EJHaem最新文献

{"title":"Paternal Split-Liver Transplantation Followed by Haploidentical Hematopoietic Cell Transplantation in an Adult Patient With Protoporphyria-Induced Liver Failure","authors":"Ulrich Stölzel,&nbsp;Lasse Jost,&nbsp;Daniel Seehofer,&nbsp;Katharina Egger-Heidrich,&nbsp;Uwe Scheuermann,&nbsp;Kristina Hölig,&nbsp;Thomas Stauch,&nbsp;Desiree Kunadt,&nbsp;Detlef Schuppan,&nbsp;Johannes Schetelig,&nbsp;Nils Wohmann,&nbsp;Martin Bornhäuser,&nbsp;Friedrich Stölzel","doi":"10.1002/jha2.1092","DOIUrl":"https://doi.org/10.1002/jha2.1092","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Erythropoietic Protoporphyria (EPP) caused skin light sensitivity and liver cirrhosis in a 35-year-old patient who subsequently developed liver-failure.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In absence of a human leukocyte antigens (HLA)-matched-unrelated donor, the father consented in donating for split liver transplantation (SLT) and allogeneic hematopoietic cell transplantation (HCT).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>After bridging therapy and successful SLT a first paternal HCT resulted in graft failure. For a second haploidentical HCT a different regimen was applied leading to engraftment while protoporphyrin (PP) blood-levels decreased to normal and skin light sensitivity skin disappeared, leading to complete remission in an immunosuppressive-free patient.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Haploidentical transplantation is a feasible treatment approach in EPP-patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Trial Registration</h3>\u0000 \u0000 <p>The authors have confirmed clinical trial registration is not needed for this submission</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1092","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143431677","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":"Acute myeloid leukemia with RAM immunophenotype: A report of three patients and comprehensive literature review","authors":"Xenia Parisi,&nbsp;Anindita Ghosh,&nbsp;L. Jeffrey Medeiros","doi":"10.1002/jha2.1074","DOIUrl":"https://doi.org/10.1002/jha2.1074","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>The RAM immunophenotype (IP) in acute myeloid leukemia (AML) is defined by blasts with bright CD56 and weak-to-negative CD45, HLA-DR, and CD38 expression. A RAM IP predominantly presents in infants who have “standard-risk disease” under current criteria but, when treated accordingly, have devastatingly high rates of minimal residual disease and relapse with lower 3-year and overall survival rates. However, given the relative rarity of this phenotype, it is neither well-defined nor readily diagnosed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We reviewed the electronic medical records of our institution from 1990 to 2024 for cases of AML expressing bright CD56 on flow cytometry and identified three cases with a RAM IP. Further, we performed a thorough literature search and reviewed impactful studies on pediatric AML and case/series reports of patients with a RAM IP, leading to the identification of 38 more cases.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 41 patients were collected. These patients were toddler age (1–3 years) with an equal sex distribution and clinically presented with low circulating blasts and cytopenias. Blasts were typically French–American–British M0 or M7. Immunophenotypically, CD33 and CD117 showed positivity in &gt;90% of cases, with CD19, CD34, CD41, and CD42b, frequently positive. Half of the cases were positive for CD7 and CD61. T-cell/myeloid markers were rare, except for cytoplasmic CD3, seen in 1/3, apparently correlating with <i>CBFAT2T3:GLIS1</i> fusions. Gains in chromosomes 21, 13, and 8 and <i>CBFAT2T3::GLIS1</i> fusions were frequent.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>AML with a RAM IP has a poor prognosis. This study offers a detailed characterization of the clinicopathologic patterns associated with this rare entity, which may help formulate the most appropriate diagnostic approach.