Hematological Oncology最新文献

{"title":"Elranatamab for Relapsed/Refractory Multiple Myeloma With Severe Renal Impairment Requiring Hemodialysis","authors":"Michèle Hoffmann,&nbsp;Barbara Jeker,&nbsp;Uyen Huynh-Do,&nbsp;Yara Banz,&nbsp;Jeanne Godau,&nbsp;Elisabeth Weber,&nbsp;Ulrike Bacher,&nbsp;Thomas Pabst","doi":"10.1002/hon.70120","DOIUrl":"https://doi.org/10.1002/hon.70120","url":null,"abstract":"<p>Relapsed/refractory multiple myeloma (RRMM) patients with dialysis-dependent renal impairment face limited therapeutic options due to exclusion from clinical trials, a lack of evidence-based guidelines, and inferior outcomes. Bispecific antibodies targeting B-cell maturation antigen (BCMA) have shown promise in RRMM treatment but remain understudied in this vulnerable population. To illustrate this issue, we introduce the case of a 68-year-old female with triple-class RRMM and end-stage renal disease requiring hemodialysis, treated with elranatamab as a second line treatment following progression after therapy with daratumumab, bortezomib, lenalidomide, and dexamethasone. Despite experiencing grade I cytokine release syndrome during the initial administrations, symptoms were managed effectively with tocilizumab and dexamethasone, allowing treatment continuation. The patient achieved a very good partial remission within 7 weeks. Although hemodialysis dependence persisted, the therapy was well-tolerated with manageable adverse events. According to the literature, BCMA-directed immunotherapies, including teclistamab, belantamab mafodotin, and idecabtagene vicleucel, have shown efficacy in dialysis-dependent RRMM patients, though data remain limited. Pharmacokinetic analyses indicate that mild or moderate renal impairment does not have a significant impact on the pharmacokinetics of elranatamab. Although no retrospective studies or case series have investigated the use of elranatamab in dialysis-dependent patients, a single case report suggests that its administration is both feasible and well-tolerated in this population despite the absence of comprehensive pharmacokinetic data. This review highlights feasibility, safety, and encouraging efficacy of elranatamab in managing RRMM in a dialysis-dependent patient, representing the second case report in the literature. By providing real-world evidence for the use of bispecific antibodies in end stage renal disease patients, this review emphasizes the potential for expanding therapeutic options to this vulnerable population while highlighting the need for vigilant monitoring of infection prevention and management. Prospective studies are warranted to validate these findings and optimize therapeutic strategies for patients with RRMM and severe renal impairment.</p>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/hon.70120","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144663792","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Deubiquitinase TRIM44 Promotes Autophagy-Mediated Chemoresistance in Diffuse Large B Cell Lymphoma","authors":"Yan Wang,&nbsp;Banban Li,&nbsp;Yanan Zhao,&nbsp;Xunxun Zhu,&nbsp;Bo Wang,&nbsp;Lizhe Yang,&nbsp;Rui Feng,&nbsp;Qingliang Teng","doi":"10.1002/hon.70119","DOIUrl":"https://doi.org/10.1002/hon.70119","url":null,"abstract":"<p>Diffuse large B cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma. Tripartite motif containing 44 (TRIM44) belonging to the TRIM family, is involved in tumor development and is highly expressed in a variety of tumors. However, the role of TRIM44 in DLBCL remains undefined. Gain and loss-of-function studies were performed on lymphoblast cell lines DB and SU-DHL-4 to investigate its function. TRIM44 overexpression significantly promoted cell proliferation and viability, whereas its silencing inhibited proliferation and induced apoptosis. TRIM44 overexpression upregulated the LC3II/LC3-I ratio and