Annals of Hematology最新文献

{"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}

{"title":"An assessment of the effectiveness of hydroxyurea therapy in children and adolescents with sickle cell anaemia at Kenyatta National Hospital","authors":"Madline Yacoub-Kenyi Leju,&nbsp;Amsalu Degu","doi":"10.1007/s00277-026-07156-0","DOIUrl":"10.1007/s00277-026-07156-0","url":null,"abstract":"<div><p>Sickle cell anaemia (SCA) is a major public health concern in Kenya, particularly affecting children and adolescents. Despite the inclusion of hydroxyurea in national treatment guidelines, its real-world use and effectiveness in Kenya remain under-documented, especially in tertiary care settings such as Kenyatta National Hospital (KNH). The drug is known to reduce vaso-occlusive crises (VOCs), transfusions, and hospitalizations, but its impact in resource-limited environments requires further investigation. Hence, this study aimed to assess the clinical and hematological effectiveness of hydroxyurea therapy in children and adolescents with sickle cell anaemia at Kenyatta National Hospital. A retrospective one-arm cohort design was used to review medical records of pediatric patients with sickle cell anaemia (≤ 16 years) who were treated with hydroxyurea at Kenyatta National Hospital between January 2020 and December 2023. Eligible patients had received hydroxyurea therapy for a minimum duration of six months and had at least 12 months of documented follow-up data. Clinical outcomes, including the frequency of vaso-occlusive crises, hospitalizations, acute chest syndrome, stroke events, and transfusion requirements, were assessed alongside hematological markers such as hemoglobin level, mean corpuscular volume, and reticulocyte count. Data were collected using a structured data abstraction tool adapted from previously published studies. Statistical analysis was conducted using IBM SPSS Statistics version 29, with one-way analysis of variance used to examine changes in clinical and hematological parameters across multiple follow-up time points. A total of 54 children and adolescents with sickle cell anaemia (mean age: 6.8 ± 4.4 years) were included, all of whom had a substantial burden of disease at baseline, marked by frequent vaso-occlusive crises (2.6 ± 1.9), hospital admissions (2.4 ± 2.0), blood transfusions (2.9 ± 1.7), and episodes of acute chest syndrome (1.3 ± 0.9). Over the 3, 6, 12, and 24-month follow-up period after initiation of hydroxyurea therapy, overall clinical outcomes showed a general decline in the proportion of patients experiencing vaso-occlusive crises, hospitalizations, transfusion needs, and infections compared with baseline. Hematological parameters demonstrated progressive improvement, with mean hemoglobin rising from 7.8 ± 1.1 g/dL at baseline to 10.5 ± 2.0 g/dL at 24 months, mean corpuscular volume increasing from 77.0 ± 21.1 fL to 96.7 ± 13.2 fL, and reticulocyte counts decreasing from 16.3 ± 11.9% to 6.0 ± 6.9%. Hydroxyurea therapy was associated with sustained improvements in hematological indices, characterized by increased hemoglobin levels and mean corpuscular volume, alongside a reduction in reticulocyte counts over the follow-up period. These changes were accompanied by reductions in key clinical complications, including vaso-occlusive crises and hospitalizations.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13574882/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148337590","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":"Allogeneic hematopoietic stem cell transplantation for a pediatric case of myelodysplastic syndrome with germline DDX41 mutation","authors":"Tiantian Hu,&nbsp;Wenli Zhang,&nbsp;Teng Zhang,&nbsp;Man Wang,&nbsp;Zongxuan Huang,&nbsp;Yanling Tao","doi":"10.1007/s00277-026-07154-2","DOIUrl":"10.1007/s00277-026-07154-2","url":null,"abstract":"<div><p>According to the 2016 World Health Organization classifcation, a germline DEAD-box helicase 41 gene (DDX41) mutation with myeloid neoplasms(MNs) has been newly classifed. Such mutations are clearly linked to late-onset MNs, with patients typically presenting in middle to old age and often manifesting as high-risk AML or MDS. These patients are sensitive to hypomethylating agents, and allogeneic hematopoietic stem cell transplantation (allo-HSCT) is currently recognized as the curative treatment modality. However, pediatric cases of MNs associated with germline DDX41 mutations are extremely rare, resulting in significant gaps in understanding their clinical characteristics, treatment strategies, and prognostic data. This article reports a pediatric patient with high-risk myelodysplastic syndrome (MDS-EB-2) associated with a germline DDX41 mutation (c.316_320del) who underwent unrelated cord blood allogeneic hematopoietic stem cell transplantation. An 8-year-old female patient achieved bone marrow remission with azacitidine and venetoclax and subsequently underwent allogeneic hematopoietic stem cell transplantation from an unrelated umbilical cord blood donor, The conditioning regimen included fludarabine, decitabine, busulfan, and cyclophosphamide, leading to sustained donor engraftment. Post-transplant complications included HHV-6 encephalitis and grade III acute graft-versus-host disease (aGVHD) of the skin and gut, which were managed and controlled. At the 13-month follow-up, the patient remained in complete hematologic remission without recurrence. This case suggests that allo-HSCT is a feasible curative option for children with high-risk MDS and germline DDX41 predisposition, highlighting the critical importance of screening potential donors for the same mutation.