SPSS version 22. ITP patients demonstrated significantly lower platelet counts (97,038 ± 64,596 vs. 256,920 ± 48,873/µL, <i>p</i> &lt; 0.001) and higher mean platelet volume values (11.67 ± 1.46 vs. 10.46 ± 0.77 fL, <i>p</i> = 0.001) compared with healthy controls. CD45RA+/CD4 + ratios were significantly lower in ITP patients (10.52 ± 5.88 vs. 14.45 ± 7.21%, <i>p</i> = 0.038). CD62L− expression was also decreased in the ITP group (51.50 ± 12.16 vs. 60.68 ± 8.40%, <i>p</i> = 0.003). Among platelet surface markers, CD41 and CD61 expressions were significantly reduced in ITP patients compared with controls (<i>p</i> &lt; 0.001 for both). Although CD42 expression did not differ significantly between the overall groups, subgroup analysis demonstrated significantly lower CD42 expression in patients with platelet counts below 30,000/µL (<i>p</i> &lt; 0.001). This study demonstrates alterations in T-cell differentiation markers and platelet surface glycoproteins in patients with chronic ITP. Reduced CD45RA+/CD4 + ratios, altered CD62L expression, and decreased CD41 and CD61 levels suggest ongoing immune dysregulation and abnormal platelet biology in these patients. Further longitudinal and functional studies are needed to clarify the mechanistic and clinical significance of these findings.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13619784/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lactate dehydrogenase (LDH) as a predictor of left atrial remodeling in patients with sickle cell anemia","authors":"Fábio Galvão,&nbsp;Felipe Soares Maia,&nbsp;Mabel Rodrigues Rios,&nbsp;Jersey Heitor da Silva Maues,&nbsp;Bruno Deltreggia Benites,&nbsp;Samuel de Souza Medina,&nbsp;Paula de Melo Campos,&nbsp;José Roberto Matos-Souza,&nbsp;Fernando Ferreira Costa,&nbsp;Wilson Nadruz,&nbsp;Sara Teresinha Olalla Saad","doi":"10.1007/s00277-026-07116-8","DOIUrl":"10.1007/s00277-026-07116-8","url":null,"abstract":"<div><p>In sickle cell anemia (SCA), multiple end-organs are affected by the chronic effects of hemolytic anemia and vaso-occlusive events, with cardiac complications being among the leading causes of death. Cardiac remodeling, including left atrial enlargement, may lead to diastolic dysfunction and increase the risk of atrial arrhythmias. Although hemolysis is an important mechanism in the systemic complications of SCA, the value of lactate dehydrogenase (LDH) as an isolated marker of atrial remodeling remains poorly explored. In this context, we evaluated whether this simple marker could predict left atrial enlargement in patients with SCA in a steady state. In a retrospective cross-sectional study, we reviewed medical records, including echocardiographic examinations, of patients with SCA treated at a referral center. LDH elevation, expressed as multiples of the upper limit of normal (LDH/ULN), was used for comparison. Eighty-one patients were included, with a mean age of 41.7 ± 11.4 years, and 60% were female. The median LDH/ULN was 1.39 (range: 0.45–5.4). Forty-four patients (54%) presented an elevated left atrial volume index (LAVi), and 52 patients (64%) had LDH/ULN &gt; 1.0. Receiver-operating characteristic (ROC) curve analysis identified 1.2 as the optimal LDH/ULN cut-off to detect elevated LAVi, and adjusted logistic regression showed that individuals with LDH/ULN &gt; 1.2 had a 3.70‑fold higher risk of elevated LAVi (confidence interval = 1.33–10.31; <i>p</i> = 0.012). Our study identified an association between LDH and increased LAVi, suggesting a potential role as a simple marker of atrial remodeling, and not solely as a marker of hemolysis.