Beclin1 expression, as well as increased autophagosomes formation, suggesting autophagy activation. Notably, TRIM44 conferred chemoresistance to doxorubicin in DB cells by increasing autophagic activity. In vivo study on mice revealed that TRIM44 overexpression increased Ki67 and PCNA expression, suggesting an increased tumor growth. Our previous work revealed that miR-665 is a tumor suppressor in DLBCL. The results of miRNA pull-down and luciferase reporter assay indicated that TRIM44 was a direct target of miR-665. In conclusion, TRIM44 promoted DLBCL progression by increasing autophagy-mediated chemoresistance, revealing the involvement of miR-665/TRIM44 axis.</p>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/hon.70119","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144647218","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of Body Composition on Treatment Toxicity and Outcomes in Patients With Primary Mediastinal Large B-Cell Lymphoma","authors":"Juliette Penichoux,&nbsp;Pierre Decazes,&nbsp;Cédric Rossi,&nbsp;Pierre Sesques,&nbsp;Corinne Haioun,&nbsp;Eric Durot,&nbsp;Nicolas Gower,&nbsp;Alexandre Willaume,&nbsp;Lucie Oberic,&nbsp;Jérome Paillassa,&nbsp;Chloé Antier,&nbsp;Loic Renaud,&nbsp;Olivier Tournilhac,&nbsp;Catherine Thieblemont,&nbsp;Caroline Besson,&nbsp;Laure Lebras,&nbsp;Sylvain Choquet,&nbsp;Katell Le Du,&nbsp;Christophe Bonnet,&nbsp;Sarah Bailly,&nbsp;Ghandi Damaj,&nbsp;Kamel Laribi,&nbsp;Roch Houot,&nbsp;Adrien Chauchet,&nbsp;Stéphanie Becker,&nbsp;David Tonnelet,&nbsp;Hervé Tilly,&nbsp;Fabrice Jardin,&nbsp;Emilie Lévêque,&nbsp;Vincent Camus","doi":"10.1002/hon.70117","DOIUrl":"https://doi.org/10.1002/hon.70117","url":null,"abstract":"<p>Primary mediastinal large B-cell lymphoma (PMBL) is a rare entity that predominantly affects young female patients and typically presents as a large and compressive anterior mediastinal mass. Accumulating evidence suggests relationships among PMBL patient body composition (BC), cancer outcomes, and treatment-related toxicities. The aim of this study was to evaluate the impact of BC on PMBL patients using PET-CT images acquired pretreatment. Two hundred nineteen patients were included in an ancillary analysis of a multicenter retrospective LYSA cohort of treatment-naïve adult PMBL patients who received first-line treatment with ACVBP, CHOP14 or CHOP21 plus anti-CD20. Anthropometric parameters were assessed from the baseline PET-CT image using two methods: (i) manual segmentation at the L3 level and (ii) automatic software-based multislice measurements with Anthropometer3DNet. The median age was 35.4 years (range 18–88 years), and the median body mass index was 23.8 kg/m² (15.6; 40.8). Overall, 137 patients were treated with R-ACVBP, 44 received R-CHOP14, and 38 were treated with R-CHOP21. Patients with low lean body mass had a higher incidence of febrile neutropenia, both in the overall cohort (25% vs. 12.6%, <i>p</i> = 0.02) and in the R-ACVBP subgroup (35.7% vs. 19.4%, <i>p</i> = 0.03). Univariate analysis showed that in patients treated with R-ACVBP, subcutaneous low adiposity, determined by 3D measurements, was associated with overall survival (OS) (<i>p</i> = 0.04). At 3 years, the OS (95% CI) was 96% (93–100) in above-median adiposity patients and 86% (78–95) in below-median adiposity patients. Low lean body mass (LBM), assessed from the pretreatment PET-CT images using automatic Anthropometer3DNet software, may serve as a predictive marker for acute treatment-related toxicity in PMBL patients, particularly those receiving the dose-intensive R-ACVBP regimen. Additionally, depletion of the subcutaneous fat mass was correlated with an increased risk of mortality, highlighting the importance of a comprehensive BC assessment in this patient population.