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13574888/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148337498","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":"Socioeconomic deprivation, disease complications and access to preventive care in sickle cell disease in mainland France","authors":"Ondine Walter,&nbsp;Pierre Cougoul,&nbsp;Maryse Lapeyre-Mestre,&nbsp;Michelle Kelly-Irving,&nbsp;Margaux Lafaurie,&nbsp;Guillaume Moulis.","doi":"10.1007/s00277-026-07038-5","DOIUrl":"10.1007/s00277-026-07038-5","url":null,"abstract":"<div><p>Several studies have assessed the impact of socioeconomic factors on the health of sickle cell patients, yielding heterogeneous results. We conducted a population-based study using a health-insurance database in France to investigate this association, using longitudinal data over a 9-year follow-up time. Socioeconomic factors were assessed using the validated French deprivation index, an area-level index of social disadvantage. The study population comprised 17,315 patients with a median age of 23 years and a median follow-up of 8.1 years. Patients living in the most disadvantaged areas were at greater risk of vaso-occlusive events (VOEs): 70.4% of patients with a median cumulative time of hospitalization of 33.5 days over the study period in the disadvantaged socioeconomic group, compared to 67.9% of patients and 29.9 days in the comparison group (p = 0.035 and p &lt; 0.001). There was no difference regarding chronic complications, but a trend towards higher mortality in the disadvantaged group (HR: 1.13, 95% CI: 0.94—1.36). There was no difference between groups in access to preventive and curative care recommended in mainland France (vaccination, folic acid supplementation, antibiotic prophylaxis, hydroxycarbamide use).</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13574879/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148337567","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":"Immune Effector Cell–associated Hemophagocytic Syndrome (IEC-HS) as a late immune-mediated toxicity after CAR-T therapy in Diffuse Large B-Cell Lymphoma (DLBCL): a case report","authors":"Sofía Rincón-López,&nbsp;Ricardo García-Muñoz,&nbsp;Johelys Atencio-Matos,&nbsp;Olalla Pereda-Sainz,&nbsp;Javier Larreina-Pérez,&nbsp;Andrea Campeny-Najara,&nbsp;Pilar Herrera Pérez,&nbsp;Montserrat Hernández-Pérez,&nbsp;Jesús Feliu,&nbsp;Ada Esteban-Figuerola","doi":"10.1007/s00277-026-07105-x","DOIUrl":"10.1007/s00277-026-07105-x","url":null,"abstract":"<div><p>Chimeric antigen receptor (CAR) T-cell therapy has dramatically improved the prognosis of patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL) [1,2]. However, immune-mediated toxicities represent a significant emerging clinical challenge, particularly those with late onset or uncommon presentations. We report the case of a 71-year-old woman with heavily pretreated DLBCL who developed immune effector cell–associated hemophagocytic syndrome (IEC-HS) with a fatal outcome six months following CAR-T infusion. This case highlights the occurrence of late -onset CAR-T-related toxicities and the potential role of CAR-T cell persistence in sustaining dysregulated immune activation.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13572328/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148337501","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":"Clinical and prognostic significance of concomitant autoimmune disease in patients with myelofibrosis","authors":"Alessandro Costa,&nbsp;Olga Mulas,&nbsp;Marianna Greco,&nbsp;Martina Piras,&nbsp;Gabriele Accossu,&nbsp;Federica Pilo,&nbsp;Giovanni Caocci","doi":"10.1007/s00277-026-07128-4","DOIUrl":"10.1007/s00277-026-07128-4","url":null,"abstract":"<div><p>There is growing interest in the association between autoimmune diseases (AID) and myeloproliferative neoplasms (MPN), yet the specific clinical and prognostic implications of AID in myelofibrosis (MF) remain insufficiently characterized. A retrospective analysis was conducted in patients diagnosed with primary MF (PMF) or secondary MF (SMF). Concomitant AID diagnoses were identified through medical record review and included only cases with documented clinical diagnosis. A total of 135 patients with MF were analyzed, including 90 (66.7%) with PMF and 45 (33.3%) with SMF. At diagnosis, 31 AID diagnoses were recorded in 29 patients (21.5%), of which 54.8% were organ-specific and 45.2% systemic. AID was more frequent in female patients (<i>p</i> = 0.005). At a median follow-up of 37.3 months, 3-year overall survival (OS) was 78%, with inferior OS in patients with systemic AID (61%; <i>p</i> = 0.028) compared to those without AID (79%) or with organ-specific AID (88%). In multivariable analysis, systemic AID (HR 2.16, p = 0.043), anemia (HR 1.79, <i>p</i> = 0.041), age (HR 1.08, <i>p</i> &lt; 0.001), and JAK-inhibitor therapy (HR 0.57, <i>p</i> = 0.023) independently predicted mortality. Severe infections and thrombotic events were more frequent in AID patients (26% vs 9.3%, <i>p</i> = 0.0082), with significance retained for infections alone (<i>p</i> = 0.016). The presence of AID did not affect the rate of leukemic transformation. Systemic AID identifies a subset of MF patients with worse survival and increased susceptibility to infections, supporting intensified surveillance and tailored management strategies in this population.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 10","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13572334/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148337573","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}