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13619708/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Real-world treatment patterns and outcomes in chronic lymphocytic leukemia: a multicenter retrospective study in Greece","authors":"Epameinondas Koumpis,&nbsp;Eva Minga,&nbsp;Persefoni Talimtzi,&nbsp;Maria Angelopoulou,&nbsp;Sofia Chatzileontiadou,&nbsp;Maria Dimou,&nbsp;Sotiria Besikli-Dimou,&nbsp;Maria Efstathopoulou,&nbsp;Dimitris Gogos,&nbsp;Evdoxia Hatjiharissi,&nbsp;Eleftheria Hatzimichael,&nbsp;Michail Iskas,&nbsp;Eliana Konstantinou,&nbsp;Ioannis Kotsianidis,&nbsp;Alexandros Machairas,&nbsp;Eugenia Mpalaoura,&nbsp;Anastasia Mpanti,&nbsp;Panagiotis Panagiotidis,&nbsp;Gerasimos Pangalis,&nbsp;Ioannis Papadopoulos,&nbsp;Maria Papaioannou,&nbsp;Marina Siakantaris,&nbsp;Niki Stavroyianni,&nbsp;Theodoros Vassilakopoulos,&nbsp;George Vrachiolias,&nbsp;Kostas Stamatopoulos,&nbsp;Anastasia Chatzidimitriou,&nbsp;Thomas Chatzikonstantinou","doi":"10.1007/s00277-026-07161-3","DOIUrl":"10.1007/s00277-026-07161-3","url":null,"abstract":"<div><h3>Purpose</h3><p>This study aims to characterize treatment patterns for chronic lymphocytic leukemia (CLL) in real-world clinical practice across Greece focusing on both treatment-naïve and relapsed or refractory (R/R) disease. We evaluated therapeutic patterns and outcomes [including overall survival (OS), time to next treatment or death (TTNT-D) and overall response rate (ORR)] and documented the incidence of other malignancies of patients who initiated first-line treatment between 2010 and 2024.</p><h3>Methods</h3><p>This is a panhellenic multicenter retrospective study of patients diagnosed with CLL between 2010 and 2020 from 8 centers in Greece.</p><h3>Results</h3><p>Overall, 1318 patients were included in the study. Fludarabine, cyclophosphamide and rituximab (FCR) was the most common first-line regimen (21.6%), followed by chlorambucil plus rituximab (16.3%) and ibrutinib-based therapies (14%). In the R/R setting, ibrutinib and venetoclax-based treatments, became the predominant therapeutic choices after 2014, replacing chemoimmunotherapy. Shifts in treatment patterns before and after 2016 reflected the incorporation of targeted agents into routine clinical practice, with a marked decline in FCR use and increased adoption of targeted therapies. Median OS was 162 months from diagnosis and 105.2 months (95% CI: 90.3–116.3) from first treatment. In patients receiving ibrutinib as first-line therapy, mOS was not reached, and median TTNT-D was 67.3 months.</p><p>Overall, 18 (1.4%) cases developed a second hematological malignancy and 59 (4.5%) were diagnosed with a solid tumor. The most common hematological malignancy was myelodysplastic neoplasms and the most common solid tumors included lung, skin, breast, colon and prostate cancers.</p><h3>Conclusion</h3><p>The findings suggest a gradual shift in CLL treatment patterns in Greece, with targeted agents increasingly replacing traditional chemoimmunotherapy in recent years and showing favorable survival outcomes.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07161-3.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148374327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Low- to moderate-dose thalidomide combination rescue after luspatercept failure in transfusion-dependent β-thalassemia: a real-world cohort study","authors":"Soulivanh Nanthachak,&nbsp;Cailan Wen,&nbsp;Zhaoping Gan,&nbsp;Lingyuan Pan,&nbsp;Rongrong Liu,&nbsp;Yumei Huang","doi":"10.1007/s00277-026-07100-2","DOIUrl":"10.1007/s00277-026-07100-2","url":null,"abstract":"<div><p>Background: A proportion of transfusion-dependent β-thalassemia (TDT) patients show suboptimal or heterogeneous response to luspatercept. We explored a structured rescue approach by adding low- to moderate-dose thalidomide after predefined luspatercept failure in real-world practice. Methods: This study was a single-center retrospective cohort study. Adults with TDT on luspatercept who met