</p>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/hon.70117","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144606568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Novel Prognostic Score for Disease Progression and Mortality in Patients With Newly Diagnosed Primary Large B-Cell Lymphoma of Immune-Privileged Sites","authors":"Ganggang Wang,&nbsp;Jiesong Wang,&nbsp;Cong Sun,&nbsp;Jingwei Yu,&nbsp;Chao Lv,&nbsp;Zheng Song,&nbsp;Xue Han,&nbsp;Lanfang Li,&nbsp;Lihua Qiu,&nbsp;Zhengzi Qian,&nbsp;Shiyong Zhou,&nbsp;Xia Liu,&nbsp;Xianhuo Wang,&nbsp;Jin He,&nbsp;Huilai Zhang","doi":"10.1002/hon.70115","DOIUrl":"https://doi.org/10.1002/hon.70115","url":null,"abstract":"<div>\u0000 \u0000 <p>Primary large B-cell lymphoma of immune-privileged sites (IP-LBCL), a recently defined entity in WHO-HAEM5, includes primary diffuse large B-cell lymphoma (DLBCL) occurring in immune-privileged areas like the central nervous system (PCNS-LBCL), vitreoretinal system (PVR-LBCL), and testis (PT-LBCL) in immunocompetent patients. This study aimed to identify prognostic factors and create a predictive model for IP-LBCL. We analyzed 213 newly diagnosed IP-LBCL patients from April 2006 to April 2023. A nomogram and prognostic index, IPLBCL-PI, were developed based on elevated LDH, ECOG ≥ 2, and PCNS-LBCL subtype as independent risk factors for poorer PFS. IPLBCL-PI categorized patients into four risk groups: low, low-intermediate, intermediate-high, and high. The model effectively predicted both PFS and OS in the training cohort and was validated in two external centers. Subgroup analyses showed that IPLBCL-PI outperformed the Nottingham/Barcelona (NB) and Memorial Sloan Kettering Cancer Center (MSKCC) models in PCNS-LBCL and was comparable to the International Prognostic Index (IPI) in PT-LBCL. IPLBCL-PI is the first prognostic model for IP-LBCL, offering risk stratification and aiding clinical decision-making for this rare entity.</p>\u0000 </div>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144589959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk Stratification for Diffuse Large B-Cell Lymphoma by Integrating Interim 18F-FDG PET-CT Analysis and the NCCN-IPI: A Multicenter Retrospective Study","authors":"Jiesong Wang,&nbsp;Yong Sun,&nbsp;Meifu Lin,&nbsp;Qinghu Lyu,&nbsp;Shudan Zhai,&nbsp;Zheng Song,&nbsp;Xia Liu,&nbsp;Lanfang Li,&nbsp;Lihua Qiu,&nbsp;Zhengzi Qian,&nbsp;Xing Wan,&nbsp;Shiyong Zhou,&nbsp;Wenchen Gong,&nbsp;Bin Meng,&nbsp;Bei Yu,&nbsp;Jin He,&nbsp;Xiaofei Ye,&nbsp;Lei Zhu,&nbsp;Xianhuo Wang,&nbsp;Huilai Zhang","doi":"10.1002/hon.70118","DOIUrl":"https://doi.org/10.1002/hon.70118","url":null,"abstract":"<div>\u0000 \u0000 <p>Our study aimed to assess the prognostic significance of the interim National Comprehensive Cancer Network International Prognostic Index and PET-CT-related parameters for predicting patient outcomes and achieving precise risk stratification for diffuse large B-cell lymphoma (DLBCL) patients. We retrospectively analyzed the clinicopathological and PET-CT data of 498 patients diagnosed with DLBCL across three medical centers in China. 418 patients were eligible for subsequent analysis after excluding those with incomplete data and 70% of which were randomly selected as the discovery cohort, whereas the remaining 30% constituted the validation cohort. The impact of candidate factors on survival was assessed via univariate and multivariate Cox proportional hazards models. The area under the curve AUC and C-index were calculated to assess the predictive performance of models. Univariate and multivariate Cox regression analyses identified changes in total lesion glycolysis (ΔTLG), iNCCN-IPI, interim abdominal residual disease (iARD) status, and changes in the maximum standardized uptake value (ΔSUVmax) as independent prognostic factors. Leveraging the outcomes of the multivariate analysis, we constructed the iPET-NCCN-IPI prognostic model and categorized DLBCL patients into two separate prognostic risk groups based on their computed Risk Scores (<i>RS = 0.90×iNCCN-IPI + 1.41×ΔTLG + 0.79×ΔSUVmax + 0.83×iARD</i>). The predictive performance of the model was validated by calculating the area under the receiver operating characteristic curve and the C-index. Notably, compared with other models, the iPET-NCCN-IPI demonstrated superior prognostic capability. In conclusion, our study indicates that the iPET-NCCN-IPI stratifies DLBCL patients into two distinct prognostic risk groups and surpasses other models in prognostic predictive ability.