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1074","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143389083","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":"Hodgkin Lymphoma Treatment With Brentuximab Vedotin in a Patient With HIV, Liver Failure, and Anemia Without Transfusion","authors":"Ellen Marin,&nbsp;Aseala Abousaud,&nbsp;Kathleen Davis,&nbsp;Bradley Sumrall,&nbsp;Shiyong Li,&nbsp;Amelia A. Langston,&nbsp;Andres Chang","doi":"10.1002/jha2.70001","DOIUrl":"https://doi.org/10.1002/jha2.70001","url":null,"abstract":"<p>Brentuximab vedotin (BV) is a drug that has improved outcomes in classical Hodgkin lymphoma (cHL). However, its safety and efficacy in patients living with HIV who present with severe liver failure and life-threating cytopenias is unclear. Here, we describe the case of a woman living with HIV diagnosed with cHL and how she recovered from a hemoglobin nadir of 24 g/L despite declining transfusion support and a bilirubin peak of 417.24 µmol/L, eventually achieving a complete response with a BV-based therapy. Studies are needed to determine the safety and efficacy of BV in patients excluded from the pivotal trials.</p><p><b>Trial Registration</b>: The authors have confirmed clinical trial registration is not needed for this submission.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143388962","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":"Treatment of Myeloproliferative Neoplasms With Janus Kinase Inhibitors: A Meta-Analysis of Cardiovascular Safety","authors":"Roberta Dunn,&nbsp;Edouard Long,&nbsp;Laura Li Gagnon,&nbsp;Claire Harrison,&nbsp;Yunfan Yang,&nbsp;Jennifer O'Sullivan","doi":"10.1002/jha2.70000","DOIUrl":"https://doi.org/10.1002/jha2.70000","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Janus kinase inhibitors (JAKis) are an integral aspect of the management of myeloproliferative neoplasms (MPNs). Part of the clinical benefit derived from JAKis may be due to reductions in thrombosis, a potentially life-threatening complication of MPNs. However, evidence has emerged of adverse cardiovascular effects secondary to JAKis. We conducted a first-of-a-kind meta-analysis of the cardiovascular safety of JAKis in the treatment of MPNs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This study was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA). Systematic searches for studies comparing JAKi treatment to a control group were conducted. Studies reporting hypertension, major adverse cardiovascular events (MACE) and thromboembolic events were included in a meta-analysis using a random-effects model for the primary analysis, and fixed-effects model for any subgroup analyses performed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 23 publications, consisting of nine clinical trials and one retrospective analysis, met the inclusion criteria. This resulted in a pooled population of 2198 patients (JAKi <i>n</i> = 1145, Control <i>n</i> = 1053). In studies reporting thromboembolic events (<i>n</i> = 9), pooled analysis revealed a significantly lower rate of thromboembolic events in the JAKi group (incidence rate ratio (IRR): 0.52, 95% CI: 0.28–0.98, <i>p</i> = 0.04). This was primarily driven by ruxolitinib studies in myelofibrosis (MF) and polycythemia vera (PV) as when a subgroup analysis of these trials was performed (<i>n</i> = 7), an even more significant reduction in thromboembolic events with JAKi treatment was found (IRR: 0.41, 95%CI: 0.26–0.64, <i>p</i> &lt; 0.001). There was no significant difference in MACE or hypertension between JAKi and control groups.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This meta-analysis suggests that JAKi treatment of MPN was associated with a reduced risk of thromboembolic events; primarily driven by studies of ruxolitinib in PV and MF. Further prospective clinical trials are warranted to confirm these findings and characterise the cardiovascular profile of other JAKis and other types of MPNs.