predefined criteria for suboptimal response (failure to achieve ≥ 33% reduction in RBC units over a 12-week window versus each patient’s baseline 12-week window, or failure to increase Hb by ≥ 1.0 g/dL under an unchanged transfusion trigger) received thalidomide 25–50 mg once daily while continuing luspatercept. Primary endpoint was 12-week transfusion independence (TI), defined as no transfusion for 12 consecutive weeks and Hb ≥ 9.0 g/dL. Secondary endpoints included ≥ 33%/≥50% reductions in RBC units over matched 12-week windows, longitudinal Hb change, and safety graded by CTCAE v5.0 and reported as exposure-adjusted incidence rates. Results: Eleven patients were included (median age 23 years; female 45.5%; β⁰/β⁰ 18.2%; prior splenectomy 63.6%). Twelve-week transfusion independence (TI) was achieved in 4/11 patients (36.4%; 95% CI 15.2–64.6). Proportions with ≥ 33% and ≥ 50% reductions in RBC units over matched 12-week windows were 45.5% (95% CI 21.3–72.0) and 45.5% (95% CI 21.3–72.0), respectively. Hemoglobin increased by 4.0 g/dL (Hodges–Lehmann, 95% CI 2.9–5.0; LMM p &lt; 0.001). Total exposure was 112 patient-months; EAIR for any-grade treatment-emergent adverse events was 8.0 per 100 patient-months. No ≥Grade 2 neurotoxicity or thrombosis occurred. Conclusions: In TDT patients with predefined luspatercept failure, adding low- to moderate-dose thalidomide was associated with improvements in hemoglobin levels and reductions in transfusion burden with favorable tolerability. These findings warrant prospective validation of this structured rescue approach.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07100-2.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148381501","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparative thromboembolic risk of romiplostim versus eltrombopag in immune thrombocytopenia: a propensity score-matched cohort study","authors":"Anand Shah,&nbsp;Rajvi Gor,&nbsp;Joshua Kra","doi":"10.1007/s00277-026-07160-4","DOIUrl":"10.1007/s00277-026-07160-4","url":null,"abstract":"<div><p>Immune thrombocytopenia (ITP) is associated with both bleeding and thrombotic complications. Thrombopoietin receptor agonists (TPO-RAs), particularly romiplostim and eltrombopag, are widely used as second-line therapy, yet comparative data on thromboembolic risk between these agents remain limited. We conducted a retrospective cohort study using the TriNetX federated research network, including adults with ITP who initiated romiplostim or eltrombopag between January 2010 and December 2025. ITP was defined using ICD-10 code D69.3. Patients with prior TPO-RA exposure, alternative thrombocytopenia etiologies, or thrombotic events within 1 year before treatment initiation were excluded. One-to-one propensity score matching was performed using demographics, comorbidities, prior therapies, concomitant medications, and laboratory variables. After matching, 3,055 patients remained in each group. Romiplostim was associated with a higher cumulative risk of any thromboembolism than eltrombopag at 30 days (7.2% vs. 5.7%; RR 1.27, 95% CI 1.05–1.54), 90 days (10.7% vs. 7.9%; RR 1.36, 95% CI 1.16–1.59), 1 year (14.9% vs. 12.2%; RR 1.22, 95% CI 1.07–1.39), and full follow-up (22.5% vs. 19.2%; RR 1.17, 95% CI 1.06–1.29). Portal vein thrombosis was more frequent with romiplostim at 1 year, although absolute event rates were low (RR 2.32, 95% CI 1.36–3.96). Mean platelet counts were similar at most time points. These findings suggest that romiplostim may be associated with higher cumulative thromboembolic risk than eltrombopag despite comparable platelet counts. Given the retrospective design and potential confounding, these results should be interpreted as hypothesis-generating.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07160-4.