</p>\u0000 </div>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144573169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Efficacy and Safety of the Addition of Mitoxantrone Hydrochloride Liposome in Conditioning Regimen for High-Risk Acute Myeloid Leukemia","authors":"Xiaoyu Zhang,&nbsp;Donglin Yang,&nbsp;Aiming Pang,&nbsp;Sizhou Feng,&nbsp;Mingzhe Han,&nbsp;Yi He,&nbsp;Erlie Jiang","doi":"10.1002/hon.70116","DOIUrl":"https://doi.org/10.1002/hon.70116","url":null,"abstract":"<p>Despite allo-HSCT being the primary curative treatment for high-risk AML, relapse-free survival (RFS) remains suboptimal due to high relapse incidence. Our research focuses on optimizing the conditioning regimen by incorporating Mitoxantrone Hydrochloride Liposome (Lipo-MIT), a novel nano-formulation with enhanced pharmacokinetic properties and demonstrated anti-leukemic efficacy. Preclinical studies have shown that Lipo-MIT significantly improves survival outcomes compared to conventional mitoxantrone, and our study aims to translate these findings into clinical practice. In this study, we present the results of a Lipo-MIT as part of the conditioning regimen for high-risk AML patients undergoing allo-HSCT. Our findings highlight the potential of Lipo-MIT to improve RFS, while also providing insights into patient selection and the refinement of Lipo-MIT-based conditioning strategies. We believe this work contributes valuable knowledge to the field and has the potential to impact clinical practice.</p>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/hon.70116","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144550835","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploratory Analysis of Practical Predictive Indices for the Efficacy of Mogamulizumab in Patients With Aggressive Adult T-Cell Leukemia-Lymphoma","authors":"Yutaka Shimazu,&nbsp;Kenta Murotani,&nbsp;Hiroki Kitabayashi,&nbsp;Yukihiro Nishio","doi":"10.1002/hon.70114","DOIUrl":"https://doi.org/10.1002/hon.70114","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <p>An exploratory analysis of past clinical trials was conducted to propose a predictive scoring system for the efficacy of mogamulizumab, an anti-CC chemokine receptor 4 (CCR4) antibody, based on easily measurable parameters. Factors affecting progression-free survival (PFS) were investigated using data from three clinical trials (NCT00920790, NCT01626664, and NCT01173887) and one clinical study (UMIN000013294) conducted in patients with relapsed/refractory (R/R) or untreated CCR4-positive aggressive adult T-cell leukemia-lymphoma (ATL) receiving mogamulizumab treatment. Twelve routinely measured clinical parameters and three calculated indices—lymphocyte-to-neutrophil count ratio, platelet-to-lymphocyte count ratio, and lymphocyte-to-monocyte count ratio (LMR)—were selected as variables. Univariate Cox proportional hazards analysis identified albumin level, disease type, lactate dehydrogenase (LDH), monocyte count, neutrophil count, and LMR as relevant factors in R/R ATL patients treated with mogamulizumab monotherapy (<i>p</i> &lt; 0.05). A predictive model constructed from multivariate analysis results stratified the monotherapy group (<i>n</i> = 69) into three subgroups, with scores of 0 (<i>n</i> = 5), 1 (<i>n</i> = 25), and 