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70000","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143389030","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":"An Economic Evaluation of the TROG 99.03 Trial: Systemic Therapy After Radiotherapy in Early-Stage Follicular Lymphoma","authors":"Daniel Erku,&nbsp;Joshua W. D. Tobin,&nbsp;John F. Seymour,&nbsp;Michael MacManus,&nbsp;Paul Scuffham,&nbsp;Greg Hapgood","doi":"10.1002/jha2.70002","DOIUrl":"https://doi.org/10.1002/jha2.70002","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The TROG 99.03 trial demonstrated improved progression-free survival for patients with early-stage follicular lymphoma (FL) treated with systemic therapy using rituximab-cyclophosphamide, vincristine, prednisolone (R-CVP) after involved-field radiotherapy (RT) versus RT. As systemic therapy was associated with more acute toxicity, the possibility of long-term toxicity, and no survival benefit yet, the cost-effectiveness of RT+R-CVP is important.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>We performed a cost-effectiveness analysis of RT (reference), RT+CVP, and RT+R-CVP from the TROG 99.03 trial.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We constructed a Markov model (15-year horizon) to compare treatments: RT (reference), RT+CVP and RT+R-CVP from the 150 patients in the TROG 99.03 trial. Median follow-up was 11.3 years (range: 4.4–17.8). Lifetime direct health care costs, quality-adjusted life-years (QALYs) and incremental cost-effectiveness ratios (ICERs) were calculated. Australian dollars AUD$50,000 was defined as the proposed willingness-to-pay threshold (WTP).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>RT+R-CVP was associated with an improvement of 0.711 QALYs compared to RT, 0.532 QALYs compared to RT+CVP, and was the dominant strategy. The costs of adverse events or retreatment for relapses or transformation had a minimal influence on the ICERs. Sensitivity analyses resulted in ICER values below the WTP with RT+R-CVP remaining the dominant strategy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>RT+R-CVP is clearly cost-effective and was the dominant strategy in early-stage FL compared to RT or RT+CVP as it delivers superior outcomes at a lower cost from the Australian tax-payer's perspective.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Trial Registration</h3>\u0000 \u0000 <p>The authors have confirmed clinical trial registration is not needed for this submission</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143389084","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":"Therapeutic Versus Non-Therapeutic Dose Anticoagulation in COVID-19 Infection: A Systematic Review and Meta-analysis of Randomised Controlled Trials","authors":"Sushil Selvarajan,&nbsp;Jisha Sara John,&nbsp;Prathap Tharyan,&nbsp;Richard Kirubakaran,&nbsp;Bhagteshwar Singh,&nbsp;Biju George,&nbsp;Joseph L. Mathew,&nbsp;Priscilla Rupali","doi":"10.1002/jha2.1100","DOIUrl":"https://doi.org/10.1002/jha2.1100","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Abnormal coagulation and thrombotic complications prompted many guidelines to recommend thromboprophylaxis for patients hospitalised with COVID-19, but the dose required for prophylaxis remains unclear. This systematic review (SR) analyses the safety and efficacy of therapeutic dose anticoagulation (TDA) versus non-therapeutic dose anticoagulation (NDA) in COVID-19 patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>According to the <i>Cochrane Handbook of Systematic Review of Interventions</i>, we performed an SR. The protocol is registered in Prospero (CRD42021269197, date 12 August 2021).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In this SR of 18 studies, TDA was shown to reduce all-cause mortality (risk ratio [RR] 0.83; 95% confidence interval [95% CI] 0.70, 0.99) in COVID-19 infection. TDA also reduced thrombosis (RR 0.55; 95% CI 0.48, 0.72) but increased major bleeding (RR 1.87; 95% CI 1.29, 2.69). A stratified analysis according to severity revealed that, in non-critical patients, TDA resulted in mortality benefit (RR 0.79; 95% CI 0.67, 0.94). In critical patients, TDA did not affect all-cause mortality (RR 1.03; 95% CI 0.89, 1.18) but reduced thrombosis (RR 0.65; 95% CI 0.48, 0.86) and increased major bleeding (RR 1.85; 95% CI 1.06, 3.23).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>TDA significantly reduced all-cause mortality and thrombosis in non-critical COVID-19 patients at the expense of increased major bleeding. In critical COVID-19, this mortality benefit was not observed.