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148374296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of race on mutational profile and survival in acute myeloid leukemia: a single-institution experience (2014–2024)","authors":"Utsav Joshi,&nbsp;Fatima Tuz Zahra,&nbsp;Pujan Patel,&nbsp;Najla Al Ali,&nbsp;Zachary Thompson,&nbsp;Nagehan Pakasticali,&nbsp;Jinming Song,&nbsp;Vishakha Agrawal,&nbsp;Rory Shallis,&nbsp;Zhuoer Xie,&nbsp;Seongseok Yun,&nbsp;Andrew Kuykendall,&nbsp;Eric Padron,&nbsp;Alison Walker,&nbsp;David Sallman,&nbsp;Rami Komrokji,&nbsp;Jeffrey Lancet,&nbsp;Mohammad Hussaini,&nbsp;Onyee Chan","doi":"10.1007/s00277-026-07145-3","DOIUrl":"10.1007/s00277-026-07145-3","url":null,"abstract":"<div><p>Acute myeloid leukemia (AML) is a biologically heterogeneous hematologic malignancy, and racial disparities in outcomes remain incompletely characterized. This retrospective study analyzed 677 patients diagnosed with AML at Moffitt Cancer Center (2014–2024) to evaluate the impact of race on genomic profiles and clinical outcomes. The cohort comprised 18.6% Black, 19.1% Hispanic, and 62.3% White patients. While ELN 2022 risk groups and most driver mutations were similarly distributed, Black patients exhibited a higher frequency of <i>KRAS</i> mutations (<i>p</i> &lt; 0.05). With a median follow-up of 3.3 years, median overall survival (OS) was 2.2 years for White, 1.5 years for Black, and 2.4 years for Hispanic patients (<i>p</i> = 0.045). Notably, no survival differences existed within favorable or adverse ELN risk categories; however, Black patients with intermediate-risk disease experienced significantly inferior OS compared to White (<i>p</i> = 0.02) and Hispanic patients (<i>p</i> = 0.003). Among those receiving intensive induction, Black patients had significantly worse OS (<i>p</i> ≤ 0.001), while Hispanic patients treated with lower-intensity regimens demonstrated inferior OS compared to White patients (<i>p</i> = 0.01). In multivariable analysis, race was not an independent predictor of OS. Survival was independently driven by prior myeloid malignancy, ELN risk, allogeneic HCT, and mutations in <i>TP53</i> and <i>IDH2</i>. Regarding race-specific molecular drivers, <i>TP53</i> and <i>IDH2</i> mutations were associated with OS in White patients, <i>TP53</i> in Black patients, and <i>IDH1</i> in Hispanic patients. These findings suggest that while racial disparities exist in specific clinical subgroups, they are primarily driven by distinct molecular profiles, cytogenetic heterogeneity, and patient-specific clinical factors rather than race alone.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07145-3.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148374298","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early versus standard anti-thymocyte globulin administration in haploidentical transplantation using post-transplant cyclophosphamide: a pilot comparative study","authors":"Ghazaleh Kheiri,&nbsp;Negar Nejati,&nbsp;Kobra Bagheri Valami,&nbsp;Bita Shahrami,&nbsp;Sahar Tavakoli,&nbsp;Neda Alijani,&nbsp;Arash Safari Sabet,&nbsp;Maryam Barkhordar","doi":"10.1007/s00277-026-07147-1","DOIUrl":"10.1007/s00277-026-07147-1","url":null,"abstract":"<div><p>Combining rabbit anti-thymocyte globulin (ATG) with post-transplant cyclophosphamide (PTCy) reduces graft-versus-host disease (GVHD) after haploidentical peripheral blood stem cell transplantation (haplo-PBSCT) but may increase cytomegalovirus (CMV) reactivation due to intensified immunosuppression. Because ATG exposure at the time of graft infusion is influenced by administration timing, we evaluated whether earlier ATG delivery modifies CMV outcomes while maintaining comparable observed transplant outcomes in this small pilot cohort. In this single-center, non-randomized