2 (<i>n</i> = 39), based on LDH (0 for &lt; 265 and 1 for ≥ 265) and LMR (0 for ≥ 3.571 and 1 for &lt; 3.571). Median PFS values were 0.57, 0.46, and 0.07 years for scores 0, 1, and 2, respectively (log-rank test: <i>p</i> = 0.005 for score 0 vs. 2; <i>p</i> &lt; 0.001 for score 1 vs. 2). The simple model combining LDH and LMR may predict PFS in patients with R/R aggressive ATL receiving mogamulizumab treatment. Since LDH and LMR are easily measurable in clinical practice, this model could help predict mogamulizumab efficacy and guide treatment decisions in this patient population.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <p><b>Trial Registration:</b> Registration number: UMIN000049135. Date of registration: October 17, 2022</p>\u0000 </section>\u0000 </div>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/hon.70114","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144503062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Safety and Efficacy of Low-Dose Venetoclax Plus Voriconazole in Patients With Acute Myeloid Leukemia Unfit for Intensive Chemotherapy","authors":"Xinyao Liu,&nbsp;Danchen Meng,&nbsp;Yuxin Li,&nbsp;Min Ruan,&nbsp;Zhenqi Huang,&nbsp;Wei Wu,&nbsp;Jian Ge,&nbsp;Jichun Yang,&nbsp;Zhangbiao Long","doi":"10.1002/hon.70113","DOIUrl":"https://doi.org/10.1002/hon.70113","url":null,"abstract":"<div>\u0000 \u0000 <p>Low-dose venetoclax plus strong CYP3A4 inhibitor voriconazole were commonly used for acute myeloid leukemia (AML) patients who were unfit for intensive chemotherapy in China. However, the efficacy and safety of this schedule have not been well investigated. We analyzed clinical data from 54 patients with a median age of 67 years. Thirty patients received a standard dose of venetoclax plus azacitidine (cohort 1), whereas another 24 patients received low-dose venetoclax plus voriconazole plus azacitidine (cohort 2). The composite complete remission (complete remission or complete remission with incomplete hematologic recovery; CR/CRi) rate was 76.7% (23/30) in cohort 1 and 87.5% (21/24) in cohort 2 (<i>p</i> = 0.483). At a median follow-up of 16 months, the median progression-free survival was 12 months in cohort 1 and 18 months in cohort 2 (<i>p</i> = 0.241). The median overall survival was 14 months in cohort 1 and 19 months in cohort 2 (<i>p</i> = 0.453). Key adverse events included cytopenia and infections. Grade 3 or higher infections occurred in 36.7% of the patients in cohort 1 and 20.8% of those in cohort 2. In conclusion, this study demonstrated the safety and effectiveness of the combination of low-dose venetoclax and voriconazole in unfit AML.</p>\u0000 </div>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144503281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Outcome of Extramedullary Multiple Myeloma in the Era of Novel Agents: Insights From a Multicenter Study","authors":"Dong Liang,&nbsp;Yurong Yan,&nbsp;Qiaoli Wang,&nbsp;Shenrui Bai,&nbsp;Weiling Xu,&nbsp;Demei Feng,&nbsp;Yuying Bu,&nbsp;Min Zeng,&nbsp;Xiaomiao Nie,&nbsp;Yuan Feng,&nbsp;Xiaoqin Chen,&nbsp;Zhongjun Xia,&nbsp;Yang Liang,&nbsp;Fengyan Jin,&nbsp;Hua Wang","doi":"10.1002/hon.70112","DOIUrl":"https://doi.org/10.1002/hon.70112","url":null,"abstract":"<div>\u0000 \u0000 <p>This study aimed to discuss the clinical outcomes of extramedullary multiple myeloma in the era of novel agents, based on the largest dataset regarding extramedullary multiple myeloma in China. This study included 597 patients without extramedullary disease (EMD) (non-EMD), 324 with extramedullary bone-related disease (EMB) and 138 with de novo extramedullary extraosseous disease (EME). There were no significant differences in overall survival (OS, <i>p</i> = 0.638) or progression-free survival (PFS, <i>p</i> = 0.195) between non-EMD and EMB patients. However, de novo EME patients exhibited significantly worse OS (<i>p</i> &lt; 0.01) and PFS (<i>p</i> &lt; 0.01) compared to both EMB and non-EMD groups. Among non-EMD and EMB patients, those with ≥ 2 high-risk cytogenetic abnormalities (HRA) experienced extremely poor prognoses, categorizing them as ultra-high-risk multiple myeloma. Similarly, de novo EME patients with ≥ 1 HRA demonstrated very poor outcomes and should also be considered ultra-high risk. Notably, single transplantation was shown to mitigate the adverse prognosis of de novo EME patients. Furthermore, the daratumumab bortezomib lenalidomide dexamethasone (DVRD) quadruplet regimen showed potential as effective frontline therapies for de novo EME patients, offering hope for improved treatment outcomes in this challenging subgroup. These findings suggest that de novo EME represents an extremely poor prognosis and should be treated as a distinct entity within the multiple myeloma population. Furthermore, the results indicate that EMB may need to be excluded from the current EMD definition to better delineate these subgroups and guide therapeutic strategies.</p>\u0000 </div>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144339313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary Extranodal Follicular Lymphoma: A Retrospective Survey of the International Extranodal Lymphoma Study Group (IELSG)","authors":"Annarita Conconi,&nbsp;Andrea Janikova,&nbsp;Barbara Vannata,&nbsp;Ana Florencia Ramírez-Ibarguen,&nbsp;Chiara Lobetti-Bodoni,&nbsp;David Belada,&nbsp;Maria Cristina Pirosa,&nbsp;Michael Mian,&nbsp;Andrés J. M. Ferreri,&nbsp;Gail Ryan,&nbsp;Gerassimos Pangalis,&nbsp;Maria Elena Cabrera,&nbsp;Stefano Luminari,&nbsp;Silvia Montoto,&nbsp;Richard Tsang,&nbsp;Igor Aurer,&nbsp;Carlo Visco,&nbsp;Gloria Margiotta Casaluci,&nbsp;Vit Prochazka,&nbsp;Samuel Hricko,&nbsp;Anastasios Stathis,&nbsp;Luca Mazzucchelli,&nbsp;Maurilio Ponzoni,&nbsp;Massimo Federico,&nbsp;Gianluca Gaidano,&nbsp;Armando Lopez-Guillermo,&nbsp;Barbara Pro,&nbsp;Davide Rossi,&nbsp;Luciano Cascione,&nbsp;Grzegorz Nowakowsky,&nbsp;Marek Trneny,&nbsp;Emanuele Zucca","doi":"10.1002/hon.70111","DOIUrl":"https://doi.org/10.1002/hon.70111","url":null,"abstract":"<div>\u0000 \u0000 <p>The characteristics at diagnosis and clinical course of primary extranodal follicular lymphoma (EFL) have not been extensively described. The International Extranodal Lymphoma Study Group (IELSG) conducted an international retrospective survey aimed to describe the clinical features at diagnosis and the outcomes of FL cases with a clinically dominant extranodal component. The dataset included 605 pathologically reviewed cases from 19 different countries, and their outcomes were compared to those of nodal follicular lymphomas. The two most common presentation sites for EFL were the skin (<i>n</i> = 334) and the gastrointestinal tract (<i>n</i> = 72), with 22 cases having primary duodenal localization. These subsets exhibited unique features at diagnosis and significantly different overall survival (OS) patterns. After a median follow-up of 5.5 years, primary cutaneous lymphomas showed a superior outcome [10-year OS: 89% (95% CI, 83%–93%)], while primary gastrointestinal lymphomas had an intermediate outcome [10-year OS: 79% (95% CI, 59%–90%)]. Among the gastrointestinal lymphomas, primary duodenal lymphomas tended toward the best outcome [10-year OS: 95% (95% CI, 69%–99%)]. Other primary extranodal sites had inferior outcomes [10-year OS: 59% (95% CI, 48%–68%)], similar to primary nodal lymphomas [10-year OS: 57% (95% CI, 49%–64%)]. These findings support the identification of specific primary FL localizations as distinct entities with particular clinical and biological characteristics.</p>\u0000 </div>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 4","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144339133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}