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1100","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143380721","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":"A Rare Case of Trans-differentiation of Follicular Lymphoma into Histiocytic Sarcoma","authors":"Dina Osman,&nbsp;Reem Ahmed","doi":"10.1002/jha2.1107","DOIUrl":"https://doi.org/10.1002/jha2.1107","url":null,"abstract":"<p>A 55-year-old previously healthy gentleman presented with a few months’ history of generalised lymphadenopathy and drenching night sweats. PET CT scan showed enlarged lymph nodes above and below the diaphragm with an SUV max of 17.4 at the left external iliac lymph node.</p><p>The lymph node biopsy showed focal effacement by a solid appearing diffuse infiltrate of large neoplastic cells with abundant cytoplasm and pleomorphic nuclei (upper panel, left H&amp;E ×20) with high mitotic rate, reflected by high Ki-67 index (Figure 1). These cells were diffusely positive for CD68 (upper panel, middle), CD163 (upper panel, right), CD4 (lower panel, left), S-100 and lysozyme, with variable OCT2 and cyclin D1 positivity. These features were strongly suggestive of high-grade histiocytic sarcoma (HS). A small focal lymphoid infiltrate of low-grade follicular lymphoma (FL) was noted in areas unaffected by the HS that was CD10+, CD20+ and strongly BCL2+ (lower panel, middle) with low Ki-67 index. Molecular analysis revealed that the HS and FL infiltrates shared t(14;18), suggesting trans-differentiation of FL into HS.</p><p>The bone marrow (BM) morphology showed heavy infiltration by the HS (lower panel, right ×100). A small kappa+ CD10+ FL infiltrate was detected by flow cytometry analysis of the BM.</p><p>The patient was treated with CHOP regimen but, unfortunately, passed away due to severe cytokine release syndrome, tumour lysis and multi-organ failure.</p><p>HS is a very rare and extremely aggressive disorder with somewhat better prognosis if diagnosed at an early stage [<span>1</span>]. It can arise de novo or via trans-differentiation from B-cell malignancies [<span>2</span>], as was demonstrated by this case.</p><p>Dina Osman and Reem Ahmed wrote the manuscript. Dina Osman took the photographs.</p><p>The authors declare no conflicts of interest.</p><p>The authors have nothing to report</p><p>Verbal consent for publication was obtained from the Late's next of kin.</p><p>The authors have confirmed clinical trial registration is not needed for this submission</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1107","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143362789","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":"Intramedullary erythrophagocytosis in myelodysplastic syndrome with heterozygous U2AF1 Q157R variant","authors":"Kritika Krishnamurthy,&nbsp;Aditi Shastri,&nbsp;Yanhua Wang","doi":"10.1002/jha2.1084","DOIUrl":"https://doi.org/10.1002/jha2.1084","url":null,"abstract":"<p>This report highlights a somewhat unique case of U2AF1 mutated myelodysplastic syndrome (MDS) with morphological evidence of increased intramedullary erythrophagocytosis, in the absence of obvious clinical signs of hemolysis. These findings merit investigation in a larger cohort of U2AF1 mutated MDS cases to further delineate the morphological spectrum of ineffective intramedullary hematopoiesis and nonimmune hemolysis, including features distinctive to S34 and Q157 variants.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1084","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143362418","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":"Immune Signatures Identify Patient Subsets Deriving Long-Term Benefit From First-Line Rituximab in Follicular Lymphoma","authors":"Ginevra Lolli,&nbsp;Alessandro Davini,&nbsp;Valentina Tabanelli,&nbsp;Maria Rosaria Sapienza,&nbsp;Federica Melle,&nbsp;Giovanna Motta,&nbsp;Marcello Del Corvo,&nbsp;Angelica Calleri,&nbsp;Anna Vanazzi,&nbsp;Paulina Nierychlewska,&nbsp;Alessio Maria Edoardo Maraglino,&nbsp;Marta Castelli,&nbsp;Maria Chiara Quattrocchi,&nbsp;Roberto Chiarle,&nbsp;Stefano Pileri,&nbsp;Corrado Tarella,&nbsp;Enrico Derenzini","doi":"10.1002/jha2.1103","DOIUrl":"https://doi.org/10.1002/jha2.1103","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The role of first-line single-agent rituximab immunotherapy in follicular lymphoma (FL) remains debated, as most patients eventually undergo chemotherapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this study, we retrospectively analyzed 81 FL patients treated with first-line single-agent rituximab monotherapy with (<i>n</i> = 53) or without (<i>n</i> = 28) consolidation. Fifty-one patients (63%) were high-tumor burden according to Group d'Etude des Lymphomes Folliculaires (GELF) criteria.