pilot study, 36 adults with acute leukemia undergoing first myeloablative haplo-PBSCT with a uniform ATG/PTCy platform were analyzed. The intervention cohort received rabbit ATG 2.5 mg/kg/day on days − 9 to − 7 (<i>n</i> = 18), while the control cohort received the same total dose on days − 3 to − 1 (<i>n</i> = 18). All patients received busulfan/cyclophosphamide conditioning, PTCy (40 mg/kg on days + 3 and + 4), and cyclosporine. Letermovir was not used. The primary endpoint was CMV reactivation (&gt; 500 copies/mL) within 90 days. The primary endpoint was not met: The 90-day cumulative incidence of CMV reactivation was similar between early and standard ATG cohorts (55.6% vs. 59.3%; <i>p</i> = 0.94). Early ATG was associated with numerically lower peak CMV viral loads (median 995 vs. 1,456 copies/mL; <i>p</i> = 0.86) and fewer cases of high-level viremia ≥ 1,000 copies/mL (22.2% vs. 33.3%). Neutrophil and platelet engraftment occurred earlier with early ATG (median 12.5 vs. 15 days, <i>p</i> &lt; 0.001; and 14 vs. 17 days, <i>p</i> = 0.042). Rates of grade III–IV acute GVHD (11.1% in both groups), relapse, non-relapse mortality, and two-year overall survival were comparable. Exploratory analyses suggested numerically lower CMV viral burden and faster hematopoietic recovery in the early ATG cohort, but these observations are hypothesis-generating only. Given the non-randomized design, historical control, and limited sample size, no conclusions regarding efficacy preservation or superiority can be drawn. Confirmation in larger, prospective, ideally randomized studies incorporating immune monitoring is warranted.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 9","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07147-1.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148366710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transcriptomic profiling of an in vivo diffuse large B-cell lymphoma model reveals molecular programs underlying CNS dissemination","authors":"So-Jeong Kim,&nbsp;Hyo-Kyung Pak,&nbsp;Hyunji Kim,&nbsp;Yongjun Lee,&nbsp;Hwal-Seok Choi,&nbsp;Jin-Woo Choi,&nbsp;Do Eon Kim,&nbsp;Jaewon Hyung,&nbsp;Hyungwoo Cho,&nbsp;Dok Hyun Yoon,&nbsp;Jin Roh,&nbsp;Chan-Sik Park","doi":"10.1007/s00277-026-07107-9","DOIUrl":"10.1007/s00277-026-07107-9","url":null,"abstract":"<div><p>Secondary central nervous system involvement (sCNSi) is a fatal complication of diffuse large B-cell lymphoma (DLBCL), yet its molecular determinants remain poorly understood. To investigate the mechanisms underlying CNS-preferential dissemination, we established an in vivo DLBCL xenograft model using two representative cell lines, OCI-LY19 and Toledo, followed by transcriptomic profiling of lymphoma cells isolated from the brain, peripheral organs, and parental cultures. Transcriptomic analyses revealed gene signatures associated with CNS-preferential dissemination, which were further evaluated in RNA-seq data from diagnostic tissues of patients with (<i>n</i> = <i>19</i>) or without (<i>n</i> = <i>16</i>) sCNSi. CNS tropism of DLBCL cells was associated with increased expression of genes involved in PI3K-Akt-mTOR and ARF6 trafficking signaling and decreased expression of sphingosine-1-phosphate receptor signaling. Patient-derived transcriptomes exhibited enrichment of pathways that were functionally related to the gene signatures observed in the xenograft model. These findings suggest that CNS-preferential dissemination in DLBCL is associated with coordinated signaling involving PI3K/ARF6 activation and suppression of S1P-mediated pathways, providing molecular insights with potential implications for risk stratification and therapeutic targeting in sCNSi.