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>After a median follow-up of 11 years, overall survival (OS) and progression-free survival (PFS) rates were 85% and 32%, respectively. Targeted gene expression profiling (T-GEP) was performed in 40 patients, revealing a 26-gene expression signature distinguishing complete responders and non-responders. This signature included genes involved in T-regulatory (Treg) and natural-killer cell activity, and interleukin-17 signaling. A simplified 14-gene prognostic score (ImSig) enabled accurate outcome stratification in terms of PFS. These data were validated in silico using two independent publicly available cohorts of FL patients treated with chemoimmunotherapy. Deconvolution analyses demonstrated an enrichment in Treg cells in high-risk ImSig patients, which was validated by immunohistochemistry.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>These findings demonstrate that the efficacy of front-line anti-CD20 immunotherapy may depend on microenvironment-related factors, and that specific immune signatures could identify patient subsets obtaining long-term benefit from a chemo-free immunotherapeutic approach.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Trial Registration</h3>\u0000 \u0000 <p>The authors have confirmed clinical trial registration is not needed for this submission.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1103","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143362419","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":"Longitudinal assessment of cerebral infarcts and small vessel disease using magnetic resonance imaging in antiphospholipid syndrome: A single-centre retrospective study","authors":"Yishi Tan,&nbsp;Andrew J. Doyle,&nbsp;Jayant Kumar,&nbsp;Peter Somerville,&nbsp;Uzma Faruqi,&nbsp;Anicee Danaee,&nbsp;Pu-Lin Luo,&nbsp;Beverley J. Hunt,&nbsp;Karen A. Breen","doi":"10.1002/jha2.1065","DOIUrl":"https://doi.org/10.1002/jha2.1065","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Stroke is the most frequent arterial thrombosis in antiphospholipid syndrome (APS) with high rates of recurrence.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods and patients</h3>\u0000 \u0000 <p>A retrospective, single-centre 10-year review of patients with APS having sequential cerebral magnetic resonance imaging (MRI) was performed to describe ischaemic features in APS and associated disease risk factors and progression over time.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 120 patients and 307 scans were included with 67% of patients receiving vitamin K antagonists (VKA). Note that 65% of patients had baseline ischaemic features with white matter hyperintensities (WMH), as a feature of small vessel disease (SVD), seen in 79% of abnormal scans. Fifteen percent of patients had progressive ischaemic changes with 83% demonstrating progressive WMH and 33% new infarcts (predominantly lacunar) on sequential scans. Progression-free survival for progressive ischaemia was 88% at 5 years. Multivariate analysis showed longer follow-up was a risk for developing progressive ischaemia (odds ratio [OR] 1.43, 95% confidence interval [CI] 1.13–1.86, <i>p</i> = 0.005). Hypertension (56% vs. 30%, <i>p</i> = 0.04) and ischaemic heart disease (22% vs. 6%, <i>p</i> = 0.04) were more prevalent with progressive ischaemia. There was no difference in progression or bleeding events according to VKA therapeutic intensity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>These results show SVD is a common feature of APS using MRI with progressive changes despite anticoagulation. Traditional risk factors for cerebrovascular disease were associated with progression.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1065","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143362752","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}