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13590448/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148359098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A systematic review of 47 published cases of extramedullary hematopoiesis in myelofibrosis: clinical patterns, treatment responses, and prognostic implications","authors":"Andrea Duminuco,&nbsp;Veronica Vecchio,&nbsp;Giulio Lavenia,&nbsp;Patrick Harrington,&nbsp;Annalisa Santisi,&nbsp;Arianna Sbriglione,&nbsp;Giuseppe A. Palumbo","doi":"10.1007/s00277-026-07146-2","DOIUrl":"10.1007/s00277-026-07146-2","url":null,"abstract":"<div><p>Extramedullary hematopoiesis (EMH) in myelofibrosis manifests beyond classical splenomegaly, causing life-threatening complications, yet lacks systematic characterization in the JAK inhibitor era. We systematically reviewed 36 publications (2011–2025), available on PubMed database, reporting 47 unique myelofibrosis-associated EMH cases, analyzing demographics, anatomic distribution, presentations, diagnostics, treatments, and outcomes.</p><p>Median age was 68 years (range 43–82; M: F 26:21); primary myelofibrosis predominated (64%). EMH favored atypical sites: abdominopelvic (28%), thoracic (26%; pulmonary/pleural), serosal (17%), cutaneous (13%). Symptomatic onset occurred in 75% (dyspnea 28%, abdominal pain 21%). CT/MRI revealed characteristic lobulated masses with T2 hypointensity; biopsy confirmed trilineage hematopoiesis with atypical megakaryocytes (CD61+). Ruxolitinib-based regimens, administered in 19 patients (40%) as EMH-directed therapy, yielded rapid palliation (dyspnea/ascites resolution), complemented by radiotherapy (11%) and splenectomy; HSCT achieved complete remission in one case. Mortality reached 38% (median follow-up 5 months), driven by EMH-related organ failure or AML transformation. Atypical EMH signals advanced myelofibrosis with dismal prognosis, responsive to JAK inhibitors/locoregional therapies but highlighting sanctuary biology. Systematic surveillance and transplant referral are imperative; prospective registries are needed to refine risk models and test novel antifibrotics.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07146-2.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148359091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case report of CD4− CD8+ CD56− TCRγδ+ T-large granular lymphocytic leukaemia with severe aplastic anemia and a PNH clone","authors":"Yufei Du,&nbsp;Jinling Zhang,&nbsp;Shengyu Jin","doi":"10.1007/s00277-026-07141-7","DOIUrl":"10.1007/s00277-026-07141-7","url":null,"abstract":"<div><p>T-large granular lymphocytic leukemia (T-LGLL) is a rare, indolent lymphoproliferative disorder of lymphocytes. Clonal expansion of T cells, driven by dysregulation of Fas-mediated apoptosis following activation, is a hallmark of this disease. In recent years, research on large granular T-cell leukemia has grown substantially compared with the past; however, most reports have focused on TCRαβ⁺ cases, while studies on other subtypes remain limited, and diagnostic experience with coexisting conditions requires further refinement. This study reports, for the first time, a case of CD4⁻ CD8⁺ CD56⁻ TCRγδ⁺ T-LGLL complicated by severe aplastic anemia (AA) and a paroxysmal nocturnal hemoglobinuria (PNH) clone. The case is characterized by positive TCRγδ gene rearrangement, with T-cell–mediated hematopoietic suppression leading to the development of AA and the PNH clone. We hope this study will address a gap in the current literature. Here, we summarize the common pathophysiological mechanisms and guiding treatment principles for these three diseases.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 9","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13546363/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148343761","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}